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Medullary Carcinoma Clinical Practice Guidelines in Oncology TM
2010
There are 3 main histologic types of thyroid carcinoma: differentiated (including papillary, follicular, and Hürthle), medullary, and anaplastic (aggressive undifferentiated tumor). Of 53,856 patients treated for thyroid carcinoma between 1985 and 1995, 80% had papillary, 11% had follicular, 3% had Hürthle cell, 4% had medullary, and 2% had anaplastic thyroid carcinoma. 1 These NCCN guidelines focus on medullary thyroid carcinoma (MTC). Another NCCN guideline addresses papillary, follicular, Hürthle cell, and anaplastic thyroid carcinomas (see NCCN Clinical Practice Guidelines in Oncology: Thyroid Carcinoma [to view the most recent version of these guidelines, visit the NCCN Web site at www.NCCN.org\]). MTC derives from the neuroendocrine parafol-The NCCN Medullary Carcinoma
Case Report and Literature Review of Medullary Thyroid Carcinoma OPEN ACCESS
American Journal of Otolaryngology and Head and Neck Surgery, 2018
Medullary Thyroid Carcinoma (MTC) accounts for approximately 1.7% of all thyroid malignancies. The majority of MTC are sporadic, but 15% to 25% of the cases result from a germline mutation in the RET proto-oncogene. Surgery is the first-line treatment modality for any patient presenting with respectable MTC. The propensity of MTC to metastasize to regional lymph nodes in the central and lateral compartments of the neck is a defining characteristic of this disease, which explains the surgical management in all clinical settings. Elimination of involved nodes can result in long-term cure or disease control, and a working knowledge of cervical lymph node anatomy and of the natural history of MTC spread within these nodal groups is important to the surgeon managing these patients. The degree and timing of the surgery are discussed centered on evidence-based medicine this case report and literature review discusses the contemporary management approach used for the evaluation, diagnosis and treatment of our patient with MTC.
Medullary thyroid carcinoma – update and present management controversies
Annals of The Royal College of Surgeons of England, 2006
INTRODUCTION Medullary thyroid carcinoma (MTC) is a rare thyroid malignancy arising from the parafollicular C cells. It accounts for 5–10% of thyroid malignancies and occurs in sporadic and hereditary forms. There are still many controversial aspects relating to the diagnosis and management of this unusual tumour in its various forms. The present article addresses the more important of these issues. METHODS A literature review was performed using Pubmed database combined with additional original papers obtained from citations in those articles identified in the original literature search. Only those articles which related specifically to the controversial issues addressed in this review were included. RESULTS Genetically determined tumours constitute approximately 25% of MTC and have special clinical interest because of their association with other endocrinopathies including phaeochromocytoma and hyperparathyroidism in the multiple endocrine neoplasia syndromes (MEN IIa and MEN IIb)...
Case Report and Literature Review of Medullary Thyroid Carcinoma
American Journal of Otolaryngology and Head and Neck Surgery
Medullary Thyroid Carcinoma (MTC) accounts for approximately 1.7% of all thyroid malignancies. The majority of MTC are sporadic, but 15% to 25% of the cases result from a germline mutation in the RET proto-oncogene. Surgery is the first-line treatment modality for any patient presenting with respectable MTC. The propensity of MTC to metastasize to regional lymph nodes in the central and lateral compartments of the neck is a defining characteristic of this disease, which explains the surgical management in all clinical settings. Elimination of involved nodes can result in longterm cure or disease control, and a working knowledge of cervical lymph node anatomy and of the natural history of MTC spread within these nodal groups is important to the surgeon managing these patients. The degree and timing of the surgery are discussed centered on evidence-based medicine this case report and literature review discusses the contemporary management approach used for the evaluation, diagnosis and treatment of our patient with MTC.
Endocrine Practice, 2013
Objective: This review outlines the advances in the diagnosis, genetic testing and the progress in medullary thyroid cancer (MTC) treatment in light of the most recent evidence. Methods: English-language articles of medullary thyroid cancer published up to 2012 were reviewed. The pertinent articles and their references were obtained and those considered relevant were reviewed for inclusion. Results: Medullary thyroid carcinoma (MTC), an uncommon neuroendocrine malignancy, accounts for 5% of thyroid cancers. MTC presents in the sporadic and also familial form (MEN 2A, MEN 2B or familial MTC syndromes). The familial forms are secondary to germline mutations in the RET proto-oncogene. Early diagnosis and treatment is paramount. Genetic testing has made possible early detection in asymptomatic carriers and high risk patients, with early or prophylactic surgery being curative in many. All carriers of a RET mutation should be evaluated and treated surgically for MTC. The primary treatment in all patients diagnosed with MTC is total thyroidectomy with central lymph node dissection. Calcitonin (Ct) and carcinoembryonic antigen (CEA) levels can be used as prognostic factors and as tumor markers. If elevated, further investigation including imaging modalities may be necessary for evaluation of metastatic disease. Surgery remains the main treatment for local and locally advanced disease. Conclusion: MTC is rare but morbidity and mortality remain high if untreated. Genetic testing should be offered to all patients. Treatment of choice remains total thyroidectomy and central lymph node dissection. Palliative treatment for advanced disease includes surgery, radiation, standard chemotherapy, chemoembolization and, more recently, targeted therapies (tyrosine kinase inhibitors).
A-Retrospective-Review-of-Patients-with-Medullary-Thyroid-Carcinoma (1).pdf
Journal of Otolaryngology Research, 2018
Introduction: Medullary thyroid cancer (MTC) is a neuroendocrine tumor of the parafollicular or C cells of the thyroid gland. The present study aims to determine the demographic, clinical and pathological characteristics of patients diagnosed with MTC at our surgical centre. Medullary thyroid cancer (MTC) is a neuroendocrine tumor of the parafollicular or C cells of the thyroid gland. MTC accounts for approximately 4% of thyroid carcinomas. The production of calcitonin is a characteristic feature of this tumor. The C cells originate from the embryonic neural crest; as a result, medullary carcinomas often have the clinical and histologic features of other neuroendocrine tumors such as carcinoid and islet-cell tumors. Most medullary thyroid carcinomas are sporadic. However, some are familial as part of the multiple endocrine neoplasia type 2 (MEN2) syndrome. The hereditary forms are linked to a germ line mutation in the rearranged during transfection (RET) oncogene, which are transmitted as an autosomal dominant trait with close to a 100% penetrance of the disease. Diagnosis of most of these tumors relies on fine-needle aspiration cytology (FNAC), which has been associated with a considerable number of false-negative results and may delay the diagnosis [1]. In addition, there is no consensus on the measurement of serum calcitonin levels in nodular thyroid diseases. Surgery is the most effective treatment for these tumors. The overall cause specific mortality of these tumors is 13.3 to 32.6% and 21.6 to 38.6% at 5 and 10 years, respectively [2]. MTC is a unique form of thyroid cancer, which differs in demography, presentation and prognosis from other types of thyroid cancer and thus presents with challenges in its management. The present study aims to determine the demographic, clinical and pathological characteristics of patients diagnosed with MTC at our surgical centre. Methodology: We retrospectively reviewed the hospital records of patients who were admitted and operated for MTC at our tertiary level referral hospital from September 2012 till February 2017. Clinical parameters like family history, presenting complaints, presence of metastasis at the time of presentation, site of metastasis and histopathological findings were noted. Duration of hospital stay and drainage was noted as well. Recurrence was defined as the reappearance of high levels of calcitonin or locoregional or distant metastasis after the disease or symptoms disappeared. Results: During the study period, four males and three females with mean age 56.45 years were operated. At the time of presentation, one patient had liver metastasis and the other had metastasis at multiple sites. All patients in the study underwent total thyroidectomy with central compartment clearance and bilateral neck dissection. On final histopathological examination, four patients had multifocal tumors and three patients had unifocal tumors. Median duration of hospital stay was 7 days and median duration of drain was 5 days. During the follow up period, two patients presented with locoregional recurrences and the two patients with distant metastasis died. Conclusion: Genetic screening and the evaluation of familial syndromes should always be considered in preoperative workup in MTC patients. Key therapeutic molecular targets in MTC need to be investigated in future studies.
Medullary Carcinoma of the Thyroid
Archives of Otolaryngology - Head and Neck Surgery, 1983
Medullary thyroid cancer (MTC) is a distinct C-cell tumor of the thyroid. We review the oncogenesis and management of both sporadic tumors and those tumors arising as part of specific inherited syndromes. The RET proto-oncogene plays a role in the development of inherited forms of MTC and has become important in the clinical management of patients and their families. The recognition of the high rate of regional nodal involvement has led to lymphadenectomy being strongly considered for patients undergoing thyroidectomy for MTC. Because of the multifocal nature of inherited disease, total thyroidectomy is the minimum surgical recommendation for all forms of medullary thyroid cancer.
Thyroid : official journal of the American Thyroid Association, 2015
The American Thyroid Association appointed a panel of experts to revise the original guidelines: "Medullary Thyroid Carcinoma: Management Guidelines of the American Thyroid Association", based on a review of the relevant literature. Relevant articles were identified using a systematic PubMed search and supplemented with additional published materials. Evidence-based recommendations were created and then categorized using criteria adapted from the United States Preventive Services Task Force, Agency for Healthcare Research and Quality. The original "Medullary Thyroid Cancer: Management Guidelines of the American Thyroid Association" provided abundant source material and an excellent organizational structure that served as the basis for the current revisions. The primary focus of this revision is on the diagnosis and treatment of patients with sporadic and hereditary MTC. The panel developed 68 evidence-based recommendations to assist clinicians in the care of pati...