Development of True Precocious Puberty Following Treatment of a Leydig Cell Tumor of the Testis

Ghazi, Ali A.; Rahimi, Farzaneh; Ahadi, Mir Mahmood S.; Sadeghi‐Nejad, Abdollah

doi:10.1515/jpem.2001.14.9.1679

Cited by 18 publications

(20 citation statements)

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“…This phenomenon was also observed in four other patients with a Leydig cell tumour (9,11–13) and furthermore in children with congenital adrenal hyperplasia, familial male precocious puberty, McCune Albright syndrome and in other rare diseases. However, the true incidence in this condition is remains unclear because long‐term follow‐ups of children with Leydig cell tumours are not available.…”

Section: Discussionmentioning

confidence: 57%

Clinical and metabolic findings in a 6‐year‐old boy with a Leydig cell tumour

et al. 2011

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Section: Discussionmentioning

confidence: 57%

Clinical and metabolic findings in a 6‐year‐old boy with a Leydig cell tumour

et al. 2011

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“…In fact, when prepubertal children are exposed to testosterone, this can trigger the development of central precocious puberty, either from the priming effect of the sex steroid on the hypothalamus or from sudden lowering of sex steroid levels following improvement in the control of gonadotropin-independent precocious puberty [7,18,19]. There are few reported cases of central precocious puberty after treatment of testicular Leydig cell tumors [5,20,21,22,23]. …”

Section: Discussionmentioning

confidence: 99%

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

Silva

Bonito-Vítor²,

Campos³

et al. 2014

Horm Res Paediatr

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Leydig cell testicular tumors are very rare in children. They can present as gonadotropin-independent precocious puberty due to excess androgen secretion. We report the case of an 8-year-old boy with isosexual precocity whose hormonal investigation showed luteinizing hormone-independent testosterone hypersecretion. Although no palpable mass was present, scrotal ultrasound revealed a testicular tumor. Testis-sparing tumor resection was performed and the histopathology analysis showed a Leydig cell tumor. After surgery the testosterone levels remained high and further examination showed gonadotropin-dependent precocious puberty, which is believed to be likely caused by the activation of the hypothalamic-pituitary axis due to a long-term exposition to sex steroids. He is currently being treated with a long-acting gonadotropin-releasing hormone analog and the process of sexual precocity has until now been suppressed.

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“…This mismanagement was partly due to the higher incidence of CAH and partly because of the misconception that 17-OH progesterone is a specific marker for CAH, while the metabolite may be highly elevated in adrenocortical tumors and also in hormone-secreting testicular tumors as well (21,22). In the case reported by Hishiki et al (23), high serum 17(OH) progesterone that was used for evaluation of CAH led to diagnosis of adrenocortical tumor in a newborn.…”

Section: Discussionmentioning

confidence: 99%

Functioning Adrenocortical Tumors in  Children-Secretory Behavior

Ghazi¹,

Mofid²,

Salehian³

et al. 2013

Jcrpe

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Objective: Adrenocortical tumors are rare childhood neoplasms. More than 95% are functional and present with virilization, Cushing’s syndrome, hypertension, or hyperestrogenism. The objective of this paper is to present the clinical, laboratory and pathological findings of this rare disease and to highlight the secretory behavior of these tumors.Methods: Clinical and laboratory data of seven Iranian children and adolescents aged between 2 and 16 years with functioning adrenocortical tumors are presented. Five patients had virilization and two had Cushing’s syndrome at the time of diagnosis. In all subjects, the tumors were removed successfully by open surgery, during which a blood sample was drawn from the corresponding adrenal vein for hormonal evaluation.Results: Peripheral blood evaluation revealed that in addition to the dominant hormone (testosterone in the cases presenting with virilization and cortisol in those with Cushing’s syndrome), significant amounts of other hormones were secreted from these tumors. Adrenal vein evaluation revealed that testosterone, dehydroepiandrosterone sulfate, estradiol, ?17(OH) progesterone, and cortisol were directly released from the tumor. The tumors weighed between 36-103 grams. The patients have since been followed for 5 to 20 years, and there have been no signs or symptoms of relapse in any of the patients.Conclusions: The study shows that functioning adrenocortical tumors should be considered in children and adolescents presenting with hyperandrogenism, Cushing’s syndrome, or hyperestrogenism. A diagnosis of a functioning adrenocortical tumor requires surgical removal as early as possible to prevent the untoward effects of virilization or corticosteroid excess. Evaluation of adrenal vein hormones showed that the steroids are secreted directly from the tumor and peripheral conversion has little contribution to the serum levels.Conflict of interest:None declared.

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Development of True Precocious Puberty Following Treatment of a Leydig Cell Tumor of the Testis

Abstract: We report a prepubertal boy who developed true precocious puberty following unilateral orchidectomy for the treatment of a Leydig cell tumor.

Cited by 18 publications

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Clinical and metabolic findings in a 6‐year‐old boy with a Leydig cell tumour

Clinical and metabolic findings in a 6‐year‐old boy with a Leydig cell tumour

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

Functioning Adrenocortical Tumors in  Children-Secretory Behavior

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Development of True Precocious Puberty Following Treatment of a Leydig Cell Tumor of the Testis

Abstract: We report a prepubertal boy who developed true precocious puberty following unilateral orchidectomy for the treatment of a Leydig cell tumor.

Cited by 18 publications

References 0 publications

Clinical and metabolic findings in a 6‐year‐old boy with a Leydig cell tumour

Clinical and metabolic findings in a 6‐year‐old boy with a Leydig cell tumour

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

Functioning Adrenocortical Tumors in Children-Secretory Behavior

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Functioning Adrenocortical Tumors in  Children-Secretory Behavior