Pituitary Tumors
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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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Information Patients and the Public (12 links)


Information for Health Professionals / Researchers (7 links)

Latest Research Publications

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

Batisse M, Raverot G, Maqdasy S, et al.
Aggressive silent GH pituitary tumor resistant to multiple treatments, including temozolomide.
Cancer Invest. 2013; 31(3):190-6 [PubMed]
Temozolomide (TMZ) has been proposed as a therapeutic option in aggressive pituitary tumors. Among the published cases, GH expressing tumors were rare. We describe a patient with initially benign silent GH adenoma that transformed into an aggressive GH secreting tumor resistant to usual therapy. MGMT expression was high and the MGMT promoter was unmethylated. Before this aggressive course, patient received three cycles of TMZ; no response was observed. Four cases of GH aggressive tumor treated by TMZ have been reported. Response to TMZ was observed in one of these four patients. Predictive factors of failure of TMZ remain unclear.


Liu X, Ma S, Dai C, et al.
Antiproliferative, antiinvasive, and proapoptotic activity of folate receptor α-targeted liposomal doxorubicin in nonfunctional pituitary adenoma cells.
Endocrinology. 2013; 154(4):1414-23 [PubMed]
There is an urgent need for novel therapeutic strategies for the treatment of nonfunctional pituitary adenomas (NFPAs), especially those that are invasive. The folate receptor (FR)α is overexpressed in several cancers, including NFPA. The aim of this study was to determine the efficacy of FRα-targeted liposomes loaded with doxorubicin (F-L-DOX) in the treatment of NFPA. We evaluated targeting, cytotoxicity, antiinvasive, and proapoptotic activity of F-L-DOX in 25 primary cell lines derived from patients with NFPAs. We found that these liposomes effectively targeted NFPA cells through FRα and that endocytosis of the liposomes was blocked by 1mM free folic acid. F-L-DOX inhibited proliferation of NFPA cells and promoted apoptosis through activation of caspase-8, caspase-9, and caspase-3/7 more effectively than L-DOX. Furthermore, F-L-DOX also exerted greater antiinvasive ability in NFPA cells than L-DOX through suppression of the secretion of matrix metalloproteinase-2 and matrix metalloproteinase-9. Addition of 1mM free folic acid significantly reduced the pleotropic effects of F-L-DOX in NFPA cells, suggesting that FRα plays a critical role in mediating the antitumor effect of F-L-DOX. Our findings warrant further investigation of F-L-DOX as an alternative therapeutic strategy for the treatment of NFPAs that express FRα.


Zieliński G, Witek P, Koziarski A, Podgórski J
Spontaneous regression of non-functioning pituitary adenoma due to pituitary apoplexy following anticoagulation treatment - a case report and review of the literature.
Endokrynol Pol. 2013; 64(1):54-8 [PubMed]
Pituitary apoplexy (PA) is a rare, potentially life-threatening medical condition due to acute ischaemia or haemorrhage of the pituitary gland. The main clinical features are: abrupt onset of severe headache, nausea, vomiting, deteriorating level of consciousness, visual impairment and/or endocrine deficiency. Correct and prompt diagnosis is essential for effective therapy, but there are no randomised studies or strict recommendations defining treatment modalities. We present the case of a 59 year-old woman with pituitary tumour apoplexy, presenting with severe headache, vomiting and visual field deterioration. The patient was treated conservatively because of her refusal of surgery and was followed-up for five years. In the course of treatment, recovery of the visual field defects, as well as right sixth cranial nerve paresis, was observed. Repeated magnetic resonance imaging (MRI) revealed regression of the tumour without signs of its re-growth. We discuss therapeutic modalities with particular emphasis on morbidity and review the literature relating to the management of pituitary tumour apoplexy.


Chentli F, Bey A, Belhimer F, Azzoug S
Spontaneous resolution of pituitary apoplexy in a giant boy under 10 years old.
J Pediatr Endocrinol Metab. 2012; 25(11-12):1177-9 [PubMed]
AIM: Pituitary gigantism is a very rare condition; the occurrence of pituitary apoplexy in children younger than 10 years old is even rarer. The aim of our study is to report this exceptional association.
OBSERVATION: A boy aged 9 years and 6 months was hospitalized for the first time in November 2011 for symptoms suggesting pituitary apoplexy. The onset of his disease was difficult to determine as his health record has been poorly maintained. On October 10, 2011, he presented to an emergency department with a sudden drop of visual acuity with diplopia and retro-orbital headaches. An ophthalmological exam found very low visual acuity (1/20) with papillary edema. An MRI of the patient's brain revealed a hemorrhagic pituitary process reaching the chiasma, which was compressed, especially on the right side. Thereafter, the patient's vision improved spontaneously. Clinical examination was normal except for gigantism (+5 SD compared to the target stature). Hormonal assessment argued for mixed secretion [growth hormone (GH) = 39 ng/mL, n ≤ 5, prolactin ( PRL) = 470 ng/mL, n < 15]. Other pituitary functions were normal. Visual acuity normalized after 2 months, and an MRI showed a spontaneous reduction of the pituitary tumor.
CONCLUSION: This unusual observation is a model of symptomatic pituitary apoplexy with spontaneous resolution in a boy with pituitary gigantism: phenomenon quite exceptional and worth to be reported.


Kwon AR, Ann JM, Shin JI, et al.
Hypodipsic hypernatremia leading to reversible renal failure following surgery for craniopharyngioma.
J Pediatr Endocrinol Metab. 2012; 25(9-10):1027-30 [PubMed]
Thirst is stimulated by increases in effective plasma osmolality that are detected by cerebral osmoreceptors located in the vascular organ of the lamina terminalis. However, surgical destruction or organic lesions of the lamina terminalis decrease the sensation of thirst in response to increased plasma osmolality. A 17-year-old boy who was diagnosed with craniopharyngioma at the age of 10 years and underwent tumor resection and gamma knife surgery was admitted for non-symptomatic severe hypernatremia. Although the sodium level was 173 mmol/L and serum osmolality was also high (371 mOsm/kg), the patient did not report increased thirst. Laboratory analysis revealed hypertonic dehydration and acute non-oliguric renal failure due to dehydration. Treatment was based on correction of hypernatremia with hydration and education about regular, periodic water ingestion. The patient's hypernatremia and acute non-oliguric renal failure resolved with controlled daily fluid intake. To our knowledge, this is the first report of decreased thirst sensation secondary to craniopharyngioma and tumor resection leading to severe hypernatremia and non-oliguric renal failure in an adolescent.


Lambert JK, Goldberg L, Fayngold S, et al.
Predictors of mortality and long-term outcomes in treated Cushing's disease: a study of 346 patients.
J Clin Endocrinol Metab. 2013; 98(3):1022-30 [PubMed] Article available free on PMC after 01/03/2014
CONTEXT: Active Cushing's disease (CD) confers a 4-fold increase in mortality and is associated with significant morbidities. Although excess mortality risk may persist even after CD treatment, predictors of risk in treated CD are not well understood.
OBJECTIVE: To identify predictors of mortality, cardiovascular (CV) disease, and recurrence after long-term follow-up among patients with treated CD.
DESIGN, SETTING, AND PATIENTS: A retrospective chart review was conducted to evaluate patients with CD who underwent transsphenoidal adenectomy with a single surgeon.
OUTCOME MEASURES: Patients were categorized based on disease response after initial treatment. Cox proportional hazard models identified predictors of mortality, recurrence, and CV outcomes in the overall cohort and each subgroup.
RESULTS: Three hundred forty-six subjects were included. Mean age was 39.9 years, and mean duration of follow-up was 6.3 years (range, 1 mo to 30 y). Duration of exposure to excess glucocorticoids, estimated by duration of symptoms before diagnosis until remission was achieved by any means, was 40.0 months. Multivariate analyses demonstrated that duration of glucocorticoid exposure elevated the risk of death (P = .038), as did older age at diagnosis (P = 0.0001) and preoperative ACTH concentration (P = .007). Among patients who achieved remission, depression increased the hazard of death (P < .01). Male sex, age at diagnosis, diabetes, and depression elevated the risk of CV disease (P < .05).
CONCLUSION: Long-term follow-up of a large cohort of treated patients with CD identified several novel predictors of mortality. These data illustrate the importance of early recognition and treatment of CD. Long-term follow-up, with management of persistent comorbidities, is needed even after successful treatment of CD.


Sun L, Gao Y, Fu C, et al.
Neuronavigation used for the transsphenoidal resection of a pituitary adenoma accompanied by a concha sphenoid sinus.
Neuro Endocrinol Lett. 2012; 33(8):765-8 [PubMed]
A concha non-pneumatized sphenoid is considered to be a contraindication for the transsphenoidal resection of a pituitary adenoma. Specifically, this anatomical variation makes it difficult to approach the sella turcica. However, in this report, an intra-operative navigational system was used as a guide to access the sella through the sphenoid sinus. This procedure was found to be both reasonable and safe.


Dai C, Zhang B, Liu X, et al.
Inhibition of PI3K/AKT/mTOR pathway enhances temozolomide-induced cytotoxicity in pituitary adenoma cell lines in vitro and xenografted pituitary adenoma in female nude mice.
Endocrinology. 2013; 154(3):1247-59 [PubMed]
Invasive pituitary adenomas (PAs) are often refractory to standard therapy and salvage treatment with temozolomide (TMZ). Hyperactivation of the phosphoinositide 3-kinase (PI3K)/AKT/mammalian target of rapamycin (mTOR) pathway contributes to chemotherapy resistance in many cancers. XL765, a novel dual-PI3K/mTOR inhibitor, has recently shown its efficacy as a monotherapy and in combination with conventional therapeutics in many cancers. The hyperactive PI3K/AKT/mTOR pathway frequently occurs in invasive PAs. In this study, we investigated whether XL765 sensitizes PA cells to TMZ in vitro and in vivo. Experiments were carried out to evaluate the effect of XL765 and TMZ alone or in combination on cell proliferation and apoptosis of PA cell lines (αT3-1, GH3, and MMQ) in vitro as well as the tumor growth and serum GH and prolactin secretions in a GH3 xenograft tumor model of female nude mice. XL765 and TMZ synergistically inhibited the growth of PA cell lines and induced apoptosis. Combination of XL765 and TMZ synergistically inhibited tumor growth, decreased serum GH and prolactin levels, and reduced the sacrifice rate of GH3 xenograft tumor models without increased systemic side effects. In addition, XL765 in combination with TMZ dramatically decreased phosphorylation of AKT and mTOR as well as the expression of Bcl-2. The increased expression of cleaved poly (ADP-ribose) polymerase and Bcl-2-associated X protein along with elevated caspase-3/7 activity were also observed in the combination group. Therefore, dual inhibitors of PI3K and mTOR may enhance alkylating agent-mediated cytotoxicity and provide a novel regimen in the treatment of invasive PAs.


Lonser RR, Wind JJ, Nieman LK, et al.
Outcome of surgical treatment of 200 children with Cushing's disease.
J Clin Endocrinol Metab. 2013; 98(3):892-901 [PubMed] Article available free on PMC after 01/03/2014
CONTEXT: Factors influencing the outcome of surgical treatment of pediatric Cushing's disease (CD) have not been fully established.
OBJECTIVE: The aim of this study was to examine features influencing the outcome of surgery for pediatric CD.
DESIGN: In this prospective observational study, the clinical, imaging, endocrinological, and operative outcomes were analyzed in consecutive patients treated at the National Institutes of Health (NIH) from 1982 through 2010.
SETTING: The study was conducted in a tertiary referral center.
RESULTS: Two hundred CD patients (106 females, 94 males) were included. Mean age at symptom development was 10.6 ± 3.6 years (range, 4.0 to 19.0 y). Mean age at NIH operation was 13.7 ± 3.7 years. Twenty-seven patients (13%) had prior surgery at another institution. Magnetic resonance imaging identified adenomas in 97 patients (50%). When positive, magnetic resonance imaging accurately defined a discrete adenoma in 96 of the 97 patients (99%), which was more accurate than the use of ACTH ratios during inferior petrosal sinus sampling to determine adenoma lateralization (accurate in 72% of patients without prior surgery). A total of 195 of the 200 patients (98%) achieved remission after surgery (189 [97%] were hypocortisolemic; 6 [3%] were eucortisolemic postoperatively). Factors associated with initial remission (P < .05) included identification of an adenoma at surgery, immunohistochemical ACTH-producing adenoma, and noninvasive ACTH adenoma. Younger age, smaller adenoma, and absence of cavernous sinus wall or other dural invasion were associated with long-term remission (P < .05). A minimum morning serum cortisol of less than 1 μg/dl after surgery had a positive predictive value for lasting remission of 96%.
CONCLUSIONS: With rare disorders, such as pediatric CD, enhanced outcomes are obtained by evaluation and treatment at centers with substantial experience. Resection of pituitary adenomas in pediatric CD in that setting can be safe, effective, and durable. Early postoperative endocrine testing predicts lasting remission. Because lasting remission is associated with younger age at surgery, smaller adenomas, and lack of dural invasion, early diagnosis should improve surgical outcome.


Hirohata T, Asano K, Ogawa Y, et al.
DNA mismatch repair protein (MSH6) correlated with the responses of atypical pituitary adenomas and pituitary carcinomas to temozolomide: the national cooperative study by the Japan Society for Hypothalamic and Pituitary Tumors.
J Clin Endocrinol Metab. 2013; 98(3):1130-6 [PubMed]
Context: Temozolomide (TMZ) is an alkylating agent and was a first-line chemotherapeutic agent for malignant gliomas. Recently, TMZ has been documented to be effective against atypical pituitary adenomas (APAs) and pituitary carcinomas (PCs). Objective: The clinical and pathological characteristics of APAs and PCs treated with TMZ in Japan were surveyed and analyzed retrospectively. Design: Members of the Japan Society of Hypothalamic and Pituitary Tumors were surveyed regarding the clinical characteristics of APAs and PCs treated with TMZ. Stored tumor samples were gathered from the responders and were assessed by the immunohistochemistry of Ki-67, O(6)-methyl-guanine-DNA methyltransferase, p53, MSH6, and anterior pituitary hormones. Responses to TMZ treatment were defined as complete response (CR), partial response (PR), progressive disease (PD), and stable disease (SD) according to RECIST (Response Evaluation Criteria in Solid Tumors) version 2.0. Subjects: Three samples from 3 subjects with APA and 11 samples from 10 subjects with PC were available. Results: The 13 subjects had APAs and PCs consisting of 5 prolactin-producing tumors, 5 ACTH-producing tumors, and 3 null cell adenomas. The clinical response to TMZ treatment was as follows: 4 cases of CR and PR (31%), 2 cases of SD (15%), 6 cases of recurrence after CR and PR (46%), and 1 case of PD (8%). However, considerable subjects had recurrent disease after a response to TMZ. The immunohistochemical findings of Ki-67, O(6)-methyl-guanine-DNA methyltransferase, and p53 did not show any significant correlation with the efficacy of TMZ. However, the immunopositivity of MSH6 was positively correlated with TMZ response (P = .015, Fisher's exact test). Conclusions: This study showed that preserving MSH6 function was contributory to the effectiveness of TMZ in malignant pituitary neoplasms. It is necessary to survey more cases and evaluate multifactor analyses.


Neidert MC, Sze L, Zwimpfer C, et al.
Soluble α-klotho: a novel serum biomarker for the activity of GH-producing pituitary adenomas.
Eur J Endocrinol. 2013; 168(4):575-83 [PubMed]
OBJECTIVE: Klotho is a lifespan-influencing gene expressed mainly in the kidneys. Soluble α-Klotho (αKL) is released into the circulation. In this study, we present baseline αKL serum levels of patients with acromegaly compared with controls with other pituitary adenomas and assess changes following transsphenoidal surgery.
DESIGN: Prospective controlled study.
METHODS: We measured soluble αKL (sandwich ELISA) and IGF1 (RIA) in sera of 14 patients (eight females and six males) with active acromegaly and in 22 control patients (13 females and nine males) operated for non-GH-producing pituitary adenomas. Immunohistochemical staining for Klotho was performed in resected adenomas and in normal pituitary tissue samples.
RESULTS: Soluble αKL was high in the acromegaly group preoperatively (median 4217 pg/ml, interquartile range (IQR) 1812-6623 pg/ml) and declined after surgery during early follow-up (2-6 days; median 645 pg/ml, IQR 550-1303 pg/ml) (P<0.001) and during late follow-up (2-3 months post-operatively; median 902 pg/ml, IQR 497-1340 pg/ml; P<0.001). In controls, preoperative soluble αKL was significantly lower than in acromegalics, 532 pg/ml (400-677 pg/ml; P<0.001). Following surgery, soluble αKL remained low during early and late follow-up - changes over time within the control group were not statistically significant. These results were independent of age, sex and kidney function. Klotho staining was equal or slightly decreased in GH-positive adenomas compared with controls.
CONCLUSION: High soluble αKL serum levels were specific to GH-producing adenomas and decreased rapidly following adenoma removal. Thus, soluble αKL appears to be a new specific and sensitive biomarker reflecting disease activity in acromegaly. Similar Klotho staining patterns in controls and acromegalics suggest that the rise in serum αKL is caused by systemic actions of pituitary GH rather than due to increased expression of Klotho by the pituitary (adenoma).


Jiang XB, He DS, Mao ZG, et al.
BMI, apolipoprotein B/apolipoprotein A-I ratio, and insulin resistance in patients with prolactinomas: a pilot study in a Chinese cohort.
Tumour Biol. 2013; 34(2):1171-6 [PubMed]
Deranged metabolic profiles and insulin resistance (IR) have been documented in patients with prolactinomas. Few data are yet available on the apolipoprotein (apo) B/apoA-I ratio and its relationship with IR in patients with prolactinomas. This study was aimed to evaluate the level of apoB/apoA-I ratio and its association with IR in a Chinese subgroup with prolactinomas. Twenty-three prolactinoma patients and 20 healthy controls were enrolled in this study. The clinical anthropometric parameters and laboratory evaluation were collected. Insulin sensitivity was estimated using homeostatic model assessment [homeostasis model assessment of insulin resistance (HOMA-IR)]. Waist circumference and body weight index (BMI) were significantly higher in patients with prolactinomas than those in the controls (p < 0.05). Meanwhile, the prevalence of general and abdominal obesity seemed more pronounced in male patients compared to that in healthy subjects (57.14 vs. 0 % and 71.43 vs. 16.7 %, respectively). Furthermore, fasting glucose, insulin, HOMA-IR, triglyceride, and apoB/apoA-I ratio were also significantly higher in prolactinoma patients, but with lower level of apoA-I (p < 0.05). Univariate regression analysis revealed that prolactin, waist circumference, BMI, and presence of hypogonadism were significantly associated with IR (p < 0.05). However, only BMI [odds ratio (OR) = 1.937, 95 % confidence interval (CI) 1.112-3.375, p = 0.02] and prolactin (OR = 5.173, 95 % CI 1.073-24.94, p = 0.041) were shown to be independent predictors for the presence of IR in multivariate logistic analysis. This study confirmed the altered metabolic profile, including body weight gain, IR, disordered lipids, and apolipoproteins in prolactinoma patients. Prolactin and BMI were independently associated with IR. The effect of apoB/apoA-I ratio on IR is warranted to be determined in further studies.


Chentli F, Belhimer F, Kessaci F, Mansouri B
Congenital craniopharyngioma: a case report and literature review.
J Pediatr Endocrinol Metab. 2012; 25(11-12):1181-3 [PubMed]
INTRODUCTION: Although craniopharyngiomas are congenital tumors, they are rarely diagnosed in the prenatal or early neonatal period. Sometimes they are very large, debilitating and life-threatening, as in the case discussed here.
OBSERVATION: A 21-month-old girl was referred for craniopharyngioma that had been diagnosed at 5 months because of neurologic complications. The pregnancy and birth were considered to be unremarkable. The birth weight was 4 kg, length 53 cm, head circumference 38 cm (n=35±1). Brain CT revealed active hydrocephalus caused by a very large solid, cystic and calcified supra sellar mass. MRI confirmed the process measuring 5 cm in height, multidirectional, diving into pituitary sella, displacing the brainstem. After several resections and reservoir drainage, the patient was blind, hemiparetic and she convulsed frequently. The patient died when she was 2.
CONCLUSION: The large head circumference at birth, the size of the tumor, blindness, and hemiparesis observed before the age of 5 months suggested an antenatal process unnoticed because of inadequate gestational monitoring.


Catli G, Abaci A, Altincik A, et al.
Hyperprolactinemia in children: clinical features and long-term results.
J Pediatr Endocrinol Metab. 2012; 25(11-12):1123-8 [PubMed]
Hyperprolactinemia is a rare endocrine disorder in childhood, which may result from hypophyseal adenoma. We aimed to review the etiologic reasons and clinical features in hyperprolactinemia patients retrospectively. The mean age of 11 female patients at diagnosis was 14.2 ± 1.3 years. Five patients had microadenoma, four patients had macroadenoma, and two patients were diagnosed with idiopathic hyperprolactinemia. The most frequent symptoms were menstrual disorders, headache, and galactorrhea, and one-third of the patients had obesity at diagnosis. There was no anterior pituitary hormone deficiency. All patients received bromocriptine as initial therapy; only two patients with macroadenoma and one patient with microadenoma were switched to cabergoline. Transsphenoidal surgery was performed for a patient with macroadenoma, who had cavernous sinus invasion and visual field defect. Medical treatment should be the first-line treatment option in both microadenoma and macroadenoma cases without any neurological signs. Surgery should be employed with limited indications.


Tamban CA, Sandoval MA, Lantion-Ang FL
Where is the culprit? A case of acromegaly that defied the management algorithm.
BMJ Case Rep. 2013; 2013 [PubMed]
A 30-year-old Filipino man presented with a 11-year history of coarse facial features and progressive enlargement of hands and feet. Initial work-up revealed elevated insulin-like growth factor-1 and non-suppressible growth hormone level after 75 g glucose challenge test. Initial cranial MRI performed in the year 2010 showed absence of pituitary adenoma. The patient was lost to follow-up. He again consulted in the year 2011 and a repeat cranial MRI and a dedicated pituitary MRI were performed and both did not reveal any pituitary mass. Further investigation included chest and abdominal CT scan, both of which did not show any neoplasm. At present, there has been no practice guideline on the management of acromegalic patients on whom the identifiable source cannot be found. The patient was given the option to undergo surgical exploration of the pituitary gland or medical treatment with somatostatin analogues. He decided to undergo surgery but has not given consent for the procedure.


Cuny T, Pertuit M, Sahnoun-Fathallah M, et al.
Genetic analysis in young patients with sporadic pituitary macroadenomas: besides AIP don't forget MEN1 genetic analysis.
Eur J Endocrinol. 2013; 168(4):533-41 [PubMed]
CONTEXT: Germline mutations in the aryl hydrocarbon receptor interacting protein gene (AIP) have been identified in young patients (age ≤30 years old) with sporadic pituitary macroadenomas. Otherwise, there are few data concerning the prevalence of multiple endocrine neoplasia type 1 (MEN1) mutations in such a population.
OBJECTIVE: We assessed the prevalence of both AIP and MEN1 genetic abnormalities (mutations and large gene deletions) in young patients (age ≤30 years old) diagnosed with sporadic and isolated macroadenoma, without hypercalcemia and/or MEN1-associated lesions.
DESIGN: The entire coding sequences of AIP and MEN1 were screened for mutations. In cases of negative sequencing screening, multiplex ligation-dependent probe amplification was performed for the detection of large genetic deletions.
PATIENTS AND SETTINGS: One hundred and seventy-four patients from endocrinology departments of 15 French University Hospital Centers were eligible for this study.
RESULTS: Twenty-one out of 174 (12%) patients had AIP (n=15, 8.6%) or MEN1 (n=6, 3.4%) mutations. In pediatric patients (age ≤18 years old), AIP/MEN1 mutation frequency reached nearly 22% (n=10/46). AIPmut and MEN1mut were identified in 8/79 (10.1%) and 1/79 (1.2%) somatotropinoma patients respectively; they each accounted for 4/74 (5.4%) prolactinoma (PRL) patients with mutations. Half of those patients (n=3/6) with gigantism displayed mutations in AIP. Interestingly, 4/12 (33%) patients with non-secreting adenomas bore either AIP or MEN1 mutations, whereas none of the eight corticotroph adenomas or the single thyrotropinoma case had mutations. No large gene deletions were observed in sequencing-negative patients.
CONCLUSION: Mutations in MEN1 can be of significance in young patients with sporadic isolated pituitary macroadenomas, particularly PRL, and together with AIP, we suggest genetic analysis of MEN1 in such a population.


Morcos RN, Kizy T
Gynecomastia: when is treatment indicated?
J Fam Pract. 2012; 61(12):719-25 [PubMed]
This algorithmic approach can simplify your clinical evaluation and help you decide whether intervention or watchful waiting is appropriate.


Ónnestam L, Berinder K, Burman P, et al.
National incidence and prevalence of TSH-secreting pituitary adenomas in Sweden.
J Clin Endocrinol Metab. 2013; 98(2):626-35 [PubMed]
CONTEXT: TSH-secreting pituitary adenomas (TSHomas) are rare. Epidemiological data are scant and there are no reports on national incidence.
OBJECTIVE: The objective of the study was to estimate the national Swedish incidence and prevalence of TSHomas.
DESIGN: This was an observational study. Setting: The study was conducted at tertiary referral centers.
PATIENTS: The Swedish Pituitary Registry and World Health Organization International Statistical Classification of Diseases and Related Health Problems coding at all university hospitals were used to identify patients diagnosed with TSHomas 1990-2010. The identified patients' medical records were studied until the latest follow-up [median 5.0 years (range < 1-20 years)].
MAIN OUTCOME MEASUREMENTS: Incidence, prevalence, demographics, tumor characteristics, treatment outcome, and thyroid hormone level at diagnosis were measured.
RESULTS: The age-standardized national incidence of 28 TSHoma patients was 0.15 per 1 million inhabitants per year, with an increasing incidence over time (0.05 per 1 million per year in 1990-1994 to 0.26 per 1 million per year in 2005-2009). The national prevalence in 2010 was 2.8 per 1 million inhabitants, in which 0.85 per 1 million had active disease. Most patients (n = 22) underwent pituitary surgery, 5 had radiotherapy, and 6 had somatostatin analogues. Eighteen patients were considered cured at the latest follow-up; 25% remained uncontrolled. Subjects treated for putative primary hyperthyroidism prior to diagnosis had TSH levels more than double those with intact thyroid at diagnosis (P = .013). The median time to diagnosis was longer for women than men (4 vs < 1 year, P = .026). More women than men were treated surgically (94.1% vs 54.5%, P = .022).
CONCLUSION: This is the first estimate of a national incidence of TSHoma. Additional epidemiological studies are needed to compare these results with other geographical areas. This study suggests an increased incidence of TSHomas, in agreement with reports on other pituitary adenomas.


Koutourousiou M, Gardner PA, Fernandez-Miranda JC, et al.
Endoscopic endonasal surgery for giant pituitary adenomas: advantages and limitations.
J Neurosurg. 2013; 118(3):621-31 [PubMed]
OBJECT: Giant pituitary adenomas (> 4 cm in maximum diameter) represent a significant surgical challenge. Endoscopic endonasal surgery (EES) has recently been introduced as a treatment option for these tumors. The authors present the results of EES for giant adenomas and analyze the advantages and limitations of this technique.
METHODS: The authors retrospectively reviewed the medical files and imaging studies of 54 patients with giant pituitary adenomas who underwent EES and studied the factors affecting surgical outcome.
RESULTS: Preoperative visual impairment was present in 45 patients (83%) and partial or complete pituitary deficiency in 28 cases (52%), and 7 patients (13%) presented with apoplexy. Near-total resection (> 90%) was achieved in 36 patients (66.7%). Vision was improved or normalized in 36 cases (80%) and worsened in 2 cases due to apoplexy of residual tumor. Significant factors that limited the degree of resection were a multilobular configuration of the adenoma (p = 0.002) and extension to the middle fossa (p = 0.045). Cavernous sinus invasion, tumor size, and intraventricular or posterior fossa extension did not influence the surgical outcome. Complications included apoplexy of residual adenoma (3.7%), permanent diabetes insipidus (9.6%), new pituitary insufficiency (16.7%), and CSF leak (16.7%, which was reduced to 7.4% in recent years). Fourteen patients underwent radiation therapy after EES for residual mass or, in a later stage, for recurrence, and 10 with functional pituitary adenomas received medical treatment. During a mean follow-up of 37.9 months (range 1-114 months), 7 patients were reoperated on for tumor recurrence. Three patients were lost to follow-up.
CONCLUSIONS: Endoscopic endonasal surgery provides effective initial management of giant pituitary adenomas with favorable results compared with traditional microscopic transsphenoidal and transcranial approaches.


Larkin S, Reddy R, Karavitaki N, et al.
Granulation pattern, but not GSP or GHR mutation, is associated with clinical characteristics in somatostatin-naive patients with somatotroph adenomas.
Eur J Endocrinol. 2013; 168(4):491-9 [PubMed]
OBJECTIVE: Somatotroph adenomas causing acromegaly are histologically classified into densely granulated (DG) and sparsely granulated (SG) subtypes with different morphology, clinical characteristics and treatment outcomes. Granulation pattern has been reported to co-segregate with a recurrent mutation at codon 49 in growth hormone receptor (GHR) and GSP oncogene. This study examines response to the octreotide suppression test (OST) in relation to granulation pattern and mutation in GHR and GSP.
DESIGN: This is a retrospective, single-centre study of 52 patients with pathologically confirmed somatotroph adenoma who were naïve to medical therapy presenting between January 2001 and October 2010.
METHODS: Clinical, radiological and hormonal data at diagnosis were recorded. GHR and GSP were genotyped, granulation pattern determined and response to the OST measured.
RESULTS: SG adenomas were larger (P=0.038), occurred in younger patients (P=0.029), were more common in females (P=0.026) and were more invasive (P<0.0001 and P=0.001), with diminished responses to the OST (P=0.007) compared with DG adenomas. GSP mutation was unrelated to granulation pattern but associated with smaller tumours (P=0.027), producing more GH (P=0.048) that responded better to the OST (P=0.022). Codon 49 of GHR was not mutated.
CONCLUSIONS: Adenoma histological phenotype, not genotype, corresponds to clinical and biochemical characteristics and response to the OST. SG adenomas constitute a clinically more unfavourable subtype but are not associated with GHR mutations in our series. Ascertainment of the adenoma subtype may become an important consideration in the management of acromegaly.


Merchant TE, Kun LE, Hua CH, et al.
Disease control after reduced volume conformal and intensity modulated radiation therapy for childhood craniopharyngioma.
Int J Radiat Oncol Biol Phys. 2013; 85(4):e187-92 [PubMed] Article available free on PMC after 15/03/2014
PURPOSE: To estimate the rate of disease control after conformal radiation therapy using reduced clinical target volume (CTV) margins and to determine factors that predict for tumor progression.
METHODS AND MATERIALS: Eighty-eight children (median age, 8.5 years; range, 3.2-17.6 years) received conformal or intensity modulated radiation therapy between 1998 and 2009. The study group included those prospectively treated from 1998 to 2003, using a 10-mm CTV, defined as the margin surrounding the solid and cystic tumor targeted to receive the prescription dose of 54 Gy. The CTV margin was subsequently reduced after 2003, yielding 2 groups of patients: those treated with a CTV margin greater than 5 mm (n=26) and those treated with a CTV margin less than or equal to 5 mm (n=62). Disease progression was estimated on the basis of additional variables including sex, race, extent of resection, tumor interventions, target volume margins, and frequency of weekly surveillance magnetic resonance (MR) imaging during radiation therapy. Median follow-up was 5 years.
RESULTS: There was no difference between progression-free survival rates based on CTV margins (>5 mm vs ≤5 mm) at 5 years (88.1% ± 6.3% vs 96.2% ± 4.4% [P=.6386]). There were no differences based on planning target volume (PTV) margins (or combined CTV plus PTV margins). The PTV was systematically reduced from 5 to 3 mm during the time period of the study. Factors predictive of superior progression-free survival included Caucasian race (P=.0175), no requirement for cerebrospinal fluid shunting (P=.0066), and number of surveillance imaging studies during treatment (P=.0216). Patients whose treatment protocol included a higher number of weekly surveillance MR imaging evaluations had a lower rate of tumor progression.
CONCLUSIONS: These results suggest that targeted volume reductions for radiation therapy using smaller margins are feasible and safe but require careful monitoring. We are currently investigating the differences in outcome based on host factors to explain the results.


Huang M, Yang W, Yu M, et al.
Retrieval of brain tumors with region-specific bag-of-visual-words representations in contrast-enhanced MRI images.
Comput Math Methods Med. 2012; 2012:280538 [PubMed] Article available free on PMC after 15/03/2014
A content-based image retrieval (CBIR) system is proposed for the retrieval of T1-weighted contrast-enhanced MRI (CE-MRI) images of brain tumors. In this CBIR system, spatial information in the bag-of-visual-words model and domain knowledge on the brain tumor images are considered for the representation of brain tumor images. A similarity metric is learned through a distance metric learning algorithm to reduce the gap between the visual features and the semantic concepts in an image. The learned similarity metric is then used to measure the similarity between two images and then retrieve the most similar images in the dataset when a query image is submitted to the CBIR system. The retrieval performance of the proposed method is evaluated on a brain CE-MRI dataset with three types of brain tumors (i.e., meningioma, glioma, and pituitary tumor). The experimental results demonstrate that the mean average precision values of the proposed method range from 90.4% to 91.5% for different views (transverse, coronal, and sagittal) with an average value of 91.0%.


McLaughlin N, Eisenberg AA, Cohan P, et al.
Value of endoscopy for maximizing tumor removal in endonasal transsphenoidal pituitary adenoma surgery.
J Neurosurg. 2013; 118(3):613-20 [PubMed]
OBJECT: Endoscopy as a visual aid (endoscope assisted) or as the sole visual method (fully endoscopic) is increasingly used in pituitary adenoma surgery. Authors of this study assessed the value of endoscopic visualization for finding and removing residual adenoma after initial microscopic removal.
METHODS: Consecutive patients who underwent endoscope-assisted microsurgical removal of pituitary adenoma were included in this study. The utility of the endoscope in finding and removing residual adenoma not visualized by the microscope was noted intraoperatively. After maximal tumor removal under microscopic visualization, surgeries were categorized as to whether additional tumor was removed via endoscopy. Tumor removal and remission rates were also noted. Patients undergoing fully endoscopic tumor removal during this same period were excluded from the study.
RESULTS: Over 3 years, 140 patients (41% women, mean age 50 years) underwent endoscope-assisted adenoma removal of 30 endocrine-active microadenomas and 110 macroadenomas (39 endocrine-active, 71 endocrine-inactive); 16% (23/140) of patients had prior surgery. After initial microscopic removal, endoscopy revealed residual tumor in 40% (56/140) of cases and the additional tumor was removed in 36% (50 cases) of these cases. Endoscopy facilitated additional tumor removal in 54% (36/67) of the adenomas measuring ≥ 2 cm in diameter and in 19% (14/73) of the adenomas smaller than 2 cm in diameter (p < 0.0001); additional tumor removal was achieved in 20% (6/30) of the microadenomas. Residual tumor was typically removed from the suprasellar extension and folds of the collapsed diaphragma sellae or along or within the medial cavernous sinus. Overall, 91% of endocrine-inactive tumors were gross-totally or near-totally removed, and 70% of endocrine-active adenomas had early remission.
CONCLUSIONS: After microscope-based tumor removal, endoscopic visualization led to additional adenoma removal in over one-third of patients. The panoramic visualization of the endoscope appears to facilitate more complete tumor removal than is possible with the microscope alone. These findings further emphasize the utility of endoscopic visualization in pituitary adenoma surgery. Longer follow-ups and additional case series are needed to determine if endoscopic adenomectomy translates into higher long-term remission rates.


Tabakow P, Czyz M, Jarmundowicz W, Lechowicz-Głogowska E
Surgical treatment of pituitary adenomas using low-field intraoperative magnetic resonance imaging.
Adv Clin Exp Med. 2012 Jul-Aug; 21(4):495-503 [PubMed]
BACKGROUND: Intraoperative magnetic resonance imaging (iMRI) is a new technique for imaging of the brain and is used with increasing frequency during neurosurgical operations, enabling the surgeon to make decisions based on real-time images.
OBJECTIVES: This paper presents the technique for the surgical treatment of pituitary adenomas using low-field iMRI, evaluates the safety of iMRI usage in pituitary surgery and examines the influence of iMRI on the extent of tumor removal.
MATERIAL AND METHODS: From October 2008 to December 2010, 18 patients were treated for pituitary adenomas using the low-field iMRI system Polestar N20. The procedures were conducted via the transsphenoidal approach, using the microscopic technique in 15 cases and endoscopically in three cases. The patients' mean age was 56 +/- 15 years; their mean American Society of Anesthesiologists (ASA) score was 2; 67% of them were male. Most of the patients were operated on for macroadenomas, 83% of which were hormonally inactive. The analysis concerned the technical aspects of iMRI usage, such as preparation and surgery time and the quality of the iMRI-scans performed. The safety of iMRI and its influence on decisions regarding further tumor resection.
RESULTS: The operations on pituitary adenomas using iMRI were safe. Only two hemorrhagic complications were noted, and they were not related to iMRI usage. The mean preparation and surgery times were 109 +/- 37 minutes and 238 +/- 188 minutes, respectively. The iMRI images of sella turcica were of satisfactory quality in 16 patients. In 50% of the cases, iMRI conducted when the surgeon believed that the desired extent of tumor resection had been attained showed that there were still tumor remnants to be resected. In 67% of these cases, continued tumor removal lead to achievement of the desired degree of resection.
CONCLUSIONS: Low-field iMRI-guided operations on pituitary tumors are safe and feasible, and they ensure an increased radicality of tumor resection.


Cackett P, Eunson G, Bath L, Mulvihill A
Proptosis as the presenting sign of giant prolactinoma in a prepubertal boy: successful resolution of hydrocephalus by use of medical therapy.
Future Oncol. 2012; 8(12):1621-6 [PubMed]
We report the case of a 13-year-old prepubertal boy who presented with a left-sided proptosis, bilateral papilloedema and hydrocephalus who was subsequently diagnosed with a giant prolactinoma invading the left orbit. He was commenced on dopamine receptor agonists in the form of quinagolide and cabergoline, and made an excellent response to medical therapy alone, with resolution of hydrocephalus, restoration of normal vision and a 98% reduction in serum prolactin. The rapid improvement achieved negated the requirement for surgery and this highlights the efficacy of the dopamine agonists in the management of giant prolactinomas, even in the presence of neurological symptoms.


El-Shehaby AM, Reda WA, Tawadros SR, Abdel Karim KM
Low-dose Gamma Knife surgery for nonfunctioning pituitary adenomas.
J Neurosurg. 2012; 117 Suppl:84-8 [PubMed]
OBJECT: The primary concern when performing Gamma Knife surgery for pituitary adenoma is preservation of vision and pituitary function while achieving tumor growth control. Higher prescribed radiation doses are typically correlated with higher incidences of postradiosurgical hormone deficiencies. The goal of the present study was to retrospectively analyze the feasibility of using a lower prescribed radiation dose in the treatment of nonfunctioning pituitary adenomas and the effect of this dose on vision, pituitary function, and tumor growth control.
METHODS: The study was conducted in 38 patients with nonfunctioning pituitary adenomas, who were treated between January 2002 and July 2008. Twenty-one patients were available for follow-up (13 men and 8 women). The mean follow-up period was 44 months (range 24-90 months). Nineteen patients had previously undergone surgery. Pituitary dysfunction developed after surgery in 3 patients. One patient had an abnormal pituitary hormone profile before radiosurgery due to an attack of pituitary apoplexy. Visual field defects were present in 12 patients. The prescribed radiation dose was 12 Gy in all patients. The tumor volume ranged from 0.5 to 11.8 cm(3) (mean 4.8 cm(3)). The maximum dose to the visual pathway was kept below 10 Gy. The mean maximum dose delivered to the visual pathway was 7.9 Gy.
RESULTS: The patients were followed up for a period of 24 to 90 months (mean 44 months). The size of the tumor decreased in 11 patients (52%) and remained stable in 9 patients (43%). In 1 patient there was tumor growth outside the previous radiation field (on the contralateral side). Among the 12 patients with visual field defects, 9 (75%) experienced an improvement and the remaining patients' vision remained stable. In only 4 patients was the visual improvement associated with tumor shrinkage. The hormone profile remained normal in all patients except for the 4 patients who had pituitary dysfunction before radiosurgery.
CONCLUSIONS: The 12-Gy prescribed dose used in this study seems to be sufficient for producing tumor control while sparing the patient from radiation-induced pituitary dysfunction. In addition, visual improvement was reported in a number of cases. A larger series and longer follow-up are required to confirm these results.


Wang SS, Zhang SM, Jing JJ
Stereoscopic virtual reality models for planning tumor resection in the sellar region.
BMC Neurol. 2012; 12:146 [PubMed] Article available free on PMC after 15/03/2014
BACKGROUND: It is difficult for neurosurgeons to perceive the complex three-dimensional anatomical relationships in the sellar region.
METHODS: To investigate the value of using a virtual reality system for planning resection of sellar region tumors. The study included 60 patients with sellar tumors. All patients underwent computed tomography angiography, MRI-T1W1, and contrast enhanced MRI-T1W1 image sequence scanning. The CT and MRI scanning data were collected and then imported into a Dextroscope imaging workstation, a virtual reality system that allows structures to be viewed stereoscopically. During preoperative assessment, typical images for each patient were chosen and printed out for use by the surgeons as references during surgery.
RESULTS: All sellar tumor models clearly displayed bone, the internal carotid artery, circle of Willis and its branches, the optic nerve and chiasm, ventricular system, tumor, brain, soft tissue and adjacent structures. Depending on the location of the tumors, we simulated the transmononasal sphenoid sinus approach, transpterional approach, and other approaches. Eleven surgeons who used virtual reality models completed a survey questionnaire. Nine of the participants said that the virtual reality images were superior to other images but that other images needed to be used in combination with the virtual reality images.
CONCLUSIONS: The three-dimensional virtual reality models were helpful for individualized planning of surgery in the sellar region. Virtual reality appears to be promising as a valuable tool for sellar region surgery in the future.


Sav A, Rotondo F, Syro LV, et al.
Biomarkers of pituitary neoplasms.
Anticancer Res. 2012; 32(11):4639-54 [PubMed]
In a wide spectrum of tumors, cell proliferation, vascularity, apoptosis, cell adhesion, and cell-cycle progression may indicate tumor progression. In this review article, the literature regarding apoptotic markers and p53, as well as cyclooxygenase-2, galectin-3, and pituitary tumor-transforming factor, proliferative markers, angiogenesis, including vascular endothelial growth factor and its receptor, pituitary tumor-transforming gene, microarrays, stem cells, and microenvironment and tumor heterogeneity are presented. Only a particular group of selected biomarkers show promise in differentiating pituitary tumors which will behave in an aggressive manner. Therefore, the most common and promising biomarkers and terms were analyzed, proposing the need for uniform design and application of methods and standardized criteria for the interpretation of results. The new spectrum of biomarkers may shed light upon the pathogenetic mechanisms and also may serve as standardized diagnostic tool for daily pathologic practice.


Badami KG, Chalmers I, Soule S, Walkden D
Difficult venepuncture--it was all in the head!
Transfus Med. 2012; 22(6):448-9 [PubMed]
AIM: To emphasise the importance of careful observations and demonstrate that significant conditions may, on occasion, be detected during blood donation.
BACKGROUND: Venepunctures, the bread and butter of blood donor services, can be difficult procedures. We describe an unusual presentation of a relatively rare disease detected during routine blood donations because of difficult venepunctures.
METHODS: Case report.
RESULTS: A growth hormone-secreting pituitary adenoma was diagnosed, and successfully treated, following the initial observation of hard-to-puncture skin during blood donations.
CONCLUSIONS: Seemingly trivial abnormalities may prove clinically important. One presentation of acromegaly is hard-to-puncture skin.


Mehta GU, Jane JA
Pituitary tumors.
Curr Opin Neurol. 2012; 25(6):751-5 [PubMed]
PURPOSE OF REVIEW: The aim of this article is to review current research in the treatment of pituitary tumors and summarize emerging medical, surgical and radiation-based therapies.
RECENT FINDINGS: Existing medical agents such as dopamine agonists and somatostatin ligand receptors are being used in novel combinations to achieve biochemical remission for pituitary tumors and reduce tumor size. Advancements have also been made in surgery to improve tumor localization by use of intraoperative imaging and to reduce morbidities, such as cerebrospinal fluid leak, associated with transsphenoidal surgery. Finally, as radiation therapy and stereotactic radiosurgery for pituitary tumors gains more widespread use, long-term data is becoming available demonstrating its durability.
SUMMARY: Although current therapeutics and treatment modalities have been in practice for several years, new applications of existing treatments continue to be developed and have potential to improve care for patients with pituitary tumors.


This page last updated: 22nd May 2013
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