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CASE REPORT |
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| Year : 2004 | Volume
: 20
| Issue : 2 | Page : 164-165 |
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Virilising tumor of testis - a case report
L Sudhakar, C Anand, K Sri Ram, G Sivasankar, N Muthulatha, MG Rajamanickam
Department of Urology, Government Royapettah Hospital, Chennai, India
Correspondence Address:
M G Rajamanickam
No.7 Anandan Street, T.Nagar, Chennai - 600 017
India
 Source of Support: None, Conflict of Interest: None  | Check |
Keywords: Leydic cell tumor, virilising tumor, Reinke′s crystals.
How to cite this article:
Sudhakar L, Anand C, Sri Ram K, Sivasankar G, Muthulatha N, Rajamanickam M G. Virilising tumor of testis - a case report. Indian J Urol 2004;20:164-5 |
 Case Report | |  |
A 9-year-old boy presented with a sudden increase in height and weight of 6 months' duration, followed by a painless scrotal swelling. Patient also noticed change in voice for the same duration. On examination, the boy had an adult look with prominent facial hairs, acne vulgaris and well developed secondary sexual characters [Figure - 1]. His height was 5'1" and weighed 46 kg. Genital examination revealed a penile length of 10.5 cm. Right testis was enlarged (4 x 3 cm), firm in consistency with diminished testicular sensation. Left testis was normal (2 x 1 cm) for prepubertal age.
On evaluation, ultrasonogram of scrotum showed an irregular hypoechoeic lesion of 1.9 x 2.8 x 2.9 cm in the lower pole of right testis. Ultrasonogram and CT scan of the abdomen were normal. Endocrine evaluation with reference to prepubertal values revealed a raised testosterone level of 18.3 ng/ml (Ref 2.7 - 10.7 ng/ml), LH was 2.23 mlU/ml (Ref 2-12 mIU/ml), FSH was 1.2 mIU/ml (Ref 3-15 mIU/ml), basal GH was 8.7 ng/ml (Ref 0-7 ng/ ml), cortisol was 9.83 mg/ml (Ref 8-9 mg/ml), DHEA was 80 mg/ml (Ref 80-560 mg/ml) and 17-OH progesterone was 10.88 ng/ml (Ref 0.4-3.6 ng/ml). (The reference values mentioned are for the prepubertal age.) HCG and AFP levels were normal. Dexamethasone suppression test (DST) was negative, which rule out adrenal hyperplasia.
Bone age study was advanced to 14-16 years. A diagnosis of a virilising tumor of the testis was made and right radical inguinal orchidectomy was done and histopathological report was benign leydig cell tumor (LCT) [Figure - 2]A. Patient was followed up for 1 year and serum testosterone levels were normal. But the virilising features however were static.
| Comments | | |
Precocious puberty in boys presents a diagnostic challenge. A normal gonadotropin level indicates an extra-pituitary cause and LCT should be entertained. LCTs are the most common gonadal stromal tumors accounting for 1-3% of all testicular tumors and 6% of testicular tumors in children. [1] They occur predominantly in boys older than 4 years and in adults between 20-50 years of age. Childhood LCTs usually are hormonally active and have a benign course. The tumor results in incomplete (peripheral) isosexual precocity accounting for 10% of all cases of precocious puberty. The hallmark of the diagnosis of LCT is the triad of precocious puberty, testicular mass and elevated urinary 17 ketosteroid level. However a marked elevation of 17 ketosteroid is characteristic of the 17-hydroxylase deficiency type of congenital adrenal hyperplasia (CAH). Thus it is mandatory to differentiate LCT from CAH. Testicular nodules in CAH often present in the second decade of life and are bilateral with a strong family history. [2] LCT should also be differentiated from leydig cell hyperplasia and tumors of adrenal rest tissue. Endocrine evaluation should include serum testosterone, LH, FSH, GH, DHEA levels and DST. Ninety per cent of LCTs are benign. Macroscopically. LCTs appear well encapsulated with compression of adjacent testicular tissue. The pathognomonic histological feature characteristic of LCT is intracytoplasmic Reinke's crystals, which is present in only about 40% of tumors [Figure - 2]B. The absolute criterion for malignancy is metastases. The treatment of LCT is radical inguinal orchidectomy. In children in whom the diagnosis is suspected preoperatively, a testis sparing enucleation is acceptable provided histological confirmation of the lesion is obtained by frozen section. [3] The pronounced virilisation along with the hormonal profile and benign histology in this case are classical of childhood LCT.
| References | | |
| 1. | Ciftei AO. Bingol-Kologlu M, Senocak ME, Tanyel FC. Munevver Buyukpamukeu and Neibil Buyukpamukeu: Testicular tumors in children. J Pediatr Surg 2001; 36: 1800.  |
| 2. | Cunnah D. Perry L, Dacie JA et al. Bilateral testicular tumors in congenital adrenal hyperplasia: a continuing diagnostic and therapeutic dilemma. Clin Endocrinol (Oxf) 1989; 30: 141. |
| 3. | Klein EA. Levin HS. Non-germ cell tumors of the testis. In: Osterling JE, Richie JP. (eds.), Urologic Oncology, Philadelphia: WB Saunders: 1997: 496-514. |
[Figure - 1], [Figure - 2]
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