Review ArticleLeydig cell hyperplasia in children: Case series and review☆
Introduction
Leydig cell hyperplasia (LCH) is rare and difficult to differentiate clinically from a Leydig cell tumour (LCT) [1], [2], [3], [4]. It is also uncertain whether LCH represents a precursor to LCT formation or whether it may be a separate, benign entity, thus making management decisions difficult [5]. In children, both LCH and LCT may present with precocious puberty and a testicular mass, and can only be differentiated by histopathology [6], [7]. Differential diagnoses of primary LCT include congenital adrenal hyperplasia, virilizing adrenal tumour, McCune–Albright syndrome, human chorionic gonadotrphin (hCG)-secreting tumour and activating LHR mutations [8], [9], [10].
Historically, orchidectomy was routinely performed in all children presenting with LCT or LCH, however, as both are almost certainly benign conditions in children; more recently testis-sparing surgery has been performed with promising results [1], [3], [9]. Here we present a series of unusual clinical presentations of LCH at two tertiary children's hospitals, over a 5-year period, with a review of the literature regarding LCH and LCT in children.
Section snippets
Case series
See Table 1 for biochemistry and histopathology results of cases.
Methods
We performed a literature search using Ovid Medline, PubMed, and Google Scholar. The search terms employed were “Leydig cell hyperplasia,” “Leydig cell tumour,” “Leydig cell adenoma,” “precocious puberty,” and “paediatric/pediatric testicular tumours.” The search produced 456 articles, from which we reviewed case reports and case series containing paediatric patients, and appropriate review articles. There were no randomized control trials identified. We excluded a large number of animal
Results
See Table 2 for a summary of results.
Discussion
We have described three presentations of LCH; two were incidental findings on ultrasound examination, a presentation not previously described in the literature. One of these two underwent surveillance over a 2-year period before excisional biopsy was performed. This patient remained symptom-free over this period, suggesting that neoplastic transformation is unlikely. However, it is uncertain what would have been the natural progression of these two patients if the mass had remained undiagnosed,
Conclusion
LCH in prepubertal children occurs rarely and usually presents in an identical fashion to LCTs. Testis-sparing surgery with regular follow-up should be considered for LCH and LCTs in a prepubertal population.
Funding
None.
Conflicts of interest
None.
References (40)
- et al.
Stromal testis tumors in children: a report from the prepubertal testis tumor registry
J Urol
(2001) - et al.
Testicular tumors in children and adolescents
J Ped Urol
(2008) - et al.
Testicular and paratesticular neoplasms in prepubertal males
J Urol
(2006) - et al.
Leydig cell hyperplasia and adenoma formation: mechanisms and relevance to humans
Reprod Toxicol
(1997) Prepubertal testicular tumor registry
J Urol
(1993)- et al.
Re: Long-term follow-up and clinical characteristics of testicular Leydig cell tumor: experience with 24 cases
J Urol
(2007) - et al.
Nodular Leydig cell hyperplasia in a boy with familial male-limited precocious puberty
J Pediatr
(2001) - et al.
Focal lobular spermatogenesis and pubertal acceleration associated with ipsilateral Leydig cell hyperplasia
Urology
(2000) - et al.
Testicular tumors in children
J Pediatr Surg
(2001) Testis-sparing surgery for benign lesions of the prepubertal testis
Urol Clin North Am
(1993)
Leydig cell tumor in a child with spermatocyte maturation and no pseudoprecocious puberty
Urology
Prepubertal testis tumors: actual prevalence rate of histological types
J Urol
Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the Prepubertal Testis Tumor Registry
J Urol
Leydig cell hyperplasia
Br J Urol
Testicular tumors in children
Am J Surg
Leydig cell tumor and hyperplasia: a review
Anal Quant Cytol Histol
Male LH-independent sexual precocity in a 3.5-year-old boy caused by a somatic activating mutation of the LH receptor in a Leydig cell tumor
J Clin Endocrinol Metab
Testis-sparing surgery for benign testicular tumors in children
J Urol
Testicular Leydig's cell hyperplasia: MR imaging and sonographic findings
AJR Am J Roentgenol
Venous sampling can be crucial in identifying the testicular origin of idiopathic male luteinising hormone-independent sexual precocity
Eur J Pediatr
Cited by (0)
- ☆
Presented as a poster presentation at the Pacific Association of Pediatric Surgeons Annual Conference 2013.