Steroid Hormone-Producing Tumors in Man

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Abstract
TUMORS derived from steroid hormone-producing cells of the adrenal, ovary, and testes are rare. The tumors are, however, important both as causes of disease entities and as useful model systems for the study of steroidogenesis. Certain features of steroidogenic tumors complicate the diagnosis, prognosis, and treatment. Steroidogenic tumors frequently are neither suspected nor detected until they are very large or metastatic. This sometimes occurs because the tumor does not produce a recognizable endocrine syndrome. Even when the tumor produces a distinct syndrome, the anatomical location of the tumor may not be inferred from steroid hormone secretory patterns or trophic hormone responsiveness. Furthermore, tumors of the adrenal (1–9) or testes (10) may cause Cushing's Syndrome, and virilization or feminization syndromes may be caused by adrenal (11, 12), testicular (13–19), or ovarian tumors (20–36). Some of these tumors respond to trophic hormone stimulation, either to the appropriate trophic hormone for the site of origin of the tumor (9, 19, 23, 25, 34) or to other trophic hormones (11, 23). After discovery, localization, and resection of a steroidogenic tumor, no characteristic of the patient or tumor will define the prognosis, and no histological definition of malignancy exists for these tumors (2–8, 16, 20, 22). Chemotherapy of tumors that cannot be completely removed by surgery is usually toxic and only marginally effective.

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