[Medullary carcinoma of the thyroid gland. Personal experience].
The authors present their experience with the diagnosis and treatment of medullary thyroid carcinoma (MTC) in a group of 175 patients in the records of the RI in Motol. From the above number 106 are alive, 69 of the patients who died were diagnosed at the time of the clinical manifestation of the disease, frequently an advanced stage of the disease. Of the living patients one third was treated by total thyroidectomy after active screening. MTC accounts under our conditions in recent years for 8.6% of all thyroid malignancies. The familial variant with autosomal dominant heredity accounts for 26.5% of the above number. Within the framework of the familial variant the most frequent finding is non-MEN MTC, only 30% MTC are part of the MEN 2A or MEN 2B syndromes. MTC in childhood belongs practically always to the familial variant. At present the authors' group comprises 13 children and adolescents under 18 years, incl. one boy with the MEN 2B syndrome who succumbed to the disease. In 8 children based on active screening total thyroidectomy was indicated, incl. 5 patients were by histological examination only hyperplasia of the C-cells, i.e. a precancerous condition, was detected. After operation repeatedly in all these patients the level of immunoreactive calcitonin is low which indicates an excellent prognosis. The authors draw attention to the importance of active screening in families of all direct relations of the patient where MTC was diagnosed. Complete removal of thyroid tissue at the time of hyperplasia of C-cells or the presence of tumourous microfoci has a marked positive effect on the subsequent fate of the patients.(ABSTRACT TRUNCATED AT 250 WORDS)
Neradilová
M
M
Endokrinologický ústav, Praha.
Nĕmec
J
J
Vlcek
P
P
Soutorová
M
M
Bednár
J
J
D000293:Adolescent; D000328:Adult; D000368:Aged; D002277:Carcinoma / Q000175:diagnosis / Q000235:genetics / Q000628:therapy; D002648:Child; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010375:Pedigree; D013964:Thyroid Neoplasms / Q000175:diagnosis / Q000235:genetics / Q000628:therapy