A comprehensive review on MEN2B.

MEN2B is a very rare autosomal dominant hereditary tumor syndrome associated with medullary thyroid carcinoma (MTC) in 100% cases, pheochromocytoma in 50% cases and multiple extra-endocrine features, many of which can be quite disabling. Only few data are available in the literature. The aim of this review is to try to give further insights into the natural history of the disease and to point out the missing evidence that would help clinicians optimize the management of such patients.

Surgery for lymph node metastases of medullary thyroid carcinoma: A review.

Medullary thyroid carcinoma (MTC) is a neuroendocrine malignancy of the thyroid C cells that occurs in hereditary and sporadic clinical settings. Metastatic spread commonly occurs to cervical and mediastinal lymph nodes. MTC cells do not concentrate radioactive iodine and are not sensitive to hormonal manipulation, and therefore surgery is the most effective option for curative therapy, reduction in tumor burden, or effective palliation.

Management of the Parathyroid Glands During Preventive Thyroidectomy in Patients With Multiple Endocrine Neoplasia Type 2.

Objectives: Patients with multiple endocrine neoplasia type 2 (MEN2) have mutations in the RET protooncogene and virtually all of them will develop medullary thyroid carcinoma. Family members identified by genetic testing are candidates for preventive thyroidectomy. Management of the parathyroids during thyroidectomy is controversial.

Anaplastic Thyroid Carcinoma, Version 2.2015.

This selection from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines) for Thyroid Carcinoma focuses on anaplastic carcinoma because substantial changes were made to the systemic therapy recommendations for the 2015 update. Dosages and frequency of administration are now provided, docetaxel/doxorubicin regimens were added, and single-agent cisplatin was deleted because it is not recommended for patients with advanced or metastatic anaplastic thyroid cancer.

Revised American Thyroid Association guidelines for the management of medullary thyroid carcinoma.

Introduction: The American Thyroid Association appointed a Task Force of experts to revise the original Medullary Thyroid Carcinoma: Management Guidelines of the American Thyroid Association.

Methods: The Task Force identified relevant articles using a systematic PubMed search, supplemented with additional published materials, and then created evidence-based recommendations, which were set in categories using criteria adapted from the United States Preventive Services Task Force Agency for Healthcare Research and Quality. The original guidelines provided abundant source material and an excellent organizational structure that served as the basis for the current revised document.

BRAF mutation is not predictive of long-term outcome in papillary thyroid carcinoma.

The BRAF mutation occurs commonly in papillary thyroid carcinoma (PTC). Previous investigations of its utility to predict recurrence-free survival (RFS) and disease-specific survival (DSS) have reported conflicting results and its role remains unclear. The purpose of this retrospective study was to determine the incidence of the BRAF mutation and analyze its relationship to clinicopathologic risk factors and long-term outcomes in the largest, single-institution American cohort to date.

Neuroendocrine tumors, version 1.2015.

Neuroendocrine tumors (NETs) comprise a broad family of tumors that may or may not be associated with symptoms attributable to hormonal hypersecretion. The NCCN Clinical Practice Guidelines in Oncology for Neuroendocrine Tumors discuss the diagnosis and management of both sporadic and hereditary NETs. This selection from the guidelines focuses on sporadic NETs of the pancreas, gastrointestinal tract, lung, and thymus.

Thyroid carcinoma, version 2.2014.

These NCCN Guidelines Insights focus on some of the major updates to the 2014 NCCN Guidelines for Thyroid Carcinoma. Kinase inhibitor therapy may be used to treat thyroid carcinoma that is symptomatic and/or progressive and not amenable to treatment with radioactive iodine. Sorafenib may be considered for select patients with metastatic differentiated thyroid carcinoma, whereas vandetanib or cabozantinib may be recommended for select patients with metastatic medullary thyroid carcinoma.

Hereditary thyroid cancer syndromes and genetic testing.

This review focuses on both hereditary medullary thyroid cancer (MTC) and hereditary nonmedullary thyroid cancer (NMTC) and discusses the genetics, clinical diagnosis and evaluation, and surgical approach to treatment of these malignancies. Areas of innovation as well as areas of debate are highlighted and management recommendations are made.

Biochemical cure after reoperations for medullary thyroid carcinoma: a meta-analysis.

Background: Despite meticulous surgical techniques, calcitonin levels remain detectable in 40 % to 66 % of patients after initial surgery for medullary thyroid carcinoma (MTC), and the optimal surgical management for persistent or recurrent disease remains controversial. Previous studies suggest that biochemical cure, defined by normalization of postoperative calcitonin measurements, predicts disease-free survival. Reoperative approaches range from targeted removal of detectable disease to comprehensive compartment-oriented lymph node clearance.

Treatment approach, surveillance, and outcome of well-differentiated thyroid cancer in childhood and adolescence.

Background: Well-differentiated thyroid carcinoma in children and adolescents is a rare disease with favorable prognosis despite regional and distant metastasis at presentation in many patients. Treatment recommendations are varied and there is little consensus on follow-up guidelines for these patients.

Methods: Medical records of patients less than 22 years of age treated at our institution were reviewed.

Adrenal myelolipoma: operative indications and outcomes.

Background: Adrenal myelolipoma (AM) is a benign lesion for which adrenalectomy is infrequently indicated. We investigated operative indications and outcomes for AM in a large single-institution series.

Subjects And Methods: A retrospective cohort study of prospectively collected data was conducted.

Pheochromocytoma in an 8-year-old patient with multiple endocrine neoplasia type 2A: implications for screening.

Childhood pheochromocytoma in the setting of multiple endocrine neoplasia type 2 (MEN2) remains rare and has not been reported under the age of 12. We present an 8-year-old female with known MEN 2A, C634Y RET mutation, diagnosed with a 6 cm pheochromocytoma requiring laparoscopic adrenalectomy. Given this patient's age at diagnosis, screening guidelines should recommend annual screening beginning at age 8 for patients with MEN 2B or MEN 2A codons 630 or 634 RET mutations.

Overexpression of miR-10a and miR-375 and downregulation of YAP1 in medullary thyroid carcinoma.

MicroRNAs are a primordial mechanism of gene expression control that appear to be crucial to cellular development and may play an important role in tumor development. Much is known about the genetics of medullary thyroid carcinomas, as approximately 25% are hereditary and harbor germ line activating mutations in the RET gene. Somatic RET mutations are also seen in roughly 50% of sporadic medullary thyroid carcinomas.

Risk factors affecting operative approach, conversion, and morbidity for adrenalectomy: a single-institution series of 402 patients.

Background: Risk factors for selecting patients for open adrenalectomy (OA) and for conversion are limited in most series. This study aimed to investigate variables that are important in selecting patients for OA, predict risk of conversion from laparoscopic adrenalectomy (LA), and impact 30-day outcomes of OA and LA.

Methods: A retrospective cohort study of prospectively collected data was conducted.

Outcomes of resection of extra-adrenal pheochromocytomas/paragangliomas in the laparoscopic era: a comparison with adrenal pheochromocytoma.

Introduction: Laparoscopic adrenalectomy (LA) is the standard for removal of adrenal pheochromocytomas (pheos), but laparoscopic (LAP) resection of paragangliomas (PGs) is controversial. This study analyzes our results of resection of PGs in the LAP era.

Methods: A retrospective record review of all patients who underwent resection of intra-abdominal PGs from 1998 to 2011 was performed.

Activation of the mTOR pathway in primary medullary thyroid carcinoma and lymph node metastases.

Purpose: Understanding the molecular pathogenesis of medullary thyroid carcinoma (MTC) is prerequisite to the design of targeted therapies for patients with advanced disease.

Experimental Design: We studied by immunohistochemistry the phosphorylation status of proteins of the RAS/MEK/ERK and PI3K/AKT/mTOR pathways in 53 MTC tissues (18 hereditary, 35 sporadic), including 51 primary MTCs and 2 cases with only lymph node metastases (LNM). We also studied 21 autologous LNMs, matched to 21 primary MTCs.

Neuroendocrine tumors.

Neuroendocrine tumors comprise a broad family of tumors, the most common of which are carcinoid and pancreatic neuroendocrine tumors. The NCCN Neuroendocrine Tumors Guidelines discuss the diagnosis and management of both sporadic and hereditary neuroendocrine tumors. Most of the recommendations pertain to well-differentiated, low- to intermediate-grade tumors.

Racial differences in survival after surgical treatment for melanoma.

Background: Surgical-treatment outcomes for melanoma in African Americans are poorly characterized as a result of low incidence of melanoma among African Americans. We examined differences by race in overall and melanoma-specific survival, stratified by receipt of surgical treatment and by specific types of surgical treatment.

Methods: Data from the 1973-2004 public-use Surveillance, Epidemiology and End Results Program (SEER) were analyzed by Cox proportional hazard models to compare the effects of surgical treatments on overall and melanoma-specific survival in blacks, whites, and other race, controlling for confounding demographic and tumor-related variables.

A novel missense mutation in the MEN1 gene in a patient with multiple endocrine neoplasia type 1.

Objective: To describe a novel germline missense mutation in exon 2 of the MEN1 gene identified in a man with multiple endocrine neoplasia type 1 (MEN 1).

Methods: We describe the patient's clinical, laboratory, and genetic data, and we review the relevant literature.

Results: A 41-year-old man with a history of primary hyperparathyroidism and left lower parathyroidectomy presented with nausea, vomiting, and hematemesis.

Medullary, anaplastic, and metastatic cancers of the thyroid.

Thyroid cancer is the most common endocrine malignancy accounting for nearly 95% of endocrine cancers according to the American Cancer Society. In 2009, more than 37,000 adults are projected to be diagnosed with thyroid cancer in the United States, and more than 1,600 people will die from the disease (www.cancer.

Management of cervical nodal metastasis detected on I-131 scintigraphy after initial surgery of well-differentiated thyroid carcinoma.

Background: The purpose of this study was to evaluate the outcome of patients with differentiated thyroid carcinoma whose posttherapy imaging demonstrated I-131 uptake in cervical lymph nodes.

Methods: In this prospective cohort study, 95 patients who underwent surgery for well-differentiated thyroid carcinoma had evidence of persistent cervical lymph node metastasis on posttherapy I-131 scintigraphy. These patients were evaluated by subsequent I-131 scintigraphy, and treated with additional I-131 therapy or surgical excision of cervical lymph nodes as clinically indicated.

Medullary thyroid carcinoma: management of lymph node metastases.

Medullary thyroid carcinoma (MTC) is a neuroendocrine malignancy of the thyroid C cells. Metastatic spread commonly occurs to cervical and mediastinal lymph nodes. MTC cells do not concentrate radioactive iodine and are not sensitive to hormonal manipulation.

Phase II clinical trial of sorafenib in metastatic medullary thyroid cancer.

Purpose: Mutations in the RET proto-oncogene and vascular endothelial growth factor receptor (VEGFR) activity are critical in the pathogenesis of medullary thyroid cancer (MTC). Sorafenib, a multikinase inhibitor targeting Ret and VEGFR, showed antitumor activity in preclinical studies of MTC.

Patients And Methods: In this phase II trial of sorafenib in patients with advanced MTC, the primary end point was objective response.