A. Adesokan et al., TESTICULAR-TUMOR OF THE ADRENOGENITAL SYNDROME - A CASE-REPORT OF AN UNUSUAL ASSOCIATION WITH MYELOLIPOMA AND SEMINOMA IN CRYPTORCHIDISM, Cancer, 80(11), 1997, pp. 2120-2127
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.