Leydig Cell Tumour and Giant Adrenal Myelolipoma Associated with Adrenogenital Syndrome: A Case Report with a Review of the Literature
Vincenzo AltieriBarbara AltieriRoberto CastellucciStefano AlbaFrancesco BottoneEugenia FragalàVincenzo MironeFrancesco Greco
7
Citation
15
Reference
10
Related Paper
Citation Trend
Abstract:
Context Male patients with congenital adrenal hyperplasia (CAH) may develop bilateral testicular adrenal rest tumours (TARTs). These tumours, in most cases, regress with glucocorticoid therapy and their histological differentiation from Leydig-cell tumors is quite difficult. Objective The aim of this study was to differentiate the histological and clinical features of the TARTs from those of the Leydig-cell tumours. Methods The authors report a case of bilateral Leydig-cell tumour associated with giant bilateral adrenal myelolipoma in a male with adrenogenital syndrome who was submitted to a bilateral orchiectomy. Results Testicular neoplasia continue to grow despite adequate hormonal treatment and a bilateral orchiectomy was performed. The histopathological examination of the specimen ultimately confirmed the diagnosis of bilateral Leydig-cell tumour. Conclusions This case shows the importance of all the relevant examinations, blood chemistry analysis, and instrumental tests in the differential diagnosis of TARTs and other testicular tumours.Keywords:
Adrenogenital syndrome
Orchiectomy
Leydig Cell Tumor
Sexual precocity in boys is defined as any sign of secondary sexual characteristics present before the age of 9. Leydig cell tumors of the testes are a rare cause of peripheral precocious puberty in boys. Here, we report 8 years and 4-month-old boys with signs of peripheral precocious puberty because of a testicular Leydig cell tumor that developed true precocious puberty after surgical removal. Examination of genitalia showed Tanner 4 hair growth. The penis length was 14.5 cm with a 2.5 cm width. The right testis was enlarged but the left testis was measured at 2cm in length and 1 cm in width. Laboratory results showed low serum gonadotropin levels and increased androgen levels. Testicular sonography reported one solid mass measured 31×28×15 millimeters. With a presumptive diagnosis of Leydig cell tumor, the patient underwent radical orchiectomy. Pathologic evaluation confirmed it. Two months after surgery, the diagnosis of central precocious puberty was confirmed according to physical examination and rising of serum gonadotropins. We started treatment with a Gonadotropin-releasing hormone (GnRH) agonist. Leydig cell tumor in children is an uncommon cause of precocious puberty. In every boy with the sign of peripheral precocious puberty and asymmetrical testicular enlargement, the testicular tumor should be considered. It may induce central precocious puberty after surgical resection and this diagnosis should be considered in the patient’s follow-up in the next visits.
Leydig Cell Tumor
Pubic hair
Orchiectomy
Cite
Citations (0)
In a 4<sup>8</sup>/<sub>12</sub>-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.
Leydig Cell Tumor
Orchiectomy
Cite
Citations (44)
Testis tumors are extremely rare tumors, especially if they are bilateral, interstitial tumors. We present a case with bilateral Leydig cell tumors, which were detected incidentally. First, radical left orchiectomy was performed and pathologic diagnosis was Leydig cell tumor. One week later, partial right orchiectomy was done. The diagnosis was the same. After the 12-month follow-up, no recurrence or metastasis was detected. We suggest organ-sparing surgery as an alternative in the treatment of bilateral Leydig cell tumors especially for young males, since this surgical approach prevents hormonal replacement therapy and maintains fertility and potency.
Leydig Cell Tumor
Orchiectomy
Testicle
Cite
Citations (7)
Gynecomastia
Adrenogenital syndrome
Leydig Cell Tumor
Orchiectomy
Cite
Citations (35)
Testicular tumors are very rare in boys, approximately 1.5% of these are Leydig cell tumors. The authors present a 4.5 year-old boy with Leydig cell tumor of left testis, which was associated with increased sex steroid production that caused precocious puberty. These tumors are benign processes in prepubertal children. Beyond the rarity of this case the authors would like to report about its diagnostic difficulties and testis-sparing remove of it. The plasma 17-hydroxyprogesterone levels provide the distinction between congenital adrenal hyperplasia and Leydig cell tumor in patient with precocious puberty.
Leydig Cell Tumor
Cite
Citations (0)
Introduction: Leydig cell tumors (LCT) are the most common hormone-secreting testicular tumors; it is a rare cause for precocious pseudo-puberty in boys. The tumors secrete high amounts of testosterone. We present two cases of LCT in prepubertal boys presenting with precocious puberty. Case Reports: Case 1. A 6-year-old boy was referred from the pediatric department with a diagnosis of precocious puberty. The patient had reported enlarged and painless swelling of the left testicle from a year ago. The puberty status of the patient was A1P3G4. Ultrasonography examination had found left testicular mass. Elective radical orchiectomy of the left testicle was performed. Histopathological analysis confirmed the diagnosis of benign LCT. Case 2. A 6-year-old boy presented with an enlarged left testicle for the last three months. Features of puberty were noted on the patient (appearance of pubic and facial hair). The puberty status of the patient was A1P3G3. Left testicle US had found homogenous, hypoechoic mass with calcification. Bone age had found increased bone maturation. Increased androgen hormones were detected through a blood test. Radical orchiectomy of the left testicle was performed. The histopathological examination showed malignant LCT. Conclusion: Leydig cell tumors uncommonly occur in children. Prepubertal-aged boys presented with asymmetrical, firm, painless testicular enlargement with signs of puberty should be evaluated for LCT. Histopathological analysis is the mainstay of diagnosis and radical orchiectomy is the treatment of choice of LCT. Keywords: precocious pseudo-puberty, Leydig cell tumor, orchiectomy
Cite
Citations (3)
Adrenogenital syndrome
Enzyme deficiency
Cite
Citations (6)
Adrenogenital syndrome
Ambiguous genitalia
Fludrocortisone
Cite
Citations (32)
Leydig cell tumors of the testis are uncommon, and bilateral lesions are extremely rare. We report a case of bilateral Leydig cell tumor of the testis treated with radical orchiectomy and contralateral subtotal orchiectomy with the intent of preservation of hormonal function and fertility.
Orchiectomy
Leydig Cell Tumor
Male fertility
Testicle
Cite
Citations (13)
Adrenogenital syndrome
Leydig Cell Tumor
Dysgerminoma
Cite
Citations (36)