Prognostic Variables and Calcitonin in Medullary Thyroid Cancer

Clark, Jonathan R.; Fridman, Tauba R.; Odell, Michael; Brierley, James D.; Walfish, Paul G.; Freeman, Jeremy L.

doi:10.1097/01.mlg.0000168114.90852.a6

Cited by 67 publications

(56 citation statements)

References 15 publications

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“…Medullary thyroid cancer (MTC) is a nonepithelial, neuroendocrine tumor with a more aggressive clinical behavior than differentiated thyroid cancer [298]. MTC comprises 5-10% of all thyroid cancers and arises from the parafollicular or C-cells of neuroendocrine origin, which produce the hormone calcitonin and make up 1% of thyroid cells.…”

Section: Calcitonin and Medullary Thyroid Cancermentioning

confidence: 99%

Amyloidogenesis of Natively Unfolded Proteins

Uversky

2008

CAR

174

152

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Aggregation and subsequent development of protein deposition diseases originate from conformational changes in corresponding amyloidogenic proteins. The accumulated data support the model where protein fibrillogenesis proceeds via the formation of a relatively unfolded amyloidogenic conformation, which shares many structural properties with the pre-molten globule state, a partially folded intermediate first found during the equilibrium and kinetic (un)folding studies of several globular proteins and later described as one of the structural forms of natively unfolded proteins. The flexibility of this structural form is essential for the conformational rearrangements driving the formation of the core cross-beta structure of the amyloid fibril. Obviously, molecular mechanisms describing amyloidogenesis of ordered and natively unfolded proteins are different. For ordered protein to fibrillate, its unique and rigid structure has to be destabilized and partially unfolded. On the other hand, fibrillogenesis of a natively unfolded protein involves the formation of partially folded conformation; i.e., partial folding rather than unfolding. In this review recent findings are surveyed to illustrate some unique features of the natively unfolded proteins amyloidogenesis.

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Section: Calcitonin and Medullary Thyroid Cancermentioning

confidence: 99%

Amyloidogenesis of Natively Unfolded Proteins

Uversky

2008

CAR

174

152

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“…In such circumstances, 10-and 20-year disease-free survival rates of our MTC series were 89 and 82.5%, respectively, and cause-specific survival rates were 96.6 and 91.7%, respectively [18]. These survival rates were better than those reported in Western countries [3,4,[10][11][12][13][14][19][20][21][22][23]. In our series, extrathyroid extension, lymph node metastasis, and a large tumor size were regarded as prognostic factors, while gender, age, and RET gene mutations (except for MEN 2B) did not affect patients' prognoses [18].…”

Section: Evaluation Of Stage Of Mtc Patientsmentioning

confidence: 71%

Validity of 6<sup>th</sup> edition of UICC TNM classification system for medullary thyroid carcinoma: A proposal for intraoperative evaluation of T category

et al. 2012

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“…Sinai study showed 5-, 10-and 20-year overall survival rates as 97, 88 and 84%, respectively. Disease-free survival rates were 97, 74 and 29% at 5, 10 and 20 years, respectively (20).…”

Section: Discussionmentioning

confidence: 98%

Medullary thyroid carcinoma: The third most common thyroid cancer reviewed

et al. 2010

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Abstract. Medullary thyroid cancer is a type of thyroid cancer of neuroendocrine origin. It occurs in hereditary and sporadic forms, and its aggressive behavior is associated with the clinical presentation and type of RET mutation. Total thyroidectomy remains the ideal choice of treatment. Early diagnosis and treatment are the fundamental for a 100% cure rate. In this study, we present our experience of 3 cases, along with a complete review of the literature derived from a Pubmed Database search. IntroductionThyroid cancer represents approximately 2% of the malignancies occurring in the US, accounting for an estimated 37,200 cancer diagnoses and 1,630 cancer-related deaths per year (1). Of these cancers, 2-3% are medullary thyroid cancer (MTC) (2,3). In the literature, sporadic carcinomas are described in 48-86% of patients and hereditary carcinomas in 14-52% of patients with MTC (4). In the familial forms of MTC, multicentric carcinomas were reported in 56-85% of patients. Both sporadic and hereditary types of MTC metastasize to the cervical lymph nodes in 68-80% of patients. The 5-and 10-year survival for medullary carcinomas is 65-89% and 71-87%, respectively (5). Average survival for MTC is lower than that for more common thyroid cancers, e.g., 83% 5-year survival for MTC compared to 90-94% 5-year survival for papillary and follicular thyroid cancer (6). Survival is correlated with stage at diagnosis, and decreased survival in MTC can be partly accounted for by a high proportion of late-stage diagnoses (7). A Surveillance, Epidemiology and End Results (SEER) population-based study of 1,252 MTC patients found that survival varied by the extent of local disease. For example, the 10-year survival rates ranged from 95.6% for disease confined to the thyroid gland to 40% for patients with distant metastases (8).MTC arises from parafollicular calcitonin-secreting cells of the thyroid gland. MTC occurs in sporadic and familial forms and may be preceded by C-cell hyperplasia (CCH), although CCH is a relatively common abnormality in middle-aged adults. In a population-based study in Sweden, 26% of patients with MTC presented with the familial form (9). A French national registry and a US clinical series both reported a higher proportion of familial cases (43 and 44%, respectively) (10). Familial cases often indicate the presence of multiple endocrine neoplasia type 2, a group of autosomal dominant genetic disorders caused by inherited mutations in the RET proto-oncogene.MTC is a malignancy arising from the parafollicular or C-cells of neuroendocrine origin. MTC was first described by Jaquet in the German literature as ʻmalignant goiter with amyloidʼ (11). C-cells are named due to their calcitonin hormone secretion and account for up to 1% of thyroid cells. These cells are found throughout the thyroid gland but are mostly located in the posterior upper third of the lateral lobes, where the majority of MTCs are found. C-cells also produce carcinoembryonic antigen (CEA) (12).MTCs usually have a slow growth rate and appea...

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Prognostic Variables and Calcitonin in Medullary Thyroid Cancer

Cited by 67 publications

References 15 publications

Amyloidogenesis of Natively Unfolded Proteins

Amyloidogenesis of Natively Unfolded Proteins

Validity of 6<sup>th</sup> edition of UICC TNM classification system for medullary thyroid carcinoma: A proposal for intraoperative evaluation of T category

Medullary thyroid carcinoma: The third most common thyroid cancer reviewed

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