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

Silva, Rita Santos; Bonito-Vítor, A.; Campos, Miguel; Fontoura, Manuel

doi:10.1159/000358084

Cited by 11 publications

(17 citation statements)

References 24 publications

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“…Leydig cell tumors are sex cord stromal tumors that arise from Leydig cells that produce testosterone ( 4 , 9 ). Testicular tumors are very rare during childhood and represent only 1% of all pediatric solid tumors ( 11 ).…”

Section: Discussionmentioning

confidence: 99%

“…The clinical presentation of Leydig tumors is characterized by isosexual precocious pseudopuberty caused by increased production of androgens, mostly testosterone, and low gonadotropin levels. These patients develop secondary sexual characteristics in the absence of hypothalamic–pituitary activation ( 4 – 9 ). Previously, treatment for these lesions was a radical orchiectomy with lymphadenectomy when regional lymph nodes were involved ( 11 ).…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

“…Central precocious puberty is caused by premature activation of the hypothalamic–pituitary–gonadal axis, while precocious pseudopuberty results from peripheral over production of sex steroids ( 1 – 9 ). Leydig cell tumors are the most common hormone-secreting testicular tumors and are an unusual cause of precocious pseudopuberty in boys ( 5 , 9 ). Surgical excision of the mass is usually curative with regression of signs, nevertheless the development of central precocious puberty after surgery can occasionally be observed ( 4 – 9 ).…”

Section: Introductionmentioning

confidence: 99%

“…Leydig cell tumors are the most common hormone-secreting testicular tumors and are an unusual cause of precocious pseudopuberty in boys ( 5 , 9 ). Surgical excision of the mass is usually curative with regression of signs, nevertheless the development of central precocious puberty after surgery can occasionally be observed ( 4 – 9 ). We report two boys who developed true precocious puberty after surgical treatment of a Leydig cell tumor of the testis that presented peripheral precocious puberty.…”

Section: Introductionmentioning

confidence: 99%

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True Precocious Puberty Following Treatment of a Leydig Cell Tumor: Two Case Reports and Literature Review

et al. 2015

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Leydig cell testicular tumors are a rare cause of precocious pseudopuberty in boys. Surgery is the main therapy and shows good overall prognosis. The physical signs of precocious puberty are expected to disappear shortly after surgical removal of the mass. We report two children, 7.5 and 7.7 year-old boys, who underwent testis-sparing surgery for a Leydig cell testicular tumor causing precocious pseudopuberty. During follow-up, after an immediate clinical and laboratory regression, both boys presented signs of precocious puberty and ultimately developed central precocious puberty. They were successfully treated with gonadotropin-releasing hormone (GnRH) analogs. Only six other cases have been described regarding the development of central precocious puberty after successful treatment of a Leydig cell tumor causing precocious pseudopuberty. Gonadotropin-dependent precocious puberty should be considered in children treated for a Leydig cell tumor presenting persistent or recurrent physical signs of puberty activation. In such cases, therapy with GnRH analogs appears to be the most effective medical treatment.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 3 more Smart Citations

True Precocious Puberty Following Treatment of a Leydig Cell Tumor: Two Case Reports and Literature Review

et al. 2015

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Gonadotrophin-Independent Precocious Puberty; Female and Male

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Grinten²

2019

Encyclopedia of Endocrine Diseases

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Clinical presentation, management and follow-up of 83 patients with Leydig cell tumors of the testis: a prospective case-cohort study

et al. 2019

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STUDY QUESTION When should ‘not so rare’ Leydig cell tumors (LCTs) of the testis be suspected, diagnosed, and treated? SUMMARY ANSWER LCTs are more frequent than generally believed, are associated with male infertility, cryptorchidism and gynecomastia, and should be treated conservatively (in compliant patients) with active surveillance, which appears to be a safe alternative to surgical enucleation. WHAT IS KNOWN ALREADY Increasing referrals for testicular imaging have led to an increase in findings of LCTs. The features and natural history of these tumors remain largely unknown, as the available studies are small and heterogeneous. LCTs were previously treated aggressively and follow-up data are lacking. STUDY DESIGN, SIZE, DURATION A case-cohort study of consecutive patients diagnosed with LCTs over a 10-year period was prospectively enrolled from 2009 to 2018 and compared to matched cohorts of patients with seminomas or no testicular lesions screened in the same timeframe. PARTICIPANTS/MATERIALS, SETTING, METHODS Of the 9949 inpatients and outpatients referred for scrotal ultrasound, a total of 83 men with LCTs were included. Enrolled subjects underwent medical history and clinical examination and were asked to undergo routine blood tests, hormone investigations (FSH, LH, total testosterone, estradiol, inhibin B, sex hormone-binding globulin (SHBG), prolactin), and semen analysis. Patients who consented also underwent contrast-enhanced ultrasound, elastography, gadolinium-enhanced scrotal magnetic resonance imaging, and hCG stimulation test (5000 IU i.m.) with serum total testosterone and estradiol measured at 0, 24, 48, and 72 hours. MAIN RESULTS AND THE ROLE OF CHANCE In total, 83 patients diagnosed with LCTs were compared against 90 patients diagnosed with seminoma and 2683 patients without testicular lesions (NoL). LCTs were diagnosed by enucleation (48.2%), orchiectomy (13.3%), or clinical surveillance (38.5%). Testicular volume, sperm concentration, and morphology were lower (P = 0.001, P = 0.001, and P < 0.001, respectively) in patients with LCTs than in the NoL group. FSH, LH, and SHBG were higher and the testosterone/LH ratio was lower in LCTs than in the NoL group (P < 0.001). The LCT group showed higher SHBG (P = 0.018), lower sperm concentration (P = 0.029), and lower motility (P = 0.049) than the seminoma group. Risk factors for LCTs were cryptorchidism (χ2 = 28.27, P < 0.001), gynecomastia (χ2 = 54.22, P < 0.001), and low testicular volume (χ2 = 11.13, P = 0.001). Five cases were recurrences or bilateral lesions; none developed metastases during follow-up (median, 66 months). LIMITATIONS, REASONS FOR CAUTION This study has some limitations. First, hCG and second-line diagnostic investigations were not available for all tumor patients. Second, ours is a referral center for infertility, thus a selection bias may have altered the baseline features of the LCT population. However, given that the comparison cohorts were also from the same center and had been managed with a similar protocol, we do not expect a significant effect. WIDER IMPLICATIONS OF THE FINDINGS LCTs are strongly associated with male infertility, cryptorchidism, and gynecomastia, supporting the hypothesis that testicular dysgenesis syndrome plays a role in their development. Patients with LCTs are at a greater risk of endocrine and spermatogenesis abnormalities even when the tumor is resected, and thus require long-term follow-up and prompt efforts to preserve fertility after diagnosis. LCTs have a good oncological prognosis when recognized early, as tissue-sparing enucleation is curative and should replace orchiectomy. Conservative surgery and, in compliant patients, active surveillance through clinical and radiological follow-up are safe options, but require monitoring of testicular failure and recurrence. STUDY FUNDING/COMPETING INTEREST(S) The project was funded by the Ministry of University and Research Grant MIUR 2015ZTT5KB. There are no conflicts of interest. TRIAL REGISTRATION NUMBER ALCeP trial (ClinicalTrials.gov Identifier: NCT01206270).

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

Cited by 11 publications

References 24 publications

True Precocious Puberty Following Treatment of a Leydig Cell Tumor: Two Case Reports and Literature Review

True Precocious Puberty Following Treatment of a Leydig Cell Tumor: Two Case Reports and Literature Review

Gonadotrophin-Independent Precocious Puberty; Female and Male

Clinical presentation, management and follow-up of 83 patients with Leydig cell tumors of the testis: a prospective case-cohort study

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