open access

Vol 57, No 4 (2006)
Original papers
Published online: 2006-07-07
Submitted: 2013-02-15
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Medullary thyroid carcinoma: the comparison of the hereditary and sporadic types of cancer

Zbigniew Wygoda, Małgorzata Oczko-Wojciechowska, Elżbieta Gubała, Agnieszka Pawlaczek, Dorota Kula, Małgorzata Wiench, Jan Włoch
Endokrynologia Polska 2006;57(4):407-414.

open access

Vol 57, No 4 (2006)
Original papers
Published online: 2006-07-07
Submitted: 2013-02-15

Abstract

Introduction: The assessment of frequency and type of mutation and differences in prognosis between sporadic and hereditary type of medullary thyroid carcinoma (MTC), based on own DNA analysis, was performed.
Material and methods: The group of 190 persons with hereditary MTC or asymptomatic mutation carriers was analyzed. Patients with sporadic MTC without RET gene mutation were included into control group (708 persons). The recognition of MTC type was based on assessment of family history, physical examination and genetic analysis. The family history consisted of information about MTC, pheochromocytoma and other neoplasms and hyperparathyroidism in relatives.
Results: The mutations located in codon 634 of exon 11 were the most often (43% of all mutations and 49% of mutations in syndrome MEN 2A/FMTC). The age of diagnosis was ranged between 7 and 71 years (mean age: 39 ± 15.2 years, median age: 41 years). In hereditary MTC the mean age of diagnosis was 27 ± 13.9 years and was significantly lower than in sporadic one, where it was 45.7 ± 14.3 years. The relationship between diagnosis, age and subtypes of hereditary MTC was assessed - no significant differences in examined subgroups were observed. The mean age of diagnosis in MEN 2A/FMTC and MEN 2A syndrome was 28-29 years, in MEN 2B - 21 years. The overall survival in sporadic MTC after 5 years was 97%, in hereditary MTC - 79%. Analysis performed after excluding suprarenal causes of death revealed no statistically significant differences in overall survival between both subtypes of MTC.
Conclusions: 1. Hereditary MTC is still diagnosed too late, besides of DNA analysis.
2. In hereditary and sporadic MTC the prognosis is comparable.

Abstract

Introduction: The assessment of frequency and type of mutation and differences in prognosis between sporadic and hereditary type of medullary thyroid carcinoma (MTC), based on own DNA analysis, was performed.
Material and methods: The group of 190 persons with hereditary MTC or asymptomatic mutation carriers was analyzed. Patients with sporadic MTC without RET gene mutation were included into control group (708 persons). The recognition of MTC type was based on assessment of family history, physical examination and genetic analysis. The family history consisted of information about MTC, pheochromocytoma and other neoplasms and hyperparathyroidism in relatives.
Results: The mutations located in codon 634 of exon 11 were the most often (43% of all mutations and 49% of mutations in syndrome MEN 2A/FMTC). The age of diagnosis was ranged between 7 and 71 years (mean age: 39 ± 15.2 years, median age: 41 years). In hereditary MTC the mean age of diagnosis was 27 ± 13.9 years and was significantly lower than in sporadic one, where it was 45.7 ± 14.3 years. The relationship between diagnosis, age and subtypes of hereditary MTC was assessed - no significant differences in examined subgroups were observed. The mean age of diagnosis in MEN 2A/FMTC and MEN 2A syndrome was 28-29 years, in MEN 2B - 21 years. The overall survival in sporadic MTC after 5 years was 97%, in hereditary MTC - 79%. Analysis performed after excluding suprarenal causes of death revealed no statistically significant differences in overall survival between both subtypes of MTC.
Conclusions: 1. Hereditary MTC is still diagnosed too late, besides of DNA analysis.
2. In hereditary and sporadic MTC the prognosis is comparable.
Get Citation

Keywords

medullary thyroid carcinoma; RET; MEN 2; FMTC

About this article
Title

Medullary thyroid carcinoma: the comparison of the hereditary and sporadic types of cancer

Journal

Endokrynologia Polska

Issue

Vol 57, No 4 (2006)

Pages

407-414

Published online

2006-07-07

Bibliographic record

Endokrynologia Polska 2006;57(4):407-414.

Keywords

medullary thyroid carcinoma
RET
MEN 2
FMTC

Authors

Zbigniew Wygoda
Małgorzata Oczko-Wojciechowska
Elżbieta Gubała
Agnieszka Pawlaczek
Dorota Kula
Małgorzata Wiench
Jan Włoch

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