Sertoli cell proliferations of the infantile testis: an intratubular form of Sertoli cell tumor?

VENARA M; REY RA; BERGADÁ I; MENDILAHARZU H; CAMPO SM; CHEMES H

AMERICAN JOURNAL OF SURGICAL PATHOLOGY

LIPPINCOTT WILLIAMS & WILKINS

Lugar: Philadelphia; Año: 2001

0147-5185

We report on six boys with intratubular Sertoli cell proliferations(ISCPs), studied by routine histologic methods, electronmicroscopy, and immunohistochemistry of anti-müllerian hormone(AMH), inhibin -subunit, 3-hydroxysteroid dehydrogenase(3-HSD), proliferative cellular nuclear antigen, andp53, and carefully followed for extended periods with periodicclinical examinations, testicular ultrasonographies, and determinationsof serum levels of AMH and inhibin B. Peutz?Jeghers syndrome was found in four of six patients, and gynecomastiaoccurred in five of six patients. One boy had isosexualpseudoprecocity. ISCPs were observed as multiple foci of seminiferoustubules with large and proliferated Sertoli cells replacinggerm cells and limited by the basement membrane.Mitotic figures, atypia, and/or interstitial invasion were notobserved. Bilateral ISCPs were the only pathologic finding inthree patients (patient nos. 1?3) and were associated with amicroscopic tumor that resembled a large-cell calcifying Sertolicell tumor (LCCSCT) in a fourth patient (patient no. 4). In thetwo remaining patients (patient nos. 5 and 6) ISCPs andLCCSCT were found in both testes. Ultrastructural examinationshowed large Sertoli cells, with round nuclei, sparse organelles,and some glycogen. Inhibin -subunit immunolocalizationwas positive in the five patients in whom it was determined(patient nos. 2?6), AMH was positive in those ISCPsassociated with tumors (patient nos. 4?6) and negative in isolatedISCPs (patient nos. 2 and 3); 3-HSD and PCNA werevariable, and p53 was negative in all ISCPs. Patient nos. 1?4have been followed for 2?19 years. One of them is currentlyentering puberty, the other two have already completed pubertyand have testes of normal size, and the remaining one is anadult with clinically normal testes and sperm production. Noneof these patients had evidence of tumor development duringfollow-up as shown by serial ultrasonographies and serum levelsof AMH and inhibin B. Patient nos. 5 and 6 who hadbilateral ISCPs and LCCSCT were orchidectomized andevolved for 2?10 years after surgery without tumor recurrence.The prognostic significance of ISCPs, particularly when theyare the only pathologic finding in a testicular biopsy, is a matterof controversy. Based on the long normal evolution, we recommenda conservative approach to therapy. The bilateraland multicentric character of ISCPs and their association withSertoli tumors and Peutz?Jeghers syndrome suggest that theyrepresent either proliferative lesions with tumorigenic potentialor the intraepithelial stage in the evolution of some testicularSertoli cell tumors.