[Results of treating medullary thyroid carcinoma: the differences between sporadic and inherited forms].
Medullary thyroid carcinoma (MTC) can be divided into two subgroups: sporadic or inherited. Hereditary form of MTC is often believed to be form with better prognosis than sporadic one. In this study the differences in MTC prognosis in Polish population of patients was analyzed. The group of 169 patients with MTC was examined. Hereditary cancer was stated in 48 (28%) patients. The median age of disease onset was 41 years (from 7 to 71 years). Genetic examination of RET protooncogene was performed in all patients. The calcitonin and CEA serum level analysis and radiological and radioisotopic examinations were used for monitoring of the disease course. Nineteen cases of MEN 2A syndrome, 11 cases of MEN 2B one and 18 cases of non classified familial MTC were recognized among patients with inherited MTC. Significantly lower age of disease onset in inherited MTC than in sporadic one was observed (27 years vs. 43.7 years, p < 0.001). Local or nodal recurrence was observed in 22 (13%) patients, distant metastases were stated in 21 (12%) patients. Basal or stimulated serum calcitonin level was increased in 85 (50%) patients. No significant differences between sporadic and inherited disease were observed. Eight patients died during observation, including 3 patients with sporadic MTC and 5 patients with inherited MTC. The updated 10-year survival rate was 97% in patients with sporadic MTC; in hereditary MTC it was about 20% worse. The complications related to the presence of adrenal tumors were the main reason for death in MEN2 and no significant differences in the course of MTC itself were observed.
Zakładu Medycyny Nuklearnej i Endokrynologii Onkologicznej, Centrum Onkologii-Instytut im. M. Skłodowskiej-Curie w Gliwicach.
Z
Z
Wygoda
J
J
Włoch
E
E
Gubała
M
M
Wiench
B
B
Jarzab
D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000368:Aged; D018276:Carcinoma, Medullary / Q000145:classification* / Q000453:epidemiology / Q000235:genetics / Q000628:therapy*; D002648:Child; D029721:Drosophila Proteins; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D018813:Multiple Endocrine Neoplasia Type 2a / Q000235:genetics / Q000628:therapy; D018814:Multiple Endocrine Neoplasia Type 2b / Q000235:genetics / Q000628:therapy; D011044:Poland; D011379:Prognosis; D011518:Proto-Oncogene Proteins / Q000235:genetics; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases / Q000235:genetics; D015996:Survival Rate; D013964:Thyroid Neoplasms / Q000145:classification* / Q000235:genetics / Q000628:therapy*
D029721:Drosophila Proteins; D011518:Proto-Oncogene Proteins; D051096:Proto-Oncogene Proteins c-ret; D020794:Receptor Protein-Tyrosine Kinases; C416649:Ret protein, Drosophila