Cytogenetic and Clinical Study on 100 Cases of Primary Amenorrhoea

Clinical, histochemical, and biochemical studies of an ovarian dysgerminoma with trophoblasts and Leydig cells

A clinico‐pathological study of gonadoblastoma

A chance finding of structures resembling gonadoblastomas in the ovaries of a child with lissencephaly prompted a detailed review of all ovarian histology obtained at autopsy over a 12 month period. Fifty-five stillbirths, infants and children were studied ranging from 20 weeks gestational age to 2.5 years post-natal age. In 19 infants structures mimicking gonadoblastomas and sex cord tumours with annular tubules were seen. In all but one case these structures were found in association with follicular cysts and they closely resembled the atretic follicles often seen in the stroma surrounding the follicular cysts. They differed from the atretic follicles only by virtue of their being larger. In addition, in several infants structures resembling Sertoli cell tubules or clusters of Leydig cells were found. When present, these structures always co-existed with sex cord tumours with annular tubules and gonadoblastoma-like lesions. The abnormal stromal lesions and follicular cysts were found most frequently at the stage of development when a massive 'physiological' reduction of oocytes occurs. It is suggested that the 'abnormal' structures identified in this report represent the 'first hit' of oncogenesis and could serve as the precursor of many of the sex cord-stromal tumours, and possibly germ cell neoplasms, seen in childhood.

Structures mimicking sex cord‐stromal tumours and gonadoblastomas in the ovaries of normal infants and children

One hundred malignant mixed germ cell tumors of the ovary that occurred in patients 3 to 55 years (mean: 20 y) of age are described. The clinical presentation was usually that of any highly malignant tumor of the ovary (abdominal pain and distension), but rarely (3 cases) endocrine manifestations were present. The tumors were usually unilateral (96%), ranged from 4 to 38 cm (mean: 16 cm), and were uniformly solid or, more often, solid and cystic; occasionally the typical appearance of dysgerminoma could be appreciated. The most common tumor type was yolk sac tumor (91%), followed by dysgerminoma (61%), immature teratoma (58%), embryonal carcinoma (38%), and choriocarcinoma (11%). A variety of admixtures were encountered; dysgerminoma and yolk sac tumor was the most common combination (25% of the tumors) with the 2 components often being sharply demarcated. Immature teratoma and yolk sac tumor was the next most common pairing (20%) followed by yolk sac tumor and embryonal carcinoma, with or without immature teratoma (16%). Tumors with a choriocarcinoma component had the most varied combinations of tumor types. Embryoid bodies were seen in 21% of the tumors, most often as fragmented forms arranged in a nodular manner with yolk sac tumor and/or embryonal carcinoma; uncommonly they occurred singly or in clusters. Numerous confluent well-formed embryoid bodies (polyembryoma) were prominent in 2 tumors. Three tumors had a focal diffuse embryoma pattern. The specific tumor types showed the known diverse spectrum of microscopic appearances, but the frequent haphazard arrangement of 2 or more subtypes often resulted in complex morphology. Overgrowth of another neoplastic component, most often primitive neuroectodermal tumor, occurred in 10% of the tumors further complicating the histologic picture. This is the largest series of ovarian malignant mixed germ cell tumors reported and details their characteristics including associations of their subtypes and the frequent apparent role of embryoid bodies in giving rise to yolk sac tumor and embryonal carcinoma components.

V. Grizelj

Papers

Precocious puberty with masculinization due to terato‐chorio‐gonadoblastoma

Primary hypogonadotrophic hypogonadism in women

Primary hypogonadotrophic hypogonadism in women