[Gonadoblastoma in an HY-negative pure XY-gonadal dysgenesis (author's transl)]

A case is reported of a gonadoblastoma in a pure XY-gonadal dysgenesis which was HY antigen negative. The salient clinical features were amenorrhoea and tall stature. The FSH was markedly increased, combined with low E2 and testosterone values. Cytogenetically a 46-XY karyotype was found. The HY antigen titre was in a male level. The gonadal streaks which were removed showed microscopically islands of tumour cells with 2 cell lines which were interpreted as germ cells and granulosa cells. Newer concepts of the influence of the HY antigen on the differentiation of the testicles and differentiation of the ovaries are discussed. Treatment consists of removal of the gonadal streaks and estrogen treatment.