Abstract
Twin brothers were evaluated at 6,83 years for precocious appearance of pubic hair at 6.5 years. Parental consanguinity and family history were negative. On the initial physical examination both patients had signs or virilisation (table) without testicular nodules.
Basal steroid levels in patients 1 and 2 were; respectively: testosterone 66 and 18ng/dl; Δ4-androstenedione 0.3 and <0.2ng/ml: DHEA 1.3 and 1.2nd/ml, DHEA-s 169 and 153ng/dl; 17-hydroxyprogesterone 0.6 and 0.5ng/ml. Both patients and prepubertal LH response to the LHRH test (ΔLH 4.6 and 7.6mIU/ml). ACTH stimultion did not demonstrate deficiency of adrenal steroidogenesis. Skull X-rays and CT scan and testicular ultrasonography were normal. Pubertal signs progressed only in case 1 and 6 months therapy with LHRH analog was clinically and biochemically ineffective, in fact, testoterone values ranged from 94 to 163 ng/dl. After 18 months, bone age advanced to 12 years in case I and remained equal to chronological age in alpable in the inferior pole of the right testis in patients 1, confirmed by ultrasonography (table). Right orchidectomy demostrated a 1.3 cm Leydig cell tumor. Seminiferous tubules showed spermatogenesis up to spermatids and the interstitium contained few partially differentiated Leydig cells. Post-operative serum testosterone values were low (12 ng/dl).
We conclude that this pair of twins with sexual precocity had a different progression: Case 1 had progressive virilisation and appearance of a Leydig cell tumor, whilst case 2 remained clinically and biochemically unchanged. The progression to a Leydig cell tumor not initially demonstrable indicates the need of close observation of patients with sexual precocity of testicular origin.
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Madureira, G., Arnhold, I., Mendonca, B. et al. SEXUAL PRECOCITY OF TESTICULAR ORIGIN IN TWINS: EVOLUTION OF A LEYDIG CELL TUMOR. Pediatr Res 28, 422 (1990). https://doi.org/10.1203/00006450-199010000-00040
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DOI: https://doi.org/10.1203/00006450-199010000-00040