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Pituitary Tumors

The pituitary is an endocrine (hormone-producing) gland located in the brain, behind the bridge of the nose. It is only about the size of a pea, but plays a vital role as the 'master gland' by making hormones that regulate growth and the activity of other glands around the body. There are different types of tumors that can develop in the pituitary. The most common type is pituitary adenoma, which is usually slow growing and benign - these can affect people of all ages, but more women are more affected than men, particularly between ages 30-60. There are many other types of pituitary tumors, including pituitary carcinoma (malignant) and spindle cell oncocytoma, pituicytomas, granular cell tumor. The tumors may be classed as 'secretory' (honrmone producing) or 'non-functional' (non or reduced hormone producing). They are also classed by size: most are microadenomas (small tumors), but a minority are macroadenomas (larger tumours).

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Latest Research Publications

This list of publications is regularly updated (Source: PubMed).

Glebauskiene B, Liutkeviciene R, Vilkeviciute A, et al.
Does MMP-9 Gene Polymorphism Play a Role in Pituitary Adenoma Development?
Dis Markers. 2017; 2017:5839528 [PubMed] Free Access to Full Article Related Publications
Purpose. To determine if the MMP-9 genotype has an influence on development of pituitary adenoma (PA). Methodology. The study enrolled n = 86 patients with PA and n = 526 healthy controls (reference group). The genotyping of MMP-9 was carried out using the real-time polymerase chain reaction method. Results. Our data demonstrated that the MMP-9 (-1562) C/C genotype was more frequent in PA group than in healthy controls (81.4% versus 64.6%, p = 0.002); C/C genotype was more frequently present in PA females compared to healthy control females, 81.5% versus 64.6%, p = 0.018, as well. MMP-9 (-1562) C/C genotype was frequently observed for all subgroups: noninvasive and invasive, nonrecurrence, and inactive PA compared to healthy controls: 81.8% versus 64.6%, p = 0.021; 81.0% versus 64.6%, p = 0.041; 81.8% versus 64.6%, p = 0.005; 100.0% versus 64.6%, p < 0.001, respectively. MMP-9 (-1562) C/C genotype was more frequent in inactive PA compared to active PA: 100.0% versus 71.4%; p < 0.001. Conclusion. MMP-9 (-1562) C/C genotype plays a role in nonrecurrence, inactive, and invasive as well as in nonivasive PA development.

Omodaka S, Ogawa Y, Sato K, et al.
Preoperative embolization and immediate removal of a giant pituitary adenoma: a case report.
BMC Res Notes. 2017; 10(1):63 [PubMed] Free Access to Full Article Related Publications
BACKGROUND: Giant pituitary adenomas, with maximum diameter of at least 40 mm, continue to involve high surgical risks despite recent advances in microsurgical and/or endoscopic surgery. We treated a case of giant pituitary adenoma with preoperative endovascular embolization in an attempt to reduce blood loss.
CASE PRESENTATION: A 48-year-old Japanese Woman presented with severe right visual disturbance. Magnetic resonance imaging revealed a giant pituitary adenoma with maximum diameter of 82 mm. Angiography revealed significant tumor stain, with blood supply mainly from the branches of the right meningohypophyseal trunk. These feeding arteries were endovascularly embolized with n-butyl cyanoacrylate. Subsequently, the tumor was safely removed by transsphenoidal surgery in two stages. The patient showed significant improvement of visual disturbance postoperatively, and was discharged without other neurological deficit. The surgical policy was explained preoperatively to the patients and written informed consents were obtained.
CONCLUSIONS: Preoperative embolization of a giant pituitary adenoma is a useful procedure that can potentially decrease the morbidity and mortality of this devastating tumor.

Kong X, Li D, Kong Y, Zhong D
Malignant adenohypophysis spindle cell oncocytoma with repeating recurrences and a high Ki-67 index.
Medicine (Baltimore). 2017; 96(4):e5657 [PubMed] Free Access to Full Article Related Publications
Adenohypophysis spindle cell oncocytoma (ASCO) is a rare tumor recently reported by Roncaroli et al in 2002. This tumor is considered a grade I tumor by the World Health Organization.We report a rare case of malignant ASCO with repeating recurrences and a high Ki-67 index-a challenging diagnosis guided by clinical presentations, radiological signs, and postoperative pathological tests.We represent a 30-year-old man who had suffered from headaches, diplopia, and impaired visual field and acuity. His magnetic resonance imaging revealed an abnormal sellar mass and was originally misdiagnosed as a pituitary macroadenoma. We present detailed analysis of the patient's disease course and review relevant literature.When surgically treated, the specimen revealed a typical histopathology pattern of ASCO. The tumor recurred for several times and the patient underwent 3 surgeries and 1 γ-knife treatment, which was accompanied by a continuously increasing Ki-67 index.This is the first reported case of malignant ASCO (WHO III-IV grade). Despite its rarity, ASCO should be considered in the differential diagnosis of sellar lesions that mimic pituitary adenomas.

Yu YL, Yang YJ, Lin C, et al.
Analysis of volumetric response of pituitary adenomas receiving adjuvant CyberKnife stereotactic radiosurgery with the application of an exponential fitting model.
Medicine (Baltimore). 2017; 96(4):e4662 [PubMed] Free Access to Full Article Related Publications
Tumor control rates of pituitary adenomas (PAs) receiving adjuvant CyberKnife stereotactic radiosurgery (CK SRS) are high. However, there is currently no uniform way to estimate the time course of the disease. The aim of this study was to analyze the volumetric responses of PAs after CK SRS and investigate the application of an exponential decay model in calculating an accurate time course and estimation of the eventual outcome.A retrospective review of 34 patients with PAs who received adjuvant CK SRS between 2006 and 2013 was performed. Tumor volume was calculated using the planimetric method. The percent change in tumor volume and tumor volume rate of change were compared at median 4-, 10-, 20-, and 36-month intervals. Tumor responses were classified as: progression for >15% volume increase, regression for ≤15% decrease, and stabilization for ±15% of the baseline volume at the time of last follow-up. For each patient, the volumetric change versus time was fitted with an exponential model.The overall tumor control rate was 94.1% in the 36-month (range 18-87 months) follow-up period (mean volume change of -43.3%). Volume regression (mean decrease of -50.5%) was demonstrated in 27 (79%) patients, tumor stabilization (mean change of -3.7%) in 5 (15%) patients, and tumor progression (mean increase of 28.1%) in 2 (6%) patients (P = 0.001). Tumors that eventually regressed or stabilized had a temporary volume increase of 1.07% and 41.5% at 4 months after CK SRS, respectively (P = 0.017). The tumor volume estimated using the exponential fitting equation demonstrated high positive correlation with the actual volume calculated by magnetic resonance imaging (MRI) as tested by Pearson correlation coefficient (0.9).Transient progression of PAs post-CK SRS was seen in 62.5% of the patients receiving CK SRS, and it was not predictive of eventual volume regression or progression. A three-point exponential model is of potential predictive value according to relative distribution. An exponential decay model can be used to calculate the time course of tumors that are ultimately controlled.

Duan K, Asa SL, Winer D, et al.
Xanthomatous Hypophysitis Is Associated with Ruptured Rathke's Cleft Cyst.
Endocr Pathol. 2017; 28(1):83-90 [PubMed] Related Publications
Xanthomatous hypophysitis is a rare inflammatory disease of the pituitary gland that can mimic a neoplastic lesion clinically and radiologically. Its pathogenesis remains largely unknown, although recent evidence suggests that pituitary inflammation may occur as a secondary reaction to mucous content released from a ruptured cyst. In a series of 1221 pituitary specimens, we identified seven cases of xanthomatous hypophysitis. Six patients had complete radiological and biochemical workup preoperatively: a cystic-appearing pituitary mass was identified in all six patients (100%) with a mean size of 2.0 cm (range 1.4-2.5 cm) on imaging, and pituitary endocrine dysfunction was noted in five patients (83.3%). In all cases, the pituitary mass was resected through an endoscopic transsphenoidal approach. Pathological examination revealed the presence of foamy macrophages admixed with variable amounts of giant cells and chronic inflammatory cells, confirming the diagnosis of xanthomatous hypophysitis. Additionally, all cases presented with concurrent findings of ruptured Rathke's cleft cyst, with the exception of one patient who had previous surgery for a Rathke's cleft cyst, followed by recurrence and diagnosis of xanthomatous hypophysitis. While accurate distinction of hypophysitis from a pituitary neoplasm can be problematic in the preoperative setting, the identification of a cystic lesion in the sella turcica should raise the possibility of such an entity in the clinical and radiological differential diagnosis. The current series provides further evidence that xanthomatous hypophysitis predominantly occurs as a secondary reaction to a ruptured Rathke's cleft cyst; thus, it is best classified as a secondary (reactive) hypophysitis.

Song YJ, Chen MT, Lian W, et al.
Surgical treatment for male prolactinoma: A retrospective study of 184 cases.
Medicine (Baltimore). 2017; 96(2):e5833 [PubMed] Free Access to Full Article Related Publications
A total of 184 cases of surgically treated male prolactinoma were analyzed retrospectively to summarize the outcome of this surgical intervention. We analyzed the general characteristics, clinical manifestations, hormone levels, imaging features, preoperative treatments, surgical outcomes, pathology results, and follow-up records for all included patients. The most common clinical manifestations included sexual dysfunction (47.4%), headache (55.9%), and visual disturbance (46.7%). Serum prolactin levels ranged from 150 to 204,952 ng/mL. Tumor size varied from 6 to 70 mm. Pituitary adenomas grew in a parasellar pattern with visual deficits occurring 40.7% of the time. After surgical therapy, 88.6% of patients achieved symptom relief, and 98.4% experienced an immediate postoperative decline in prolactin level. Fifty-seven patients (31.0%) achieved initial remission, and 26 patients (45.6%) experienced recurrence. Hence, our results suggest that in male prolactinoma characterized by a large pituitary diameter and high serum prolactin level, tumor size predicts the degree of gross resection. The prognostic predictors included preoperative tumor growth pattern and Ki-67 index.Citation: Yi-jun S, Mei-ting C, Wei L, Bing X, Yong Y, Ming F, Ren-zhi W. (2016) Surgical treatment for male prolactinoma: a retrospective study of 184 cases.

Mezzomo LC, Pesce FG, Marçal JM, et al.
Decreased TAp63 and ΔNp63 mRNA Levels in Most Human Pituitary Adenomas Are Correlated with Notch3/Jagged1 Relative Expression.
Endocr Pathol. 2017; 28(1):13-21 [PubMed] Related Publications
Despite recent advances in molecular genetics, the pituitary adenoma initiation, development, progress, and the molecular basis of their unique features are still poorly understood. In this sense, it is proposed that stem cell could be involved in pituitary adenoma tumorigenesis. It is suggested that TP63 has important functions in stem cells, and it may have interplay of TP63 and Notch and its ligand Jagged in this process. This study aimed to evaluate the distinct expression of TP63 isoforms (TAp63 and ΔNp63), as well as its correlation with Notch3 receptor and its ligand Jagged1 in human pituitary adenomas at the messenger RNA (mRNA) level. We included 77 pituitary adenoma tumor samples from patients who underwent surgical resection. The expression levels of TP63 isoforms (TAp63 and ΔNp63) and Notch3 and its ligand Jagged1 were evaluated by qRT-PCR using isoform-specific primers. We also evaluated proliferation index immunohistochemically using KI-67 antibody. The expression levels were associated with clinical outcomes, as age, gender, tumor size, and tumor subtype. In summary, we found that mRNA expression of both TP63 isoforms decreased in pituitary adenomas compared with normal pituitary control. On the other hand, there was an increase of relative Notch3 and Jagged1 mRNA expression in the majority of examined samples. The mRNA expression of three genes evaluated was correlated and statistically significantly. There was no significant association between gene expression and the analyzed clinical data. The current study has provided the first time evidence that Tap63 and ΔNp63 isoforms are underexpressed in most pituitary adenomas. These results are correlated with Notch3 and its ligand Jagged1 overexpression, corroborating previous studies pointing its antagonistic interactions.

Tan TS, Patel L, Gopal-Kothandapani JS, et al.
The neuroendocrine sequelae of paediatric craniopharyngioma: a 40-year meta-data analysis of 185 cases from three UK centres.
Eur J Endocrinol. 2017; 176(3):359-369 [PubMed] Related Publications
OBJECTIVES: The management of paediatric craniopharyngiomas was traditionally complete resection (CR), with better reported tumour control compared to that by partial resection (PR) or limited surgery (LS). The subsequent shift towards hypothalamic sparing, conservative surgery with adjuvant radiotherapy (RT) to any residual tumour aimed at reducing neuroendocrine morbidity, has not been systematically studied. Hence, we reviewed the sequelae of differing management strategies in paediatric craniopharyngioma across three UK tertiary centres over four decades.
METHODS: Meta-data was retrospectively reviewed over two periods before (1973-2000 (Group A: n = 100)) and after (1998-2011 (Group B: n = 85)) the introduction of the conservative strategy at each centre.
RESULTS: Patients had CR (A: 34% and B: 19%), PR (A: 48% and B: 46%) or LS (A: 16% and B: 34%), with trends reflecting the change in surgical approach over time. Overall recurrence rates between the two periods did not change (A: 38% vs B: 32%). More patients received RT in B than A, but recurrence rates were similar: for A, 28% patients received RT with 9 recurrences (32%); for B, 62% received RT with 14 recurrences (26%). However, rates of diabetes insipidus (P = 0.04), gonadotrophin deficiency (P < 0.001) and panhypopituitarism (P = 0.001) were lower in B than those in A. In contrast, post-operative obesity (BMI SDS >+2.0) (P = 0.4) and hypothalamic (P = 0.1) and visual (P = 0.3) morbidity rates were unchanged.
CONCLUSION: The shift towards more conservative surgery has reduced the prevalence of hormone deficiencies, including diabetes insipidus, which can be life threatening. However, it has not been associated with reduced hypothalamic and visual morbidities, which remain a significant challenge. More effective targeted therapies are necessary to improve outcomes.

Eroukhmanoff J, Tejedor I, Potorac I, et al.
MRI follow-up is unnecessary in patients with macroprolactinomas and long-term normal prolactin levels on dopamine agonist treatment.
Eur J Endocrinol. 2017; 176(3):323-328 [PubMed] Related Publications
OBJECTIVE: Both antitumor and antisecretory efficacies of dopamine agonists (DA) make them the first-line treatment of macroprolactinomas. However, there is no guideline for MRI follow-up once prolactin is controlled. The aim of our study was to determine whether a regular MRI follow-up was necessary in patients with long-term normal prolactin levels under DA.
PATIENTS AND METHODS: We conducted a retrospective multicenter study (Marseille, Paris La Pitie Salpetriere and Nancy, France; Liege, Belgium) including patients with macroprolactinomas (largest diameter: >10 mm and baseline prolactin level: >100 ng/mL) treated by dopamine agonists, and regularly followed (pituitary MRI and prolactin levels) during at least 48 months once normal prolactin level was obtained.
RESULTS: In total, 115 patients were included (63 men and 52 women; mean age at diagnosis: 36.3 years). Mean baseline prolactin level was 2224 ± 6839 ng/mL. No significant increase of tumor volume was observed during the follow-up. Of the 21 patients (18%) who presented asymptomatic hemorrhagic changes of the macroprolactinoma on MRI, 2 had a tumor increase (2 and 7 mm in the largest size). Both were treated by cabergoline (1 mg/week) with normal prolactin levels obtained for 6 and 24 months. For both patients, no further growth was observed on MRI during follow-up at the same dose of cabergoline.
CONCLUSION: No significant increase of tumor size was observed in our patients with controlled prolactin levels on DA. MRI follow-up thus appears unnecessary in patients with biologically controlled macroprolactinomas.

Li CZ, Li CC, Hsieh CC, et al.
Fatal antiphospholipid syndrome following endoscopic transnasal-transsphenoidal surgery for a pituitary tumor: A case report.
Medicine (Baltimore). 2017; 96(1):e5774 [PubMed] Free Access to Full Article Related Publications
INTRODUCTION: The fatal type of antiphospholipid syndrome is a rare but life-threating condition. It may be triggered by surgery or infection. Endoscopic transnasal-transsphenoidal surgery is a common procedure for pituitary tumor. We report a catastrophic case of a young woman died of fatal antiphospholipid syndrome following endoscopic transnasal-transsphenoidal surgery.
METHODS AND RESULT: A 31-year-old woman of a history of stroke received endoscopic transnasal-transsphenoidal surgery for a pituitary tumor. The whole procedure was smooth. However, the patient suffered from acute delirium on postoperative day 4. Then, her consciousness became comatose state rapidly with dilatation of pupils. Urgent magnetic resonance imaging of brain demonstrated multiple acute lacunar infarcts. The positive antiphosphoipid antibody and severe thrombocytopenia were also noted. Fatal antiphospholipid syndrome was diagnosed. Plasma exchange, corticosteroids, anticoagulant agent were prescribed. The hemodynamic condition was gradually stable. However, the consciousness was still in deep coma. The patient died of organ donation 2 months later.
CONCLUSION: If patients have a history of cerebral stroke in their early life, such as a young stroke, the APS and higher risk of developing fatal APS after major surgery should be considered. The optimal management of APS remains controversial. The best treatment strategies are only early diagnosis and aggressive therapies combing of anticoagulant, corticosteroid, and plasma exchange. The intravenous immunoglobulin is prescribed for patients with refractory APS.

Yao Y, Liu Y, Wang L, et al.
Clinical characteristics and management of growth hormone excess in patients with McCune-Albright syndrome.
Eur J Endocrinol. 2017; 176(3):295-303 [PubMed] Related Publications
OBJECTIVE: McCune-Albright syndrome (MAS) is a sporadic, postzygotic disease presenting with fibrous dysplasia, cafe-au-lait spots and multiple endocrinopathies. Growth hormone (GH) excess is an uncommon but potentially severe complication of MAS. This study aims to describe the clinical manifestations of GH excess in the context of MAS and analyze the responses of these patients to treatments.
DESIGN: Retrospective clinical study.
METHODS: Clinical data from 52 MAS patients were analyzed. Serum GH and IGF1 levels, as well as nadir GH levels after an oral glucose tolerance test and alkaline phosphatase (ALP) levels were determined before and after the treatment.
RESULTS: In total, 13 MAS patients (25%) had the complication of GH excess, including 10 males (76.9%). Among them, all had FD, and 6 patients had sphenoidal bone involvement. Visual deficits were present in 8 patients, and hearing deficits were present in 5. Olfactory dysfunction was observed in 3 patients. Evident pituitary adenomas were confirmed in 9 patients by MRI. These patients underwent surgery with or without pretreatment of long-acting somatostatin analogue octreotide, and 6 achieved complete remission. The serum ALP levels decreased significantly after treatment for GH excess.
CONCLUSIONS: MAS with GH excess is more common in male patients. GH excess can lead to more severe skeletal lesions in MAS patients involving more of the craniofacial bones. Complete trans-sphenoidal complete tumor excision with neuronavigational guidance is effective and could lower ALP levels. LAR is recommended as a preoperative treatment and when patients fail to achieve complete remission after surgery.

Ritvonen E, Pitkänen E, Karppinen A, et al.
Impact of AIP and inhibitory G protein alpha 2 proteins on clinical features of sporadic GH-secreting pituitary adenomas.
Eur J Endocrinol. 2017; 176(2):243-252 [PubMed] Related Publications
INTRODUCTION: In sporadic acromegaly, downregulation of AIP protein of the adenomas associates with invasive tumor features and reduced responsiveness to somatostatin analogues. AIP is a regulator of Gai signaling, but it is not known how the biological function of the Gai pathway is controlled.
AIM: To study GNAS and AIP mutation status, AIP and Gai-2 protein expressions, Ki-67 proliferation indices and clinical parameters in patients having primary surgery because of acromegaly at a single center between years 2000 and 2010.
RESULTS: Sixty patients (F/M, 31/29), mean age 49 (median 50), mean follow-up 7.7 years (range 0.6-14.0) underwent primary surgery. Four adenoma specimens (6.8%) harbored an AIP and 21 (35.6%) an activating GNAS (Gsp+) mutation. Altogether 13/56 (23%) adenomas had low AIP protein levels, and 14/56 (25%) low Gai-2 staining. In regression modeling, AIP expression associated with Gai-2 (P = 2.33 × 10(-9)) and lower Ki-67 (P = 0.04). In pairwise comparison, low AIP protein predicted high GH at last follow-up (mean 7.7 years after surgery, q = 0.045). Extent of treatments given for acromegaly associated with higher preoperative GH (P = 7.94 × 10(-4)), KNOSP (P = 0.003) and preoperative hypopituitarism (P = 0.03) and remission at last follow-up with change in 3-month postoperative IGF1 (P = 2.07 × 10(-7)).
CONCLUSIONS: We demonstrate, for the first time, that AIP protein expression associates with Gai-2 protein intensities in sporadic somatotropinomas, suggesting a joint regulation on somatostatin signaling. Low AIP level associates with higher proliferative activity and predicts high GH concentrations after long-term follow-up. The AIP mutation rate of 6.8% is fairly high, reflecting the genetic composition of the Finnish population.

He W, Huang L, Shen X, et al.
Relationship between RSUME and HIF-1α/VEGF-A with invasion of pituitary adenoma.
Gene. 2017; 603:54-60 [PubMed] Related Publications
The RWD-containing sumoylation enhancer (RSUME) can stabilize hypoxia-inducible factor-1α (HIF-1α) which promotes vascular endothelial growth factor-A (VEGF-A) expression. RSUME plays an important role in promoting the invasion of pituitary adenoma. In this study, we compared the mRNA and protein levels of RSUME, HIF-1α, and VEGF-A in pituitary adenoma tissue and analyzed the correlation. We found that the expression levels of RSUME, HIF-1α, and VEGF-A in invasive pituitary adenoma were significantly higher than in noninvasive pituitary adenoma. Moreover, a positive correlation was found between RSUME and HIF-1α/VEGF pathways. RSUME and HIF-1α were treated with hypoxia-mimicking CoCl2 and transfected into AtT-20 and GT1.1 cell lines to determine the relationship between them. It was found that RSUME effects post-transcriptional expression of HIF-1α regulated VEGF-A secretion. Reducing RSUME expression using siRNA transfection resulted in a decrease of the invasion inhibition rate of AtT-20 cells, as determined using Transwell and MTT assays. Together, we found that RSUME silencing can inhibit the invasion of pituitary adenoma cells.

Chinezu L, Vasiljevic A, Trouillas J, et al.
Silent somatotroph tumour revisited from a study of 80 patients with and without acromegaly and a review of the literature.
Eur J Endocrinol. 2017; 176(2):195-201 [PubMed] Related Publications
BACKGROUND: Silent somatotroph tumours are growth hormone (GH) immunoreactive (IR) pituitary tumours without clinical and biological signs of acromegaly. Their better characterisation is required to improve the diagnosis.
MATERIALS AND METHODS: Twenty-one silent somatotroph tumours were compared to 59 somatotroph tumours with acromegaly. Tumours in each group were classified into GH and plurihormonal (GH/prolactin (PRL)/±thyroid-stimulating hormone (TSH)) and into densely granulated (DG) and sparsely granulated (SG) types. The two groups were then compared with regards to proliferation (Ki-67, p53 indexes and mitotic count), differentiation (expression of somatostatin receptors SSTR2A-SSTR5 and transcription factor Pit-1) and secretory activity (% of GH- and PRL-IR cells).
RESULTS: The silent somatotroph tumours represented 2% of all tested pituitary tumours combined. They were more frequent in women than in men (P = 0.002), more frequently plurihormonal and SG (P < 0.01), with a lower percentage of GH-IR cells (P < 0.0001) compared to those with acromegaly. They all expressed SSTR2A, SSTR5 and Pit-1. The plurihormonal (GH/PRL/±TSH) tumours were mostly observed in women (sex ratio: 3/1) and in patients who were generally younger than those with acromegaly (P < 0.001). They were larger (P < 0.001) with a higher Ki-67 index (P = 0.007).
CONCLUSIONS: The silent somatotroph tumours are not uncommon. Their pathological diagnosis requires the immunodetection of GH and Pit-1. They are more frequently plurihormonal and more proliferative than those with acromegaly. A low secretory activity of these tumours might explain the normal plasma values for GH and insulin-like growth factor 1 (IGF1) and the absence of clinical signs of acromegaly.

Zoli M, Sambati L, Milanese L, et al.
Postoperative outcome of body core temperature rhythm and sleep-wake cycle in third ventricle craniopharyngiomas.
Neurosurg Focus. 2016; 41(6):E12 [PubMed] Related Publications
OBJECTIVE One of the more serious risks in the treatment of third ventricle craniopharyngiomas is represented by hypothalamic damage. Recently, many papers have reported the expansion of the indications for the endoscopic endonasal approach (EEA) to be used for these tumors as well. The aim of this study was to assess the outcome of sleep-wake cycle and body core temperature (BCT), both depending on hypothalamic control, in patients affected by craniopharyngiomas involving the third ventricle that were surgically treated via an EEA. METHODS All consecutive adult patients with craniopharyngiomas that were treated at one center via an EEA between 2014 and 2016 were prospectively included. Each patient underwent neuroradiological, endocrinological, and ophthalmological evaluation; 24-hour monitoring of the BCT rhythm; and the sleep-wake cycle before surgery and at follow-up of at least 6 months. RESULTS Ten patients were included in the study (male/female ratio 4:6, mean age 48.6 years, SD 15.9 years). Gross-total resection was achieved in 8 cases. Preoperative BCT rhythm was pathological in 6 patients. After surgery, these disturbances resolved in 2 cases, improved in another 3, and remained the same in 1 patient; also, 1 case of de novo onset was observed. Before surgery the sleep-wake cycle was pathological in 8 cases, and it was restored in 4 patients at follow-up. After surgery the number of patients reporting diurnal naps increased from 7 to 9. CONCLUSIONS The outcome of the sleep-wake cycle and BCT analyzed after EEA in this study is promising. Despite the short duration of the authors' experience, they consider these results encouraging; additional series are needed to confirm the preliminary findings.

Robinson LC, Santagata S, Hankinson TC
Potential evolution of neurosurgical treatment paradigms for craniopharyngioma based on genomic and transcriptomic characteristics.
Neurosurg Focus. 2016; 41(6):E3 [PubMed] Related Publications
The recent genomic and transcriptomic characterization of human craniopharyngiomas has provided important insights into the pathogenesis of these tumors and supports that these tumor types are distinct entities. Critically, the insights provided by these data offer the potential for the introduction of novel therapies and surgical treatment paradigms for these tumors, which are associated with high morbidity rates and morbid conditions. Mutations in the CTNNB1 gene are primary drivers of adamantinomatous craniopharyngioma (ACP) and lead to the accumulation of β-catenin protein in a subset of the nuclei within the neoplastic epithelium of these tumors. Dysregulation of epidermal growth factor receptor (EGFR) and of sonic hedgehog (SHH) signaling in ACP suggest that paracrine oncogenic mechanisms may underlie ACP growth and implicate these signaling pathways as potential targets for therapeutic intervention using directed therapies. Recent work shows that ACP cells have primary cilia, further supporting the potential importance of SHH signaling in the pathogenesis of these tumors. While further preclinical data are needed, directed therapies could defer, or replace, the need for radiation therapy and/or allow for less aggressive surgical interventions. Furthermore, the prospect for reliable control of cystic disease without the need for surgery now exists. Studies of papillary craniopharyngioma (PCP) are more clinically advanced than those for ACP. The vast majority of PCPs harbor the BRAF(v600e) mutation. There are now 2 reports of patients with PCP that had dramatic therapeutic responses to targeted agents. Ongoing clinical and research studies promise to not only advance our understanding of these challenging tumors but to offer new approaches for patient management.

Wannemuehler TJ, Rubel KE, Hendricks BK, et al.
Outcomes in transcranial microsurgery versus extended endoscopic endonasal approach for primary resection of adult craniopharyngiomas.
Neurosurg Focus. 2016; 41(6):E6 [PubMed] Related Publications
OBJECTIVE Craniopharyngiomas have historically been resected via transcranial microsurgery (TCM). In the last 2 decades, the extended endoscopic endonasal (transtuberculum) approach to these tumors has become more widely accepted, yet there remains controversy over which approach leads to better outcomes. The purpose of this study is to determine whether differences in outcomes were identified between TCM and extended endoscopic endonasal approaches (EEEAs) in adult patients undergoing primary resection of suprasellar craniopharyngiomas at a single institution. METHODS A retrospective review of all patients who underwent resection of their histopathologically confirmed craniopharyngiomas at the authors' institution between 2005 and 2015 was performed. Pediatric patients, revision cases, and patients with tumors greater than 2 standard deviations above the mean volume were excluded. The patients were divided into 2 groups: those undergoing primary TCM and those undergoing a primary EEEA. Preoperative patient demographics, presenting symptoms, and preoperative tumor volumes were determined. Extent of resection, tumor histological subtype, postoperative complications, and additional outcome data were obtained. Statistical significance between variables was determined utilizing Student t-tests, chi-square tests, and Fisher exact tests when applicable. RESULTS After exclusions, 21 patients satisfied the aforementioned inclusion criteria; 12 underwent TCM for resection while 9 benefitted from the EEEA. There were no significant differences in patient demographics, presenting symptoms, tumor subtype, or preoperative tumor volumes; no tumors had significant lateral or prechiasmatic extension. The extent of resection was similar between these 2 groups, as was the necessity for additional surgery or adjuvant therapy. CSF leakage was encountered only in the EEEA group (2 patients). Importantly, the rate of postoperative visual improvement was significantly higher in the EEEA group than in the TCM group (88.9% vs 25.0%; p = 0.0075). Postoperative visual deterioration only occurred in the TCM group (3 patients). Recurrence was uncommon, with similar rates between the groups. Other complication rates, overall complication risk, and additional outcome measures were similar between these groups as well. CONCLUSIONS Based on this study, most outcome variables appear to be similar between TCM and EEEA routes for similarly sized tumors in adults. The multidisciplinary EEEA to craniopharyngioma resection represents a safe and compelling alternative to TCM. The authors' data demonstrate that postoperative visual improvement is statistically more likely in the EEEA despite the increased risk of CSF leakage. These results add to the growing evidence that the EEEA may be considered the approach of choice for resection of select confined primary craniopharyngiomas without significant lateral extension in centers with experienced surgeons. Further prospective, multiinstitutional collaboration is needed to power studies capable of fully evaluating indications and appropriate approaches for craniopharyngiomas.

Martinez-Gutierrez JC, D'Andrea MR, Cahill DP, et al.
Diagnosis and management of craniopharyngiomas in the era of genomics and targeted therapy.
Neurosurg Focus. 2016; 41(6):E2 [PubMed] Related Publications
Craniopharyngiomas are rare intracranial neoplasms that pose clinical challenges due to their location adjacent to vital structures. The authors have previously shown high mutation rates of BRAF V600E in papillary craniopharyngioma and of CTNNB1 in adamantinomatous craniopharyngioma. These activating driver mutations are potential therapeutic targets, and the authors have recently reported a significant response to BRAF/MEK inhibition in a patient with multiply recurrent PCP. As these targetable mutations warrant prospective research, the authors will be conducting a national National Cancer Institute-sponsored multicenter clinical trial to investigate BRAF/MEK inhibition in the treatment of craniopharyngioma. In this new era of genomic discovery, the treatment paradigm of craniopharyngioma is likely to change.

Stache C, Bils C, Fahlbusch R, et al.
Drug priming enhances radiosensitivity of adamantinomatous craniopharyngioma via downregulation of survivin.
Neurosurg Focus. 2016; 41(6):E14 [PubMed] Related Publications
OBJECTIVE In this study, the authors investigated the underlying mechanisms responsible for high tumor recurrence rates of adamantinomatous craniopharyngioma (ACP) after radiotherapy and developed new targeted treatment protocols to minimize recurrence. ACPs are characterized by the activation of the receptor tyrosine kinase epidermal growth factor receptor (EGFR), known to mediate radioresistance in various tumor entities. The impact of tyrosine kinase inhibitors (TKIs) gefitinib or CUDC-101 on radiation-induced cell death and associated regulation of survivin gene expression was evaluated. METHODS The hypothesis that activated EGFR promotes radioresistance in ACP was investigated in vitro using human primary cell cultures of ACP (n = 10). The effects of radiation (12 Gy) and combined radiochemotherapy on radiosensitivity were assessed via cell death analysis using flow cytometry. Changes in target gene expression were analyzed by quantitative real-time polymerase chain reaction (qRT-PCR). Survivin, identified in qRT-PCR to be involved in radioresistance of ACP, was manipulated by small interfering RNA (siRNA), followed by proliferation and vitality assays to further clarify its role in ACP biology. Immunohistochemically, survivin expression was assessed in patient tumors used for primary cell cultures. RESULTS In primary human ACP cultures, activation of EGFR resulted in significantly reduced cell death levels after radiotherapy. Treatment with TKIs alone and in combination with radiotherapy increased cell death response remarkably, assessed by flow cytometry. CUDC-101 was significantly more effective than gefitinib. The authors identified regulation of survivin expression after therapeutic intervention as the underlying molecular mechanism of radioresistance in ACP. EGFR activation promoting ACP cell survival and proliferation in vitro is consistent with enhanced survivin gene expression shown by qRT-PCR. TKI treatment, as well as the combination with radiotherapy, reduced survivin levels in vitro. Accordingly, ACP showed reduced cell viability and proliferation after survivin downregulation by siRNA. CONCLUSIONS These results indicate an impact of EGFR signaling on radioresistance in ACP. Inhibition of EGFR activity by means of TKI treatment acts as a radiosensitizer on ACP tumor cells, leading to increased cell death. Additionally, the results emphasize the antiapoptotic and pro-proliferative role of survivin in ACP biology and its regulation by EGFR signaling. The suppression of survivin by treatment with TKI and combined radiotherapy represents a new promising treatment strategy that will be further assessed in in vivo models of ACP.

Turel MK, Tsermoulas G, Gonen L, et al.
Management and outcome of recurrent adult craniopharyngiomas: an analysis of 42 cases with long-term follow-up.
Neurosurg Focus. 2016; 41(6):E11 [PubMed] Related Publications
OBJECTIVE The treatment of recurrent and residual craniopharyngiomas is challenging. In this study the authors describe their experience with these tumors and make recommendations on their management. METHODS The authors performed an observational study of adult patients (≥ 18 years) with recurrent or residual craniopharyngiomas that were managed at their tertiary center. Retrospective data were collected on demographics and clinical, imaging, and treatment characteristics from patients who had a minimum 2-year follow-up. Descriptive statistics were used and the data were analyzed. RESULTS There were 42 patients (27 male, 15 female) with a mean age of 46.3 ± 14.3 years. The average tumor size was 3.1 ± 1.1 cm. The average time to first recurrence was 3.6 ± 5.5 years (range 0.2-27 years). One in 5 patients (8/42) with residual/recurrent tumors did not require any active treatment. Of the 34 patients who underwent repeat treatment, 12 (35.3%) had surgery only (transcranial, endoscopic, or both), 9 (26.5%) underwent surgery followed by adjuvant radiation therapy (RT), and 13 (38.2%) received RT alone. Eighty-six percent (18/21) had a gross-total (n = 4) or near-total (n = 14) resection of the recurrent/residual tumors and had good local control at last follow-up. One of 5 patients (7/34) who underwent repeat treatment had further treatment for a second recurrence. The total duration of follow-up was 8.6 ± 7.1 years. The average Karnofsky Performance Scale score at last follow-up was 80 (range 40-90). There was 1 death. CONCLUSIONS Based on this experience and in the absence of guidelines, the authors recommend an individualized approach for the treatment of symptomatic or growing tumors. This study has shown that 1 in 5 patients does not require repeat treatment of their recurrent/residual disease and can be managed with a "scan and watch" approach. On the other hand, 1 in 5 patients who had repeat treatment for their recurrence in the form of surgery and/or radiation will require further additional treatment. More studies are needed to best characterize these patients and predict the natural history of this disease and response to treatment.

Prieto R, Pascual JM, Rosdolsky M, et al.
Craniopharyngioma adherence: a comprehensive topographical categorization and outcome-related risk stratification model based on the methodical examination of 500 tumors.
Neurosurg Focus. 2016; 41(6):E13 [PubMed] Related Publications
OBJECTIVE Craniopharyngioma (CP) adherence strongly influences the potential for achieving a radical and safe surgical treatment. However, this factor remains poorly addressed in the scientific literature. This study provides a rational, comprehensive description of CP adherence that can be used for the prediction of surgical risks associated with the removal of these challenging lesions. METHODS This study retrospectively analyzes the evidence provided in pathological, neuroradiological, and surgical CP reports concerning 3 components of the CP attachment: 1) the intracranial structures attached to the tumor; 2) the morphology of the adhesion; and 3) the adhesion strength. From a total of 1781 CP reports published between 1857 and 2016, a collection of 500 CPs providing the best information about the type of CP attachment were investigated. This cohort includes autopsy studies (n = 254); surgical studies with a detailed description or pictorial evidence of CP adherence (n = 298); and surgical CP videos (n = 61) showing the technical steps for releasing the attachment. A predictive model of CP adherence in hierarchical severity levels correlated with surgical outcomes was generated by multivariate analysis. RESULTS The anatomical location of the CP attachment occurred predominantly at the third ventricle floor (TVF) (54%, n = 268), third ventricle walls (23%, n = 114), and pituitary stalk (19%, n = 94). The optic chiasm was involved in 56% (n = 281). Six morphological patterns of CP attachment were identified: 1) fibrovascular pedicle (5.4%); 2) sessile or patch-like (21%); 3) cap-like (over the CP top, 14%); 4) bowl-like (around the CP bottom, 13.5%); 5) ring-like (encircling central band, 19%); and 6) circumferential (enveloping the entire CP, 27%). Adhesion strength was classified in 4 grades: 1) loose (easily dissectible, 8%); 2) tight (requires sharp dissection, 32%); 3) fusion (no clear cleavage plane, 40%); and 4) replacement (loss of brain tissue integrity, 20%). The types of CP attachment associated with the worst surgical outcomes are the ring-like, bowl-like, and circumferential ones with fusion to the TVF or replacement of this structure (p < 0.001). The CP topography is the variable that best predicts the type of CP attachment (p < 0.001). Ring-like and circumferential attachments were observed for CPs invading the TVF (secondary intraventricular CPs) and CPs developing within the TVF itself (infundibulo-tuberal CPs). Brain invasion and peritumoral gliosis occurred predominantly in the ring-like and circumferential adherence patterns (p < 0.001). A multivariate model including the variables CP topography, tumor consistency, and the presence of hydrocephalus, infundibulo-tuberal syndrome, and/or hypothalamic dysfunction accurately predicts the severity of CP attachment in 87% of cases. CONCLUSIONS A comprehensive descriptive model of CP adherence in 5 hierarchical levels of increased severity-mild, moderate, serious, severe, and critical-was generated. This model, based on the location, morphology, and strength of the attachment can be used to anticipate the surgical risk of hypothalamic injury and to plan the degree of removal accordingly.

Apps JR, Martinez-Barbera JP
Molecular pathology of adamantinomatous craniopharyngioma: review and opportunities for practice.
Neurosurg Focus. 2016; 41(6):E4 [PubMed] Related Publications
Since the first identification of CTNNB1 mutations in adamantinomatous craniopharyngioma (ACP), much has been learned about the molecular pathways and processes that are disrupted in ACP pathogenesis. To date this understanding has not translated into tangible patient benefit. The recent development of novel techniques and a range of preclinical models now provides an opportunity to begin to support treatment decisions and develop new therapeutics based on molecular pathology. In this review the authors summarize many of the key findings and pathways implicated in ACP pathogenesis and discuss the challenges that need to be tackled to translate these basic science findings for the benefit of patients.

Kshettry VR, Do H, Elshazly K, et al.
The learning curve in endoscopic endonasal resection of craniopharyngiomas.
Neurosurg Focus. 2016; 41(6):E9 [PubMed] Related Publications
OBJECTIVE There is a paucity of literature regarding the learning curve associated with performing endoscopic endonasal cranial base surgery. The purpose of this study was to determine to what extent a learning curve might exist for endoscopic endonasal resection in cases of craniopharyngiomas. METHODS A retrospective review was performed for all endoscopic endonasal craniopharyngioma resections performed at Thomas Jefferson University from 2005 to 2015. To assess for a learning curve effect, patients were divided into an early cohort (2005-2009, n = 20) and a late cohort (2010-2015, n = 23). Preoperative demographics, clinical presentation, imaging characteristics, extent of resection, complications, tumor control, and visual and endocrine outcomes were obtained. Categorical variables and continuous variables were compared using a 2-sided Fisher's exact test and t-test, respectively. RESULTS Only the index operation performed at the authors' institution was included. There were no statistically significant differences between early and late cohorts in terms of patient age, sex, presenting symptoms, history of surgical or radiation treatment, tumor size or consistency, hypothalamic involvement, or histological subtype. The rate of gross-total resection (GTR) increased over time from 20% to 65% (p = 0.005), and the rate of subtotal resection decreased over time from 40% to 13% (p = 0.078). Major neurological complications, including new hydrocephalus, meningitis, carotid artery injury, or stroke, occurred in 6 patients (15%) (8 complications) in the early cohort compared with only 1 (4%) in the late cohort (p = 0.037). CSF leak decreased from 40% to 4% (p = 0.007). Discharge to home increased from 64% to 95% (p = 0.024). Visual improvement was high in both cohorts (88% [early cohort] and 81% [late cohort]). Rate of postoperative panhypopituitarism and permanent diabetes insipidus both increased from 50% to 91% (p = 0.005) and 32% to 78% (p = 0.004), which correlated with a significant increase in intentional stalk sacrifice in the late cohort (from 0% to 70%, p < 0.001). CONCLUSIONS High rates of near- or total resection and visual improvement can be achieved using an endoscopic endonasal approach for craniopharyngiomas. However, the authors did find evidence for a learning curve. After 20 cases, they found a significant decrease in major neurological complications and significant increases in the rates of GTR rate and discharge to home. Although there was a large decrease in the rate of postoperative CSF leak over time, this was largely attributable to the inclusion of very early cases prior to the routine use of vascularized nasoseptal flaps. There was a significant increase in new panhypopituitarism and diabetes insipidus, which is attributable to increase rates of intentional stalk sacrifice.

Abou-Al-Shaar H, Abd-El-Barr MM, Zaidi HA, et al.
Frontal dermoid cyst coexisting with suprasellar craniopharyngioma: a spectrum of ectodermally derived epithelial-lined cystic lesions?
Neurosurg Focus. 2016; 41(6):E16 [PubMed] Related Publications
There is a wide group of lesions that may exist in the sellar and suprasellar regions. Embryologically, there is varying evidence that many of these entities may in fact represent a continuum of pathology deriving from a common ectodermal origin. The authors report a case of a concomitant suprasellar craniopharyngioma invading the third ventricle with a concurrent frontal lobe cystic dermoid tumor. A 21-year-old man presented to the authors' service with a 3-day history of worsening headache, nausea, vomiting, and blurry vision. Magnetic resonance imaging depicted a right frontal lobe lesion associated with a separate suprasellar cystic lesion invading the third ventricle. The patient underwent a right pterional craniotomy for resection of both lesions. Gross-total resection of the right frontal lesion was achieved, and subtotal resection of the suprasellar lesion was accomplished with some residual tumor adherent to the walls of the third ventricle. Histopathological examination of the resected right frontal lesion documented a diagnosis of dermoid cyst and, for the suprasellar lesion, a diagnosis of adamantinomatous craniopharyngioma. The occurrence of craniopharyngioma with dermoid cyst has not been reported in the literature before. Such an association might indeed suggest the previously reported hypothesis that these lesions represent a spectrum of ectodermally derived epithelial-lined cystic lesions.

Bakhsheshian J, Jin DL, Chang KE, et al.
Risk factors associated with the surgical management of craniopharyngiomas in pediatric patients: analysis of 1961 patients from a national registry database.
Neurosurg Focus. 2016; 41(6):E8 [PubMed] Related Publications
OBJECTIVE Patient demographic characteristics, hospital volume, and admission status have been shown to impact surgical outcomes of sellar region tumors in adults; however, the data available following the resection of craniopharyngiomas in the pediatric population remain limited. The authors sought to identify potential risk factors associated with outcomes following surgical management of pediatric craniopharyngiomas. METHODS The Nationwide Inpatient Sample database and Kids' Inpatient Database were analyzed to include admissions for pediatric patients (≤ 18 years) who underwent a transcranial or transsphenoidal craniotomy for resection of a craniopharyngioma. Patient-level factors, including age, race, comorbidities, and insurance type, as well as hospital factors were collected. Outcomes analyzed included mortality rate, endocrine and nonendocrine complications, hospital charges, and length of stay. A multivariate model controlling for variables analyzed was constructed to examine significant independent risk factors. RESULTS Between 2000 and 2011, 1961 pediatric patients were identified who underwent a transcranial (71.2%) or a transsphenoidal (28.8%) craniotomy for resection of a craniopharyngioma. A major predilection for age was observed with the selection of a transcranial (23.4% in < 7-year-olds, 28.1% in 7- to 12-year-olds, and 19.7% in 13- to 18-year-olds) versus transphenoidal (2.9% in < 7-year-olds, 7.4% in 7- to 12-year-olds, and 18.4% in 13- to 18-year-olds) approach. No significant outcomes were associated with a particular surgical approach, except that 7- to 12-year-old patients had a higher risk of nonendocrine complications (relative risk [RR] 2.42, 95% CI 1.04-5.65, p = 0.04) with the transsphenoidal approach when compared with 13- to 18-year-old patients. The overall inpatient mortality rate was 0.5% and the most common postoperative complication was diabetes insipidus (64.2%). There were no independent factors associated with inpatient mortality rates and no significant differences in outcomes among groups based on sex and race. The average length of stay was 11.8 days, and the mean hospital charge was $116,5 22. Hospitals with medium and large bed capacity were protective against nonendocrine complications (RR 0.53, 95% CI 0.3-0.93, p = 0.03 [medium]; RR 0.45, 95% CI 0.25-0.8, p < 0.01 [large]) and total complications (RR 0.73, 95% CI 0.55-0.97, p = 0.03 [medium]; RR 0.68, 95% CI 0.51-0.9, p < 0.01 [large]) when compared with hospitals with small bed capacity (< 200 beds). Patients admitted to rural hospitals had an increased risk for nonendocrine complications (RR 2.56, 95% CI 1.11-5.9, p = 0.03). The presence of one or more medical comorbidities increased the risk of higher total complications (RR 1.38, 95% CI 1.14-1.68), p < 0.01 [1 comorbidity]; RR 2.37, 95% CI 1.98-2.84, p < 0.01 [≥ 2 comorbidities]) and higher total hospital charges (RR 2.9, 95% CI 1.08-7.81, p = 0.04 [1 comorbidity]; RR 9.1, 95% CI 3.74-22.12, p < 0.01 [≥ 2 comorbidities]). CONCLUSIONS This analysis identified patient age, comorbidities, insurance type, hospital bed capacity, and rural or nonteaching hospital status as independent risk factors for postoperative complications and/or increased hospital charges in pediatric patients with craniopharyngioma. Transsphenoidal surgery in younger patients with craniopharyngioma was a risk factor for nonendocrine complications.

Moussazadeh N, Prabhu V, Bander ED, et al.
Endoscopic endonasal versus open transcranial resection of craniopharyngiomas: a case-matched single-institution analysis.
Neurosurg Focus. 2016; 41(6):E7 [PubMed] Related Publications
OBJECTIVE The authors compared clinical and radiological outcomes after resection of midline craniopharyngiomas via an endoscopic endonasal approach (EEA) versus an open transcranial approach (TCA) at a single institution in a series in which the tumors were selected to be equally amenable to gross-total resection (GTR) with either approach. METHODS A single-institution retrospective review of previously untreated adult midline craniopharyngiomas was performed. Lesions were evaluated by 4 neurosurgeons blinded to the actual approach used to identify cases that were equally amenable to GTR using either an EEA or TCA. Radiological and clinical outcome data were assessed. RESULTS Twenty-six cases amenable to either approach were identified, 21 EEA and 5 TCA. Cases involving tumors that were resected via a TCA had a trend toward larger diameter (p = 0.10) but were otherwise equivalent in preoperative clinical and radiological characteristics. GTR was achieved in a greater proportion of cases removed with an EEA than a TCA (90% vs 40%, respectively; p = 0.009). Endoscopic resection was associated with superior visual restoration (63% vs 0%; p < 0.05), a decreased incidence of recurrence (p < 0.001), lower increase in FLAIR signal postoperatively (-0.16 ± 4.6 cm(3) vs 14.4 ± 14.0 cm(3); p < 0.001), and fewer complications (20% vs 80% of patients; p < 0.001). Significantly more TCA patients suffered postoperative cognitive loss (80% vs 0; p < 0.0001). CONCLUSIONS An EEA is a safe and effective approach to suprasellar craniopharyngiomas amenable to GTR. For this select group of cases, the EEA may provide higher rates of GTR and visual improvement with fewer complications compared with a TCA.

Morisako H, Goto T, Goto H, et al.
Aggressive surgery based on an anatomical subclassification of craniopharyngiomas.
Neurosurg Focus. 2016; 41(6):E10 [PubMed] Related Publications
OBJECTIVE Craniopharyngiomas remain a particularly formidable challenge in the neurosurgical field. Because these lesions involve the hypothalamus and ophthalmological systems, their resection is associated with either higher rates of mortality and recurrence or a lower rate of radical resection. The authors report the outcomes of aggressive surgeries based on an anatomical subclassification of craniopharyngiomas. METHODS Clinical and ophthalmological examinations, imaging studies, endocrinological studies, neuropsychological function, and surgical complications in all patients who had undergone microsurgical resection for craniopharyngioma at Osaka City University hospital between January 2000 and December 2014 were retrospectively reviewed through the medical records. Radical resections were planned in all of the patients. To help choose the correct surgical approach, craniopharyngiomas were classified based on tumor origin. The 4 possible groups included the intrasellar type, prechiasmatic type, retrochiasmatic type, and intra-third ventricle type. A multistage surgery was planned in some cases. RESULTS Seventy-two cases of craniopharyngioma were resected. Thirty-two patients (44.4%) had undergone previous surgical procedures at other institutions. Thirty-five cases (48.6%) were classified as retrochiasmatic, 19 (26.4%) as prechiasmatic, 12 (16.7%) as intra-third ventricle, and 6 (8.3%) as intrasellar. In 26 cases (36.1%), multistage surgery was required to complete the radical resection. Overall, 41 cases involved an orbitozygomatic approach; 21, a transpetrosal approach; 21, an interhemispheric approach; and 14, a transsphenoidal approach. In 3 cases, other approaches were applied. Gross-total resection was achieved in 43 patients (59.7%), near-total resection in 28 (38.9%), and partial resection in only 1 patient (1.4%). The mean follow-up period after resection was 4.7 years. Tumor recurrence or regrowth occurred in 15 (20.8%) of the 72 patients, with 14 of the 15 cases successfully controlled after additional resections and stereotactic radiosurgery. However, 1 patient died of uncontrollable tumor progression, and 2 patients died of unrelated diseases during the follow-up. Overall, disease in 69 (95.8%) of 72 patients was well controlled at the last follow-up. CONCLUSIONS Aggressive tumor resection is the authors' treatment policy for craniopharyngioma. Using an anatomical subclassification of craniopharyngioma to choose the most appropriate surgical approach is helpful in achieving that goal of aggressive resection.

Lamiman K, Wong KK, Tamrazi B, et al.
A quantitative analysis of craniopharyngioma cyst expansion during and after radiation therapy and surgical implications.
Neurosurg Focus. 2016; 41(6):E15 [PubMed] Related Publications
OBJECTIVE When complete resection of craniopharyngioma is not achievable or the sequelae are prohibitive, limited surgery and radiation therapy have demonstrated excellent local disease control while minimizing treatment-related sequelae. When residual tissue exists, there is a propensity for further cyst development and expansion during and after radiation therapy. This can result in obstructive hydrocephalus, visual changes, and/or clinical decline. The authors present a quantitative analysis of cyst expansion during and after radiotherapy and examine how it affected subsequent management. METHODS The authors performed an institutional review board-approved retrospective study of patients with histologically confirmed craniopharyngioma treated between 2000 and 2015 with surgery and intensity-modulated radiation therapy (IMRT) at a single institution. Volumetric measurements of cyst contours were generated by radiation oncology treatment planning software postoperatively, during IMRT, and up to 12 months after IMRT. Patient, tumor, and treatment-related variables were collected until the last known follow-up and were analyzed. RESULTS Twenty-seven patients underwent surgery and IMRT. The median total radiation dose was 54 Gy. Of the 27 patients, 11 patients (40.7%) demonstrated cyst expansions within 1 year of IMRT. Of note, all tumors with cyst expansion were radiographically Puget Grade 2. Maximal cyst expansion peaked at 4.27 months following radiation therapy, with a median volume growth of 4.1 cm(3) (mean 9.61 cm(3)) above the postoperative cyst volume. Eight patients experienced spontaneous cyst regression without therapeutic intervention. Three patients experienced MRI-confirmed cyst enlargement during IMRT, all of whom required adaptive planning to ensure adequate coverage of the entire tumor volume. Two of these 3 patients required ventriculoperitoneal shunt placement and additional intervention. One underwent additional resection, and the other had placement of an intracystic catheter for aspiration and delivery of intracystic interferon within 12 months of completing IMRT. All 3 patients now have stable disease. CONCLUSIONS Craniopharyngioma cyst expansion occurred in approximately 40% of the patients during or after radiotherapy. In the majority of patients, cyst expansion was a self-limiting process and did not confer a worse outcome. During radiotherapy, cyst expansion may be apparent on image-guided radiation therapy. Adaptive IMRT planning may be required to ensure that the intended IMRT dose covers the entire tumor and cyst volume. The sequelae of cyst expansion include progressive hydrocephalus, which may be treated with a shunt. For patients with solitary cyst expansion, cyst aspiration and/or intracystic interferon may result in disease control.

Liu JK, Sevak IA, Carmel PW, Eloy JA
Microscopic versus endoscopic approaches for craniopharyngiomas: choosing the optimal surgical corridor for maximizing extent of resection and complication avoidance using a personalized, tailored approach.
Neurosurg Focus. 2016; 41(6):E5 [PubMed] Related Publications
Resection remains the mainstay of treatment for craniopharyngiomas with the goal of radical resection, if safely possible, to minimize the rate of recurrence. Endoscopic endonasal and microscopic transcranial surgical approaches have both become standard methods for the treatment for craniopharyngiomas. However, the approach selection paradigm for craniopharyngiomas is still a point of discussion. Choosing the optimal surgical approach can play a significant role in maximizing the extent of resection and surgical outcome while minimizing the risks of potential complications. Craniopharyngiomas can present with a variety of different sizes, locations, and tumor consistencies, and each individual tumor has distinct features that favor one specific approach over another. The authors review standard cranial base techniques applied to craniopharyngioma surgery, using both the endoscopic endonasal approach and traditional open microsurgical approaches, and analyze factors involved in approach selection. They discuss their philosophy of approach selection based on the location and extent of the tumor on preoperative imaging as well as the advantages and limitations of each surgical corridor, and they describe the operative nuances of each technique, using a personalized, tailored approach to the individual patient with illustrative cases and videos.

Wang L, Shi X, Liu F, Qian H
Bypass surgery to treat symptomatic fusiform dilation of the internal carotid artery following craniopharyngioma resection: report of 2 cases.
Neurosurg Focus. 2016; 41(6):E17 [PubMed] Related Publications
Fusiform dilation of the internal carotid artery (FDICA) is an infrequent vascular complication following resection of suprasellar lesions in the pediatric population, and its course appears to be benign without apparent clinical symptoms. However, data correlating symptomatic FDICA with bypass surgery are scarce. The authors here report 2 symptomatic cases that were treated using internal maxillary artery bypass more than 5 years after total removal of a craniopharyngioma at an outside institution. Both cases of FDICA were resected to relieve the mass effect and to expose the craniopharyngioma. The postoperative course was uneventful, and radiological imaging revealed graft conduit patency. To the authors' knowledge, this is the first reported use of extracranial to intracranial bypass to treat FDICA following removal of a suprasellar lesion. Their findings suggest that bypass surgery is a useful therapeutic approach for symptomatic cases of FDICA and total removal of recurrent craniopharyngioma. Moreover, the indications for surgical intervention and treatment modalities are discussed in the context of previous relevant cases.

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