Abstract
Females with PJS (mucocutaneous pigmentation and intestinal polyposis) have an increased risk of ovarian tumors, with 5% developing a pathologically distinct neoplasm described as a sex cord tumor with annular tubules. We evaluated a 6 yr old male with PJS for bilateral gynecomastia. Testes both measured 3 ml and were normal to palpation. Although two initial estradiol levels were less than 10 pg/ml, a subsequent value was moderately elevated at 24 pg/ml. T4, FSH, LH, T, prolactin and DHAS were all within the normal range for his age. Bone age was slightly advanced at 8.2 yr. Abdominal and pelvic ultrasound, including both adrenals, was normal. Ultrasonography of the testes, however, demonstrated multiple echogenic areas in both testes. Open biopsy revealed multiple minute foci of large-cell, calcifying Sertoli cell tumors. Since the lesions did not appear to be malignant by histology, and were bilateral, the testes were not removed. Subsequently, there has been no progression of the gynecomastia, and serum estradiol has remained <20 pg/ml. Although similar tumors have been reported in two other prepubertal males with PJS, this case is unique in that the tumors were 1) bilateral and multifocal, 2) nonpalpable, and 3) detected by ultrasound. Taken together, these reports strongly suggest that males, as well as females, with PJS are at risk for gonadal tumors and, as such, all patients with PJS should be closely followed for the potential development of gonadal tumors.
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Wilson, D., Pitts, W., Hintz, R. et al. 165 TESTICULAR TUMORS WITH PEUTZ-JEGHERS SYNDROME (PJS). Pediatr Res 19, 631 (1985). https://doi.org/10.1203/00006450-198506000-00185
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DOI: https://doi.org/10.1203/00006450-198506000-00185