Ten-Year Follow-Up in a Boy with Leydig Cell Tumor after Selective Surgery

Abstract

In a 4⁸/₁₂-year-old boy with precocious puberty and an enlarged right testis, a Leydig cell tumor was diagnosed. Surgical exploration revealed an encapsulated tumor which was selectively removed without orchiectomy. Within 1 year the signs of precocious puberty disappeared. Ten years later, the patient remained free of disease and had developed normal spontaneous puberty. Despite of highly advanced bone age at the time of diagnosis (13 years according to Greulich and Pyle), his height at age 15 was in the upper normal range and within the familial target height. Most of these prepubertal patients affected by this tumor underwent orchiectomy, although no malignant course of Leydig cell tumors before puberty has been reported. This work provides the first example of long-term follow-up of a prepubertal boy after testis-sparing surgery for a Leydic cell tumor. We conclude that selective removal of the tumor may be the procedure of choice in this entity.

Bilateral Leydig cell tumor of the testis: a

“…Inguinal orchiectomy should be performed with early control of the spermatic cord and without violation of the scrotal skin. Testis sparing surgery with enucleation of the mass has been reported in children and younger adults in order to maintain fertility [13–15]. Bilateral radical orchiectomy was performed in our case; because both tumors originated from the mediastinum testis, testis sparing surgery could not be performed.…”

Leydig-Cell Tumour in Children: Variable Clinical Presentation, Diagnostic Features, Follow-Up and Genetic Analysis of Four Cases

“…However, as testosterone is normally synthesized by Leydig-cells, and they are likewise capable to testosterone synthesis when they undergo neoplasia, this finding is not surprising. Testis-sparing enucleation of the tumour was performed in these patients [23] and the diagnosis of Leydig-cell tumour was confirmed by histological examination in all four subjects (fig. 1b).…”

“…Thirdly, not only age but also pubertal stage related, laboratory specific normative data should be used. Further, testes sparing enucleation of a Leydig-cell tumour seems to be sufficient in childhood, but the boy’s scrotum and further development needs ongoing follow-up as rarely a malignant process may occur in the future [23]. In addition, considering the previously reported mutations found in a few of Leydig-cell tumours [3,4,5,6,7,8,9,10,11], the absence of any activating mutations in LHR , as well as in both the ‘hot spot’ regions within the G-alpha subunits ( gsp and gip2 ) and in the regulatory ‘hot spot’ of the CDK4 genes in our patients, indicates molecular heterogeneity among Leydig-cell tumours, corresponding to its highly variable phenotype, which has not been reported previously.…”

Gonadotropin-Dependent Precocious Puberty in an 8-Year-Old Boy with Leydig Cell Testicular Tumor

“…Our patient was treated with testis-sparing surgery, according to the practices that have been favored in recent reports [1,8,15,16]. It is reasonable to consider a direct enucleation of the tumor with sparing of the testis when normal testicular tissue volume surrounding the mass is acceptable, mainly in prepubertal patients [1,8,15,16].…”

“…It is reasonable to consider a direct enucleation of the tumor with sparing of the testis when normal testicular tissue volume surrounding the mass is acceptable, mainly in prepubertal patients [1,8,15,16]. In general, after the removal of the tumor, there is normal spermatogenesis and fertility is not affected in the long term [17].…”