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Medullary thyroid carcinoma: the comparison of the hereditary and sporadic types of cancer
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Abstract
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
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.
Keywords
medullary thyroid carcinoma; RET; MEN 2; FMTC
About this articleTitle
Medullary thyroid carcinoma: the comparison of the hereditary and sporadic types of cancer
Journal
Issue
Article type
Original paper
Pages
407-414
Published online
2006-07-07
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561
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1542
Bibliographic record
Endokrynol Pol 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
