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Occurrence of tumors in dysgenetic gonads
Author(s) -
Teter Jerzy,
Boczkowski Krzysztof
Publication year - 1967
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/1097-0142(196708)20:8<1301::aid-cncr2820200814>3.0.co;2-4
Subject(s) - gonadoblastoma , dysgerminoma , gonadal dysgenesis , turner syndrome , gonad , medicine , karyotype , seminoma , y chromosome , somatic cell , gynecology , pathology , physiology , endocrinology , ovary , biology , chromosome , genetics , chemotherapy , gene
The occurrence of gonadal tumors was studied in 51 patients with gonadal dysgenesis. The patients have female legal sex and complained of primary amenorrhea. They were classified into two main groups: Turner's syndrome—25 cases; and gonadal dysgenesis (without somatic malformations)—26 cases. Of 25 cases of Turner's syndrome two cases of gonadal tumors were found—one gonadoblastoma and one interstitioma. Of 26 cases of gonadal dysgenesis without somatic malformations eight tumors were found—dysgerminoma (seminoma) in four cases, gonadoblastoma in three cases and a Brenner tumor in one. In all ten cases with gonadal tumors a negative sex chromatin pattern was found. Cytogenetic analysis revealed a normal male 46/XY karyotype in seven cases and in one case a 45/XO//46/XY mosaic was present. In all ten cases high secretion of gonadotrophins was encountered. The authors conclude that persistent germ cells from the embryonic stage, within malformed testicular elements or within so‐called bisexual formations play an important role in pathogenesis. The authors point to the danger of gonadal neoplasm in patient with female phenotype, primary amenorrhea, signs of masculinization and presence of a negative sex chromatin pattern and a Y chromosome.