Review Article
Leydig cell hyperplasia in children: Case series and review

https://doi.org/10.1016/j.jpurol.2016.12.028Get rights and content

Summary

Introduction

Leydig cell hyperplasia (LCH) and Leydig cell tumours (LCTs) in children are rare, typically presenting with precocious puberty. Previously, orchidectomy was the routine management; however, more recently, testis-sparing surgery has been performed with good results. We present a series of unusual presentations of LCH, raising new management questions, and a review of the literature regarding LCH and LCT in children.

Study design

We performed a literature search using Ovid Medline, PubMed, and Google Scholar, producing 456 articles. We reviewed all case reports and series containing paediatric patients, and relevant review articles.

Results

We report three cases of LCH, two of which were incidental findings. All three cases underwent testis-sparing surgery. In the literature there were seven cases of LCH and 101 cases of LCT in prepubertal children. The most common presentation was with precocious puberty. Three cases of LCH and more than two-thirds of LCTs were managed with orchidectomy and overall only 11% of the cases underwent testes-sparing surgery (24% did not specify operative management). There were no reports of recurrence or malignancy.

Discussion

Our case series presents three new clinical presentations of LCH that have not previously been reported in the literature: one of incomplete precocious puberty and two with incidental findings on ultrasound in asymptomatic children. Historically, children with the classic presentation of precocious puberty and a testicular lesion have been managed with orchidectomy. Nowadays, many clinicians advocate testes-sparing surgery given there have been no cases of malignancy. In children with no clinical or biochemical signs of precocious puberty, lesions identified on ultrasound can be safely monitored for a period of time. However, if the lesion does not regress, excisional biopsy is recommended to establish the diagnosis, ideally before the onset of puberty.

Conclusion

Leydig cell hyperplasia and tumours in pre-pubertal children are benign. Testes-sparing surgery with regular follow-up appears to be safe management.

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)

  • S.K. Polepalle et al.

    Leydig cell tumor in a child with spermatocyte maturation and no pseudoprecocious puberty

    Urology

    (2003)
  • H. Pohl et al.

    Prepubertal testis tumors: actual prevalence rate of histological types

    J Urol

    (2004)
  • J.H. Ross et al.

    Clinical behavior and a contemporary management algorithm for prepubertal testis tumors: a summary of the Prepubertal Testis Tumor Registry

    J Urol

    (2002)
  • C.K. Naughton et al.

    Leydig cell hyperplasia

    Br J Urol

    (1998)
  • R. Houser et al.

    Testicular tumors in children

    Am J Surg

    (1965)
  • M. Colecchia et al.

    Leydig cell tumor and hyperplasia: a review

    Anal Quant Cytol Histol

    (2007)
  • A. Richter-Unruh et al.

    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

    (2002)
  • J.S. Valla

    Testis-sparing surgery for benign testicular tumors in children

    J Urol

    (2001)
  • L.R. Carucci et al.

    Testicular Leydig's cell hyperplasia: MR imaging and sonographic findings

    AJR Am J Roentgenol

    (2003)
  • A. Richter-Unruh et al.

    Venous sampling can be crucial in identifying the testicular origin of idiopathic male luteinising hormone-independent sexual precocity

    Eur J Pediatr

    (2002)
  • Cited by (0)

    Presented as a poster presentation at the Pacific Association of Pediatric Surgeons Annual Conference 2013.

    View full text