Dysgerminoma of the Ovary with Widespread Metastases

Abstract

In 1931, Meyer first applied the term “dysgerminoma” to the ovarian counterpart of the testicular seminoma. Although there are approximately 200 cases reported in the literature up to 1939 (1), the dysgerminoma is a relatively uncommon ovarian neoplasm, its frequency being variously reported as 3 to 10 per cent of primary malignant tumors of the ovary (1, 5, 6, 7). It appears typically at an early age, becoming manifest most often in the second to fourth decades, and is characterized by a progressively enlarging pelvic mass which may reach large proportions. When this finding is associated with pseudohermaphroditism or other genital maldevelopment, the diagnosis should be strongly suspected. Novak (5), however, has pointed out that more than one-half of his patients had normal genital development. Various degrees of ascites may be present in about 10 per cent of patients (7). The tumor is believed to be hormonally indifferent (3, 5), in conformity with Meyer's concept of its origin from “an undifferentiated form of germinal cells which have lost their faculty of becoming either masculine or feminine in type” (4). On the other hand, careful hormone studies have not been carried out in a sufficient number of patients to determine subclinical amounts of hormone production. This has been emphasized by Seegar (7). The tumor is grossly rubbery or brainlike in consistency, and microscopically is made up of uniform cells separated into groups by fibrous septa which usually contain lymphoid cells. In some specimens there are scattered microscopic granulomatous nodules resembling tubercles. The degree of malignancy cannot be judged from the microscopic appearance and is determined best at operation by the presence or absence of invasion or metastasis. According to Novak (5), treatment should consist of removal of the involved ovary and adnexa. The opposite ovary should be removed only if involved. Opinion in the literature differs on the incidence of bilateral occurrence: Seegar (7) states that 20 per cent are bilateral, while Dockerty and MacCarty (1) give a higher figure, 35 per cent. The tumor is radiosensitive, and radiotherapy should play an important role in the management of recurrence and metastases (2, 5, 8). The tendency of the testicular seminoma to widespread and early metastasis is well known. The dysgerminoma of ovarian origin, on the other hand, tends to grow to large dimensions and to invade locally the pelvic structures. Distant metastases are rare and, when they occur, often are confined to the abdominal viscera. Seegar (7) states that in his review of 79 cases reported in the literature and in 19 of his own patients there were only 3 with metastases and these were all within the abdomen (kidney, pancreas, spleen, and liver). The overall five-year survival rate as reported by Seegar (7) is 50 per cent.