TESTICULAR-TUMOR OF THE ADRENOGENITAL SYNDROME - A CASE-REPORT OF AN UNUSUAL ASSOCIATION WITH MYELOLIPOMA AND SEMINOMA IN CRYPTORCHIDISM

BACKGROUND. Males with congenital adrenal hyperplasia may develop bila teral testicular masses in early adult life. These are not malignant a nd generally regress with corticosteroid therapy. The authors report a case occurring in a 44-year-oId man with associated seminoma and myel olipoma in an undescended testis. METHODS. The testicular tumors were analyzed by histologic, flow cytometric, and ultrastructural technique s. RESULTS. The tumors in both testes were comprised of polygonal cell s with abundant granular eosinophilic cytoplasm, occasionally with bro wn (lipochrome) pigment and round nuclei of various sizes with promine nt nucleoli. These cells were grouped into nodules by dense and someti mes thick fibrous trabeculae in the right testis. The areas correspond ing to the fibrous trabeculae in the left (intraabdominal) testis were replaced by mixture of hematopoietic (myeloid) and fatty tissue in va rious proportions characteristic of myelolipoma. The left testis also had a well demarcated tumor that was diagnostic of seminoma. Electron microscopy demonstrated abundant smooth endoplasmic reticulum, a moder ate number of mitochondria with tubulovesicular cristae, lipid droplet s, and lipofuscin granules in the polygonal cells. No Reinke's crystal s were observed. The patient received corticosteroids for his adrenoco rticoid deficiency and also underwent external beam irradiation to the retroperitoneum for seminoma. CONCLUSIONS. This case illustrates an u nusual presentation of a testicular tumor in a patient with the adreno genital syndrome as well as with myelolipoma and seminoma in a cryptor chid testis. The possibility of an associated neoplasm that could be p otentially fatal should be considered whenever a testicular tumor of t he adrenogenital syndrome continues to grow despite adequate hormonal treatment. (C) 1997 American Cancer Society.