Leydig cell tumor of the testis with azoospermia and elevated delta4 androstenedione: case report.

C. Leroy,A. Derveaux,P. Puech,J-M. Rigot,J. Prasivoravong,F. Marcelli,X. Leroy,V. Mitchell,A-L. Barbotin

doi:10.1186/s12610-016-0041-8

Abstract

BackgroundSecreting interstitial cell (Leydig cell) tumors are rare. In adults, the clinical picture and steroid levels are variable.Case presentationThis paper presents a case of left testicular tumor, showing azoospermia with normal serum level of total testosterone, collapsed FSH and LH, and high delta4 androstenedione. Histopathological investigation revealed a Leydig cell tumor. TESE allowed spermatozoa extraction and freezing. Testicular histology found hypospermatogenesis and germ-cell aplasia with interstitial fibrosis. Surgical resection of the tumor resulted in normalization of gonadotropins and fall in serum delta4 androstenedione to subnormal levels in the postoperative period confirming that the tumor was secreting delta4 androstenedione. It was hypothesized that high delta4 androstenedione resulted in intra tumoral 17 β-HSD overtaken by delta4 androstenedione or that 17 β-HSD activity in the tumor was different from that of normal Leydig cells. Three months after surgery sperm analysis found a complete recovery of spermatogenesis. A spontaneous pregnancy occurred 3 months after surgery and a girl was born.ConclusionsIn this case, the diagnosis of testicular Leydig cell tumor secreting delta4 androstenedione was made in a context of azoospermia.

Highlights

Les tumeurs testiculaires interstitielles à expression endocrine sont rares
This paper reports a case of testicular Leydig cell tumor secreting delta[4] androstenedione in a patient with azoospermia
Total suppression of Luteinizing hormone (LH) levels and almost complete suppression of Follicle stimulating hormone (FSH) levels were associated with azoospermia, despite intratesticular testosterone secretion by the tumor, and azoospermia proved to be fully reversible within less than three months after tumor removal and normalization of hormone levels, which shows that normal secretion of gonadotropins, plays a major role in maintaining spermatogenesis

Summary

Introduction

Les tumeurs testiculaires interstitielles (ou tumeurs testiculaires à cellules de Leydig) à expression endocrine sont rares. Chez l’adulte le tableau clinique et le bilan hormonal sont variables. Présentation du cas: Cet article présente le cas d’une tumeur testiculaire gauche dans un contexte d’azoospermie. Le bilan hormonal montre des gonadotrophines effondrées, une testostéronémie normale et une delta[4] androstenedione augmentée. L’examen anatomopathologique a mis en évidence une tumeur à cellule de Leydig. Dans les suites de l’orchidectomie partielle gauche les taux de gonadotrophines se sont normalisés ainsi que le taux de delta[4] androstenedione. L’hypothèse physiopathologique est que l’augmentation de la delta[4] androstenedione résulte de la sursaturation de la 17 β-HSD intra-tumoral ou que l’activité de la 17 β-HSD intra-tumoral est différente de celle dans les cellules de Leydig normales. Trois mois après la chirurgie, le spermogramme a montré une normalisation des paramètres spermatiques et une grossesse spontanée est survenue permettant la naissance d’une petite fille

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