FEMINIZING SERTOLI-CELL TUMORS IN BOYS WITH PEUTZ-JEGHERS SYNDROME

We report the pathology findings in two cases of multicentric Sertoli cell testicular tumors in two young boys with probable Peutz-Jeghers s yndrome. Four cases of such tumors occurring in boys with Peutz-Jegher s syndrome were previously reported. Each of the two boys reported in this paper had prominent gynecomastia, rapid growth, and advanced bone age. Serum levels of estradiol were markedly elevated. Anti-mullerian hormone was measured in the serum of one of the boys and was in the n ormal range for age. Bilateral orchiectomy was performed in each case because the neoplastic growth would most likely result in sterility, a nd curtailment of height potential was threatened from continued eleva tion of estradiol levels. Microscopically, greatly enlarged seminifero us tubules packed with ovoid Sertoli-like cells were present. Prominen t eosinophilic basement membrane surrounded the tubules and intersecte d between the cells, forming hyalinized ovoid globules and microcalcif ications. Ultrastructure revealed lamination of basement membranes sur rounding adjacent cells, ovoid cells with abundant cytoplasm, and limi ted smooth endoplasmic reticulum. Studies of testicular tumor tissue f rom both cases revealed increased transcription of the aromatase cytoc hrome P450 gene using promoter II, the promoter directing aromatase ex pression in the normal ovary and testis. The levels of transcripts wer e comparable to corpus luteum, thus resulting in increased estrogen sy nthesis. Transcripts specific for placental-type aromatase promoters ( I.1 and I.2) were not detected in significant levels in these tumors.