Endocrine Malignancies
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Adrenocortical Cancer
Multiple Endocrine Neoplasia /Familial Thyroid Ca.
Pancreas Cancer
Parathyroid Cancer
Pheochromocytoma and Paraganglioma
Pituitary Cancer
Thymoma and Thymic Carcinoma
Thyroid Cancer
Medical Terminology for Cancer: The Endocrine System
Endocrinology / General Resources
Latest Research Publications

Endocrinology / General Resources (5 links)

Latest Research Publications

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

Bayley JP, Oldenburg RA, Nuk J, et al.
Paraganglioma and pheochromocytoma upon maternal transmission of SDHD mutations.
BMC Med Genet. 2014; 15:111 [PubMed] Related Publications
BACKGROUND: The SDHD gene encodes a subunit of the mitochondrial tricarboxylic acid cycle enzyme and tumor suppressor, succinate dehydrogenase. Mutations in this gene show a remarkable pattern of parent-of-origin related tumorigenesis, with almost all SDHD-related cases of head and neck paragangliomas and pheochromocytomas attributable to paternally-transmitted mutations.
METHODS: Here we explore the underlying molecular basis of three cases of paraganglioma or pheochromocytoma that came to our attention due to apparent maternal transmission of an SDHD mutation. We used DNA analysis of family members to establish the mode of inheritance of each mutation. Genetic and immunohistochemical studies of available tumors were then carried out to confirm SDHD-related tumorigenesis.
RESULTS: We found convincing genetic and immunohistochemical evidence for the maternally-related occurrence of a case of pheochromocytoma, and suggestive evidence in a case of jugular paraganglioma. The third case appears to be a phenocopy, a sporadic paraganglioma in an SDHD mutation carrier with no immunohistochemical or DNA evidence to support a causal link between the mutation and the tumor. Microsatellite analysis in the tumor of patient 1 provided evidence for somatic recombination and loss of the paternal region of chromosome 11 including SDHD and the maternal chromosome including the centromere and the p arm.
CONCLUSIONS: Transmission of SDHD mutations via the maternal line can, in rare cases, result in tumorigenesis. Despite this finding, the overwhelming majority of carriers of maternally-transmitted mutations will remain tumor-free throughout life.

Related: Chromosome 11 Pheochromocytoma and Paraganglioma

Wagle N, Grabiner BC, Van Allen EM, et al.
Response and acquired resistance to everolimus in anaplastic thyroid cancer.
N Engl J Med. 2014; 371(15):1426-33 [PubMed] Related Publications
Everolimus, an inhibitor of the mammalian target of rapamycin (mTOR), is effective in treating tumors harboring alterations in the mTOR pathway. Mechanisms of resistance to everolimus remain undefined. Resistance developed in a patient with metastatic anaplastic thyroid carcinoma after an extraordinary 18-month response. Whole-exome sequencing of pretreatment and drug-resistant tumors revealed a nonsense mutation in TSC2, a negative regulator of mTOR, suggesting a mechanism for exquisite sensitivity to everolimus. The resistant tumor also harbored a mutation in MTOR that confers resistance to allosteric mTOR inhibition. The mutation remains sensitive to mTOR kinase inhibitors.

Related: Thyroid Cancer Everolimus (Afinitor)

Chung H, Chapman WC
Liver transplantation for metastatic neuroendocrine tumors.
Adv Surg. 2014; 48:235-52 [PubMed] Related Publications
The ideal management of NET must be addressed on a case-by-case basis, with consideration given to patient factors, disease burden, and clinical tumor activity. Outcome improvement for LT in the setting of metastatic disease requires better characterization of the biological behavior of NETs and further identification of factors to be included in the selection criteria. Box 3 summarizes the many areas that have been, and are currently, undergoing investigation. LT as an attempt for cure rather than palliation is a justified treatment option for well-selected patients with metastatic neuroendocrine tumors of the pancreas and GI system. Optimization of pretransplantation staging and patient management algorithms, patient selection, and posttransplant management options are areas that need to be better defined. Further investigations for defining reproducible prognostic factors, consistent histopathologic evaluation, and uniform preoperative staging and site-specific data are needed. With the advancement of newer treatment modalities, it is necessary to define the role of LT along with the optimal perioperative management of existing and recurrent disease.

Related: Gastrointestinal System Cancers Cancer of the Pancreas Pancreatic Cancer

Poruk KE, Firpo MA, Mulvihill SJ
Screening for pancreatic cancer.
Adv Surg. 2014; 48:115-36 [PubMed] Related Publications
Even with improved cancer care generally, the incidence and death rate is increasing for pancreatic cancer. Concern exists that a further increase in deaths caused by pancreatic cancer will be seen as other causes of death, such as heart disease and other cancers, decline. Critical exploration of screening high-risk patients as a tool to reduce deaths from pancreatic cancer should be considered. Technological advances and improved understanding of pancreatic cancer biology provides an opportunity to identify and test a panel of early detection biomarkers easily, accurately, and inexpensively measured in blood, urine, stool, or saliva samples. These biomarkers may have additional usefulness in staging, stratification for treatment, establishing prognosis, and assessing response to therapy in this disease. Screening may prove to be one of several strategies to improve outcomes in a disease that has otherwise been difficult to defeat.

Related: Cancer of the Pancreas Pancreatic Cancer

Marchegiani G, Fernández-del Castillo C
Is it safe to follow side branch IPMNs?
Adv Surg. 2014; 48:13-25 [PubMed] Related Publications
Management of Bd-IPMN remains challenging. Critical appraisal of the published literature reveals that the actual treatment of what is presumed to be Bd-IPMN remains unsatisfactory, with a high rate of surgically overtreated patients. Until we accrue more precise knowledge of the natural history of Bd-IPMN, management of patients with this presumed diagnosis should be individually tailored and preferably carried out in centers with a high expertise. For now, the authors strongly think that the old guidelines should be followed in most patients because these have proven to correctly identify lesions that can be safely followed. Although the new guidelines allow for follow-up of lesions greater than 3 cm, and for the most part this is safe, they should be used cautiously in younger patients because very close surveillance would be required for their long remaining lifespan.

Related: Cancer of the Pancreas Pancreatic Cancer USA

Abid N, Mnif H, Mellouli M, et al.
Uterine tumour resembling ovarian sex cord tumours presenting as multiple endometrial and cervical uterine polyps: a case report.
Pathologica. 2014; 106(2):73-6 [PubMed] Related Publications
BACKGROUND: Uterine tumours resembling ovarian sex-cord tumours (UTROSCT) are very rare, benign uterine tumours, composed solely of sex cord elements. These tumours have a polyphenotypic immunophentype that favours a derivation from uterine mesenchymal stem cells.
CASE REPORT: A 43-year-old female presented with recurrent vaginal bleeding. On hysteroscopy, she had multiple endometrial and cervical polyps that were removed endoscopically. Histologically, the specimen contained epithelioid cells arranged in tubules, trabeculae and anastomosing cords, without significant cellular atypia or mitotic activity. Immunohistochemical studies were performed. The tumour was found to be diffusely positive for vimentin, calretinin and desmin, focally positive for cytokeratin, CD99 and inhibin and negative for chromogranin and CD10. A subsequent total hysterectomy was performed and revealed neoplastic infiltration of the myometrium.
CONCLUSION: A polyphenotypic immunophenotype is a characteristic feature of UTROSCT, and may be helpful in diagnosis and in exclusion of other lesions. Familiarity with this tumour by gynaecologists and pathologists is essential to avoid misdiagnosis:correct diagnosis of this neoplasm is important in patient management.

Related: Endometrial (Uterus) Cancer Endometrial Cancer Ovarian Cancer Cervical Cancer

Roncati L, Pignatti E, Vighi E, et al.
Pre-miR146a expression in follicular carcinomas of the thyroid.
Pathologica. 2014; 106(2):58-60 [PubMed] Related Publications
INTRODUCTION: Micro-RNA, a new class of small, non-coding RNAs, have been shown to be deregulated in several human carcinomas. In particular, SNP rs2910164 in pre-miR146a appears to be correlated with papillary thyroid carcinoma and may be involved in its genetic predisposition. Since data on follicular thyroid carcinomas (FTC) are lacking, we evaluated the involvement of SNP rs2910164 in FTC.
METHODS: Thirty-nine cases of FTC and 20 follicular adenomas, defined according to WHO criteria, were selected. DNA and RNA were extracted from formalin-fixed paraffin-embedded blocks of both neoplastic and non-neoplastic areas. The DNA region of pre-miR146a, containing SNP rs2910164, was sequenced. Total RNA including miRNAs was used for stem-loop RT reactions, and applying a standard TaqMan PCR kit protocol for real-time PCR. Wilcoxon signed-rank test and Friedman test were used for statistical analyses.
RESULTS: In 31% of FTC, the G allele was observed in neoplastic tissues, compared with the non-neoplastic areas (p < 0.05), whereas the CC phenotype was completely absent in tumours. Moreover, the expression of pre-miR146a was found to be significantly down-regulated in neoplastic tissues from FTC cases (p = 0.043), although no significant differences were seen in follicular thyroid adenomas.
DISCUSSION: The expression profile of pre-miR146a can be correlated with FTC tumourigenesis. The G allele in SNP rs2910164 appears to be correlated with the transition from normal to neoplastic tissue. The GG and GC alleles appear to be associated with an increased risk for FTC, while the CC allele seems to play a protective role.

Related: Thyroid Cancer

Limaiem F, Arfa N, Ben Hassen E, et al.
Neuroendocrine tumours of the pancreas: a clinicopathological study of nine cases including six insulinomas.
Pathologica. 2014; 106(2):51-7 [PubMed] Related Publications
BACKGROUND: Pancreatic neuroendocrine tumours (pNET) are relatively uncommon, accounting for 1-2% of all pancreatic neoplasms. They are characterised by varying clinical presentation, tumour biology and prognosis.
AIM: To provide an updated overview on clinicopathological features, treatment and outcome of pNET.
PATIENTS AND METHODS: In our retrospective study, we reviewed 9 cases of pNET that were diagnosed at the Pathology Department of Mongi Slim Hospital over an 11-year period (2003- 2013). Relevant clinical information and microscopic slides were available in all cases and were retrospectively reviewed. The latest WHO classification (2010) was adopted.
RESULTS: Our study group included 3 men and 6 women (M/F ratio 0.5) with an age between 20 and 75 years (mean = 52 years). Pancreatic neuroendocrine tumours ranged in size from 0.5 to 10 cm (mean 4 cm). The sites of pNET were the head of the pancreas (n = 4), the body of the pancreas (n = 3) and the tail of the pancreas (n = 2). Enucleation of the tumour was performed in five cases, Three patients underwent distal pancreatectomy and splenectomy, whereas only one patient had central pancreatectomy. Histopathological examination of the surgical specimen coupled with immunohistochemical study established a diagnosis of pNET grade 1 (G1) in seven cases and grade 2 (G2) in two cases.
CONCLUSION: Pancreatic neuroendocrine tumours are a heterogeneous group of neoplasms with distinct tumour genetics, biology and clinicopathological features. Accurate clinical and pathologic diagnosis is an important first step in developing an appropriate management plan.

Related: Cancer of the Pancreas Pancreatic Cancer

Gilani SM, Tashjian R, Barawi M, Al-Khafaji B
Cytologic features of solid pseudopapillary neoplasms of the pancreas: a single institutional experience based on evaluation of diagnostic utility of endoscopic ultrasound-guided fine needle aspiration (EUS-FNA).
Pathologica. 2014; 106(2):45-50 [PubMed] Related Publications
BACKGROUND: Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) is an important modality for diagnosing solid and cystic pancreatic lesions. The objectives of this retrospective study are to review the cytologic criteria used to diagnose pancreatic solid pseudopapillary neoplasms (SPNs) and to evaluate the utility of EUS-FNA by correlating cytologic and histologic samples.
CASE REVIEWS: Of the 924 pancreatic FNAs performed at our institution from January 2002 through February 2013, four histologically confirmed cases of SPN were identified; three had an initial cytologic diagnosis of SPN. All four cases lacked on-site evaluation. Cytologic smears were assessed by two reviewers for the presence of a cellular aspirate, fibrovascular stalks lined by neoplastic cells with pale to finely granular cytoplasm, and monotonous, oval nuclei containing delicate chromatin, inconspicuous nucleoli, and grooves and inclusions. Three cases were diagnosed as SPN on cytologic examination and confirmed histologically. The remaining case was deemed a pancreatic endocrine neoplasm on cytology, but SPN on final histology. The most consistent cytologic feature we encountered was the presence of a cellular aspirate containing fibrovascular stalks lined by monotonous neoplastic cells with oval nuclei and nuclear grooves.
CONCLUSION: We conclude that EUS-FNA is an effective diagnostic tool in the diagnosis of pancreatic SPNs.

Related: Cancer of the Pancreas Pancreatic Cancer

Takeshita T, Ninoi T, Maebayashi T, et al.
Diffusion-weighted magnetic resonance imaging findings in a patient with struma ovarii.
Osaka City Med J. 2014; 60(1):45-52 [PubMed] Related Publications
In this report, the magnetic resonance imaging (MRI) appearance of struma ovarii (SO) in a patient who underwent diffusion-weighted imaging (DWI) of the pelvis and subsequent histological analysis is described. The solid portion of SO showed a high apparent diffusion coefficient (ADC) value, indicating unrestricted diffusion, and each loculus of SO showed different ADC values due to the different viscosity of the cyst contents in each loculus. These unique and characteristic DWI findings may serve as a helpful sign in making the correct diagnosis of SO when DWI findings are interpreted in conjunction with conventional MRI findings.

Related: Ovarian Cancer

Ma BY, Parajuly SS, Ying SX, Lan PY
Application of shear wave elastography in fine needle aspiration biopsy for thyroid nodule.
J Pak Med Assoc. 2014; 64(8):954-7 [PubMed] Related Publications
Ultrasound guided fine needle aspiration biopsy (FNAB) is the first-line procedure in diagnosing thyroid nodules. However, emerging studies have reported its unsatisfactory cell sample for cytology evaluation. In this case report, shear wave elastography (SWE) guided FNAB on a thyroid nodule is presented. Biopsies were performed on both hard and soft areas of the same nodule on the SWE image. Cytological results demonstrated the sufficient diagnostic components and typical nuclear features of papillary carcinoma found on the specimen from the hard area. This case study indicates SWE is a useful complementary tool to conventional B-mode ultrasound in guiding thyroid nodule FNAB.

Hannan A, Awan UE, Siddiqui N, Muzaffar N
Malignant transformations in ovarian teratomas: a report of four cases.
J Pak Med Assoc. 2014; 64(8):946-8 [PubMed] Related Publications
Mature cystic teratoma (MCT) is a common ovarian neoplasm in young females. A secondary malignant transformation occurs rarely in cystic teratomas at an older age. These secondary malignant neoplasms most commonly are squamous cell carcinomas (SCCs). Various mechanisms are reported, but the exact aetiology is unknown. We report three cases of SCC arising in cystic teratoma and one case of papillary thyroid neoplasm as secondary transformation. The SCCs were arising from the cyst wall, while the papillary thyroid malignancy arose from the normal-looking thyroid epithelium. Histologically, all SCC cases were poorly differentiated. Poor prognostic features for secondary transformations include size more than 10 cm, older age and rapid growth. Data is scarce regarding their appropriate treatment. However, surgical debulking is necessary. Platinum-based adjuvant regimens and taxanes are recommended in cases of advanced disease. In this paper we review and share our experience with this rare disorder.

Related: Ovarian Cancer

Russell SS
Testicular cancer: overview and implications for health care providers.
Urol Nurs. 2014 Jul-Aug; 34(4):172-6, 192 [PubMed] Related Publications
Testicular cancer is the most common cancer in young men 15 to 35 years of age. This diagnosis, while devastating to the young man has, with aggressive treatment, the potential of a cure. This article will focus on the symptoms, the diagnosis, and the treatment of testicular cancer. In addition to nursing care during those stages, the nursing implications in educating those at risk along with early detection of this cancer will be the considered in this article.

Related: Testicular Cancer

Pokharna P, Sharma N, Tiwari A, et al.
Testicular embryonal cell carcinoma presenting as haemoptysis and skin nodules.
Indian J Chest Dis Allied Sci. 2014 Apr-Jun; 56(2):125-7 [PubMed] Related Publications
Embryonal cell carcinoma affects young males in the prime of their life with majority of tumours already having metastasised at the time of diagnosis. Subcutaneous metastasis from embryonal carcinoma is rare and is associated with widespread disease and poor prognosis. We report a case of 22-year-old male who presented with haemoptysis and skin nodules. Fine needle aspiration cytology of skin nodules and the lung lesion led to the diognosis of testicular embryonal cell carcinoma.

Related: Testicular Cancer

Teng FF, Kalloger SE, Brotto L, McAlpine JN
Determinants of quality of life in ovarian cancer survivors: a pilot study.
J Obstet Gynaecol Can. 2014; 36(8):708-15 [PubMed] Related Publications
OBJECTIVE: Ovarian cancer treatments and outcomes vary substantially, yielding a diverse group of survivors. Few data exist on quality of life (QoL) concerns and the foremost needs of these patients. Our goal was to conduct a pilot study to determine the QoL needs of ovarian cancer survivors to establish priorities for future interventions.
METHODS: In this cross-sectional study, the European Organization for Research and Treatment of Cancer Quality of Life Questionnaires (EORTC QLQ-C30 and OV28) QoL questionnaires and one investigator-derived questionnaire were administered in an outpatient setting. Clinical parameters were abstracted and tested for associations with QoL measures.
RESULTS: A total of 102 women consented to participate and completed all components. Their mean age was 58 years (range 29 to 85), with 80% having epithelial ovarian carcinoma and 66% high-grade serous carcinoma. Women with stage I (28%), II (15%), III (47%), and IV (10%) lesions were represented in the primary treatment (25%), surveillance (46%), recurrent (23%), and palliative (7%) phases of the survivorship continuum. Fifty-one percent characterized their disease burden as "quite a bit" or "very much," and this did not vary by histology or diagnoses. Global QoL did not vary by clinico-pathologic parameters. Cardiovascular and respiratory comorbidities were associated with EORTC scores in physical functioning (P=0.027 for cardiovascular and P=0.041 for respiratory), global QoL (P=0.03 for cardiovascular and P=0.039 for respiratory), and sexual health (P=0.025 for cardiovascular). Task completion/memory/concentration, anxiety, and fatigue were the distress categories given highest priority by respondents.
CONCLUSION: In women with ovarian cancer, clinical factors such as age, stage, and histology did not have a significant impact on QoL. Psychosocial factors have a larger impact on global QoL than physical symptoms.

Related: Ovarian Cancer

Santini F, Marzullo P, Rotondi M, et al.
Mechanisms in endocrinology: the crosstalk between thyroid gland and adipose tissue: signal integration in health and disease.
Eur J Endocrinol. 2014; 171(4):R137-52 [PubMed] Related Publications
Obesity and thyroid diseases are common disorders in the general population and they frequently occur in single individuals. Alongside a chance association, a direct relationship between 'thyroid and obesity' has been hypothesized. Thyroid hormone is an important determinant of energy expenditure and contributes to appetite regulation, while hormones and cytokines from the adipose tissue act on the CNS to inform on the quantity of energy stores. A continuous interaction between the thyroid hormone and regulatory mechanisms localized in adipose tissue and brain is important for human body weight control and maintenance of optimal energy balance. Whether obesity has a pathogenic role in thyroid disease remains largely a matter of investigation. This review highlights the complexity in the identification of thyroid hormone deficiency in obese patients. Regardless of the importance of treating subclinical and overt hypothyroidism, at present there is no evidence to recommend pharmacological correction of the isolated hyperthyrotropinemia often encountered in obese patients. While thyroid hormones are not indicated as anti-obesity drugs, preclinical studies suggest that thyromimetic drugs, by targeting selected receptors, might be useful in the treatment of obesity and dyslipidemia.

Related: Thyroid Cancer

Likhtarov I, Kovgan L, Masiuk S, et al.
Thyroid cancer study among Ukrainian children exposed to radiation after the Chornobyl accident: improved estimates of the thyroid doses to the cohort members.
Health Phys. 2014; 106(3):370-96 [PubMed] Article available free on PMC after 01/03/2015 Related Publications
In collaboration with the Ukrainian Research Center for Radiation Medicine, the U.S. National Cancer Institute initiated a cohort study of children and adolescents exposed to Chornobyl fallout in Ukraine to better understand the long-term health effects of exposure to radioactive iodines. All 13,204 cohort members were subjected to at least one direct thyroid measurement between 30 April and 30 June 1986 and resided at the time of the accident in the northern parts of Kyiv, Zhytomyr, or Chernihiv Oblasts, which were the most contaminated territories of Ukraine as a result of radioactive fallout from the Chornobyl accident. Thyroid doses for the cohort members, which had been estimated following the first round of interviews, were re-evaluated following the second round of interviews. The revised thyroid doses range from 0.35 mGy to 42 Gy, with 95% of the doses between 1 mGy and 4.2 Gy, an arithmetic mean of 0.65 Gy, and a geometric mean of 0.19 Gy. These means are 70% of the previous estimates, mainly because of the use of country-specific thyroid masses. Many of the individual thyroid dose estimates show substantial differences because of the use of an improved questionnaire for the second round of interviews. Limitations of the current set of thyroid dose estimates are discussed. For the epidemiologic study, the most notable improvement is a revised assessment of the uncertainties, as shared and unshared uncertainties in the parameter values were considered in the calculation of the 1,000 stochastic estimates of thyroid dose for each cohort member. This procedure makes it possible to perform a more realistic risk analysis.

Related: Thyroid Cancer

Vrndic OB, Radivojevic SD, Jovanovic MD, et al.
Oxidative stress in patients with differentiated thyroid cancer: early effects of radioiodine therapy.
Indian J Biochem Biophys. 2014; 51(3):223-9 [PubMed] Related Publications
Ionizing radiation in differentiated thyroid cancer (DTC) patients treated with radioiodine (131-I) produces reactive oxygen species (ROS), which could induce oxidative stress with disturbance of redox balance. The aim of this study was to evaluate oxidative stress in DTC patients treated with 3.7 or 5.5 GBq of 131-I using values for serum malondialdehyde (MDA, a marker of oxidative stress), uric acid (to determine antioxidant status) and total antioxidative status (TAS). The study population included 20 DTC patients and 20 healthy controls. Significant differences in MDA concentrations were found between DTC patients before 131-I therapy and control subjects (p = 0.001), while TAS values were similar in both populations (p > 0.05). There was a negative correlation between MDA concentrations and TAS in the DTC group before therapy (R2 = 0.2973, p = 0.013). Three days after 131-I therapy, MDA concentrations were higher than the pretreatment values (3.36 +/- 1.69 nmol/mL vs. 2.93 +/- 1.31 nmol/mL; p = 0.006), while serum uric acid concentrations declined progressively from 341.0 +/- 80.39 micromol/L to 304.25 +/- 77.25 micromol/L (p = 0.026) in 3 days and 291.2 +/- 88.86 micromol/L (p = 0.009) in 7 days after 131-I therapy. There was no dose-dependent effect on MDA, or uric acid concentrations and TAS. Thus, 131-I therapy in DTC patients induced oxidative stress, which was accompanied by a simultaneous and extended reduction in uric acid concentration, but without significant disturbances in TAS. This is the first study that evaluated TAS capacity in DTC patients before and 7 days after 131-I therapy. The relatively stabile TAS values in these patients indicated a good protection from oxidative stress induced by high doses of ionizing radiation.

Related: Thyroid Cancer

Roch AM, Ceppa EP, Al-Haddad MA, et al.
The natural history of main duct-involved, mixed-type intraductal papillary mucinous neoplasm: parameters predictive of progression.
Ann Surg. 2014; 260(4):680-8; discussion 688-90 [PubMed] Related Publications
OBJECTIVE: As such, the natural history of MPD-involved IPMN is poorly understood.
BACKGROUND: The high-risk of malignancy associated with main pancreatic duct (MPD)-involved intraductal papillary mucinous neoplasm (IPMN) has been established by surgical series. The International Consensus Guidelines recommend surgical resection of MPD-involved IPMN in fit patients.
METHODS: A review of a prospectively collected database (1992-2012) of patients with IPMN undergoing primary surveillance was performed. Invasive progression was defined as invasive carcinoma on pathology and/or positive cytopathology. Analyses included univariate, logistic regression, and receiver operating characteristic curve analyses.
RESULTS: A total of 503 patients with IPMN underwent primary surveillance, 70 for MPD-involved, mixed-type IPMN. Indications for intensive surveillance of these 70 high-risk patients were comorbidities, patient choice, and early/borderline MPD dilation (42%, 51%, and 7%, respectively). Mean follow-up was 4.7 years. Nine patients (13%) progressed at a mean of 3.5 (range, 1-9) years during follow-up. Univariate analyses yielded weight loss, interval (from isolated branch-duct IPMN) to MPD involvement, diffuse MPD dilation, increase of MPD diameter, absence of extra pancreatic cysts, elevated serum CA19-9 levels, and elevated serum alkaline phosphatase levels as significant. Maximum MPD and/or branch-duct diameter were not significant. In logistic regression, diffuse MPD dilation, serum CA19-9 and serum alkaline phosphatase levels, and absence of extra pancreatic cysts were predictors of invasiveness. The receiver operating characteristic curve indicated that the combination of these 4 factors achieved an accuracy of 98% in predicting progression.
CONCLUSIONS: Primary surveillance of mixed-type IPMN may be a reasonable strategy in select patients. Diffuse MPD dilation, serum CA19-9, serum alkaline phosphatase, and absence of extrapancreatic cysts predict patients likely to progress during primary surveillance.

Related: Cancer of the Pancreas Pancreatic Cancer

Croome KP, Farnell MB, Que FG, et al.
Total laparoscopic pancreaticoduodenectomy for pancreatic ductal adenocarcinoma: oncologic advantages over open approaches?
Ann Surg. 2014; 260(4):633-8; discussion 638-40 [PubMed] Related Publications
OBJECTIVE: To directly compare the oncologic outcomes of TLPD and OPD in the setting of pancreatic ductal adenocarcinoma.
BACKGROUND: Total laparoscopic pancreaticoduodenectomy (TLPD) has been demonstrated to be feasible and may have several potential advantages over open pancreaticoduodenectomy (OPD), including lower blood loss and shorter hospital stay. Whether potential advantages could allow patients to recover in a timelier manner and pursue adjuvant treatment options remains to be answered.
METHODS: We reviewed data for all patients undergoing TLPD (N = 108) or OPD (N = 214) for pancreatic ductal adenocarcinoma at our institution between January 2008 and July 2013.
RESULTS: Neoadjuvant therapy, tumor size, node positivity, and margin-positive resection were not significantly different between the 2 groups. Median length of hospital stay was significantly longer in the OPD group (9 days; range, 5-73 days) than in the TLPD group (6 days; range, 4-118 days; P < 0.001). There was a significantly higher proportion of patients in the OPD group (12%) who had a delay of greater than 90 days or who did not receive adjuvant chemotherapy at all compared with that in the TLPD group (5%; P = 0.04). There was no significant difference in overall survival between the 2 groups (P = 0.22). A significantly longer progression-free survival was seen in the TLPD group than in the OPD group (P = 0.03).
CONCLUSIONS: TLPD is not only feasible in the setting of pancreatic ductal adenocarcinoma but also has advantages such as shorter hospital stay and faster recovery, allowing patients to recover in a timelier manner and pursue adjuvant treatment options. This study also demonstrated a longer progression-free survival in patients undergoing TLPD than those undergoing OPD.

Related: Cancer of the Pancreas Pancreatic Cancer

Adam MA, Pura J, Gu L, et al.
Extent of surgery for papillary thyroid cancer is not associated with survival: an analysis of 61,775 patients.
Ann Surg. 2014; 260(4):601-5; discussion 605-7 [PubMed] Related Publications
OBJECTIVE: To examine the association between the extent of surgery and overall survival in a large contemporary cohort of patients with papillary thyroid cancer (PTC).
BACKGROUND: Guidelines recommend total thyroidectomy for PTC tumors >1 cm, based on older data demonstrating an overall survival advantage for total thyroidectomy over lobectomy.
METHODS: Adult patients with PTC tumors 1.0-4.0 cm undergoing thyroidectomy in the National Cancer Database, 1998-2006, were included. Cox proportional hazards models were applied to measure the association between the extent of surgery and overall survival while adjusting for patient demographic and clinical factors, including comorbidities, extrathyroidal extension, multifocality, nodal and distant metastases, and radioactive iodine treatment.
RESULTS: Among 61,775 PTC patients, 54,926 underwent total thyroidectomy and 6849 lobectomy. Compared with lobectomy, patients undergoing total thyroidectomy had more nodal (7% vs 27%), extrathyroidal (5% vs 16%), and multifocal disease (29% vs 44%) (all Ps < 0.001). Median follow-up was 82 months (range, 60-179 months). After multivariable adjustment, overall survival was similar in patients undergoing total thyroidectomy versus lobectomy for tumors 1.0-4.0 cm [hazard ratio (HR) = 0.96; 95% confidence interval (CI), 0.84-1.09); P = 0.54] and when stratified by tumor size: 1.0-2.0 cm [HR = 1.05; 95% CI, 0.88-1.26; P = 0.61] and 2.1-4.0 cm [HR = 0.89; 95% CI, 0.73-1.07; P = 0.21]. Older age, male sex, black race, lower income, tumor size, and presence of nodal or distant metastases were independently associated with compromised survival (P < 0.0001).
CONCLUSIONS: Current guidelines suggest total thyroidectomy for PTC tumors >1 cm. However, we did not observe a survival advantage associated with total thyroidectomy compared with lobectomy. These findings call into question whether tumor size should be an absolute indication for total thyroidectomy.

Related: Thyroid Cancer

Tosheva E, Yonkov A, Stojanov V, et al.
Clinical case of neuroendocrine malignant tumor with unusual localization in the adrenal cortex.
Khirurgiia (Sofiia). 2014; (1):39-42 [PubMed] Related Publications
The authors present unusual clinical case with atypical diagnostic work-up and histological result of adrenal neuroendocrine carcinoma.

Related: Adrenocortical Cancer Adrenocortical Carcinoma - Molecular Biology

Tignanelli CJ, Herrera Loeza SG, Yeh JJ
KRAS and PIK3CA mutation frequencies in patient-derived xenograft models of pancreatic and colorectal cancer are reflective of patient tumors and stable across passages.
Am Surg. 2014; 80(9):873-7 [PubMed] Related Publications
One obstacle in the translation of advances in cancer research into the clinic is a deficiency of adequate preclinical models that recapitulate human disease. Patient-derived xenograft (PDX) models are established by engrafting patient tumor tissue into mice and are advantageous because they capture tumor heterogeneity. One concern with these models is that selective pressure could lead to mutational drift and thus be an inaccurate reflection of patient tumors. Therefore, we evaluated if mutational frequency in PDX models is reflective of patient populations and if crucial mutations are stable across passages. We examined KRAS and PIK3CA gene mutations from pancreatic ductal adenocarcinoma (PDAC) (n = 30) and colorectal cancer (CRC) (n = 37) PDXs for as many as eight passages. DNA was isolated from tumors and target sequences were amplified by polymerase chain reaction. KRAS codons 12/13 and PIK3CA codons 542/545/1047 were examined using pyrosequencing. Twenty-three of 30 (77%) PDAC PDXs had KRAS mutations and one of 30 (3%) had PIK3CA mutations. Fifteen of 37 (41%) CRC PDXs had KRAS mutations and three of 37 (8%) had PIK3CA mutations. Mutations were 100 per cent preserved across passages. We found that the frequency of KRAS (77%) and PIK3CA (3%) mutations in PDAC PDX was similar to frequencies in patient tumors (71 to 100% KRAS, 0 to 11% PIK3CA). Similarly, KRAS (41%) and PIK3CA (8%) mutations in CRC PDX closely paralleled patient tumors (35 to 51% KRAS, 12 to 21% PIK3CA). The accurate mirroring and stability of genetic changes in PDX models compared with patient tumors suggest that these models are good preclinical surrogates for patient tumors.

Related: Colorectal (Bowel) Cancer Cancer of the Pancreas Pancreatic Cancer KRAS gene

Diegidio P, Kolok D, Brown W, Camps JI
Papillary thyroid cancer in identical adolescent twins with osteogenesis imperfecta and Hashimoto's thyroiditis: is there a genetic link?
Am Surg. 2014; 80(9):849-50 [PubMed] Related Publications
The relationship between Hashimoto's thyroiditis (HT) and papillary thyroid cancer (PTC) remains controversial. Researchers have identified multiple genes that could put patients with HT at risk for PTC. None are related to osteogenesis imperfecta (OI). We present identical twin sisters with OI who developed PTC in the setting HT.

Related: Thyroid Cancer

Bhalla A, Sandhu F, Sieber S
Primary adrenal leiomyosarcoma: a case report and review of the literature.
Conn Med. 2014; 78(7):403-7 [PubMed] Related Publications
Primary adrenal leiomyosarcoma has been reported previously in 25 patients. The patient presented herein is the only case where the definitive diagnosis was made with core needle biopsy evaluation. A 45-year-old male presented with pain in the back and right groin. Radiological evaluation demonstrated a heterogeneous 11 cm right adrenal mass, multiple liver masses, and an enlarged aortocaval lymph node. No retroperitoneal mass was identified. Core needle biopsies revealed a malignant mesenchymal neoplasm composed of atypical spindle shaped cells arranged in intersecting fascicles, with high mitotic activity and focal tumor necrosis. Immunohistochemical stains revealed immunoreactivity for smooth muscle actin and desmin. S-100 and c-kit stains were negative. The diagnosis of adrenal leiomyosarcoma with liver metastasis was rendered. It was an aggressive tumor with clinical presentation at Figure 1. Core needle biopsy, a an advanced stage. Definitive diagnosis of this tumor by core needle biopsy can obviate the need for surgical biopsy in patients with advanced disease.

Zhang L, Xue Y, Jiang B, et al.
Multiscale agent-based modelling of ovarian cancer progression under the stimulation of the STAT 3 pathway.
Int J Data Min Bioinform. 2014; 9(3):235-53 [PubMed] Related Publications
This research is developed to simulate ovarian cancer progression with signal transducers and activators of the transcription 3 (STAT 3) pathway. The main focus is on studying how the STAT 3 pathway affects the cancer cells' biomechanical phenotype under the stimulation of the interleukin-6 (IL-6) cytokine and various well-known microscopic factors. The simulated results agreed with recent experimental evidence that ovarian cancer cells with a stimulated STAT 3 pathway have high survival rates and drug resistance. And we discussed how the IL6 and these well-known microscopic factors impacted the cancer progression.

Related: Apoptosis Ovarian Cancer

Ozcan HN, Oguz B, Sen HS, et al.
Imaging features of primary malignant pancreatic tumors in children.
AJR Am J Roentgenol. 2014; 203(3):662-7 [PubMed] Related Publications
OBJECTIVE: The purpose of this article is to describe the sonographic, CT, and MRI features of primary malignant pancreatic tumors of childhood.
CONCLUSION: Most children with a pancreatic tumor present with a solid pseudopapillary tumor that is usually well marginated and has solid and cystic areas surrounded by a fibrous capsule. Pancreatoblastoma is more aggressive than solid pseudopapillary tumor. The imaging features are those of a large heterogeneous tumor. Ductal adenocarcinoma is rare in children and has a poor prognosis.

Related: Cancer of the Pancreas Pancreatic Cancer

Hahn SY, Han BK, Ko EY, et al.
Ultrasound findings of papillary thyroid carcinoma originating in the isthmus: comparison with lobe-originating papillary thyroid carcinoma.
AJR Am J Roentgenol. 2014; 203(3):637-42 [PubMed] Related Publications
OBJECTIVE: The goal of this study was to analyze the differences in ultrasound characteristics of papillary thyroid carcinoma (PTC) originating in the thyroid isthmus versus that originating from the lobes.
MATERIALS AND METHODS: From a retrospective review of our institution's database of records dated between January 2007 and December 2008, we identified 48 patients with classic PTCs located in the isthmus. All the patients had undergone preoperative ultrasound imaging, total thyroidectomy with bilateral central lymph node dissection, and postoperative follow-up for at least 2 years. As a control group, 96 patients with classic PTCs located in the lobe who had undergone total thyroidectomy with bilateral central lymph node dissection during the same period were randomly matched to the study patients for age, sex, and tumor size.
RESULTS: According to the clinicopathologic analyses, the incidence of extrathyroidal extension was higher in the patients with a tumor originating in the isthmus than in the control group (p = 0.026). According to the imaging analyses, the tumors originating in the isthmus more frequently had a circumscribed margin (p = 0.030), a wider-than-tall shape (p < 0.001), and the suspicion of extrathyroidal extension (p < 0.001) than those originating from the lobes.
CONCLUSION: The results of this study showed that PTCs originating in the isthmus were more likely to have extrathyroidal extension than those originating from the lobes. Therefore, careful ultrasound evaluation should be performed on masses in the thyroid isthmus even if ultrasound shows a circumscribed mass with a wider-than-tall shape.

Gnjidić Z, Kudelić N, Sajko T, et al.
Surgical treatment of prolactinomas--our experience.
Coll Antropol. 2014; 38(2):571-6 [PubMed] Related Publications
The dilemma of whether to apply surgical or drug treatment to prolactinomas has been ongoing for the past 30 years. The aim of this study is to compare the early postoperative values of prolactin (PRL) in two groups of patients with prolactinomas: those who underwent primary surgical-treatment, and those who underwent surgery after a dopamine agonist (DA) therapy. We present the results of surgical treatment on a series of 161 patients with prolactinomas. Surgery was the primary treatment in 65 patients, while 96 patients had surgery following a long-term treatment with a DA. All surgically treated prolactinomas were operated in the standard transsphenoidal, microsurgical approach. The criteria for hyperprolactinemia remission was a PRL level under 25 ng/ml. Early normalization of PRL was achieved in 92% of those patients who underwent primary surgical-treatment, yet it was achieved in only 42% of patients who were operated on after receiving a long-term drug treatment with a DA. The highest prevalence of postoperative normalization of PRL was achieved in a group of patients with microadenomas who were primarily operated on (98%). The worst results in postoperative normalization of PRL were found in the group of patients with macroadenomas who received a long-term drug treatment with a DA first. These results show our surgical experience in treating prolactinomas. Using surgical treatment, the best clinical outcome was achieved with microprolactinomas and intrasellar, well-confined macroprolactinomas. Nevertheless, we stress the need of an individualized approach and recommend treatment in multidisciplinary centres for pituitary diseases.

Related: Pituitary Tumors

Mikuz G, Böhm GW, Behrend M, et al.
Therapy-resistant metastasizing anaplastic spermatocytic seminoma: a cytogenetic hybrid: a case report.
Anal Quant Cytopathol Histpathol. 2014; 36(3):177-82 [PubMed] Related Publications
BACKGROUND: Anaplastic spermatocytic seminoma is a rare variant of the conventional spermatocytic seminoma, with only 6 cases reported up to now. The anaplastic variant contains only the medium-sized cell type, hallmarked by large-sized nucleoli, whereas the small lymphocyte-like and giant cells typical of the conventional spermatocytic seminoma are lacking.
CASE: We report herein an unusual case of a 40-year-old man with an anaplastic spermatocytic seminoma which metastasized first to the retroperitoneal lymph nodes and, something never before reported, subsequently to the lung and other organs. The immunophenotype with c-kit and SALL4 positive and PLAP, as well OCT 3/4 negative tumor cells were identical to those of conventional spermatocytic seminoma. Cytogenetically the tumor cells showed a gain of chromosome 9, typical for spermatocytic seminoma, but simultaneously also the short arm 12p were overexpressed--an overexpression crucial to the aggressive behavior of seminomas and other nonseminomatous tumors but never before encountered in spermatocytic seminoma.
CONCLUSION: The current opinion is that seminoma and nonseminomatous germ cell tumors develop from a common primitive progenitor cell, whereas spermatocytic seminomas develop from differentiated spermatogonia. The herein presented cytogenetic hybrid tumor shows that a crossover between the two different histogenetic "tracks" is possible.

Related: FISH Germ Cell Tumors Testicular Cancer

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