[Medullary thyroid carcinoma: the comparison of the hereditary and sporadic types of cancer].
BACKGROUND
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.


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.
Wygoda
Zbigniew
Z
Department of Nuclear Medicine and Endocrine Oncology, Comprehensive Cancer Center and M. Sklodowska-Curie Memorial Institute of Oncology, Gliwice Branch, Gliwice. zwgoda@poczta.onet.pl
Oczko-Wojciechowska
Małgorzata
M
Gubała
Elzbieta
E
Pawlaczek
Agnieszka
A
Kula
Dorota
D
Wiench
Małgorzata
M
Włoch
Jan
J
D000293:Adolescent; D000328:Adult; D000368:Aged; D018276:Carcinoma, Medullary / Q000145:classification* / Q000175:diagnosis / Q000235:genetics*; D002648:Child; D004252:DNA Mutational Analysis / Q000379:methods; D004273:DNA, Neoplasm; D005260:Female; D020022:Genetic Predisposition to Disease; D006801:Humans; D008297:Male; D008875:Middle Aged; D009377:Multiple Endocrine Neoplasia / Q000145:classification / Q000235:genetics; D017354:Point Mutation / Q000235:genetics; D011518:Proto-Oncogene Proteins / Q000235:genetics*; D051096:Proto-Oncogene Proteins c-ret; D012307:Risk Factors; D013964:Thyroid Neoplasms / Q000145:classification* / Q000175:diagnosis / Q000235:genetics*
D004273:DNA, Neoplasm; D011518:Proto-Oncogene Proteins; D051096:Proto-Oncogene Proteins c-ret