Cassio A, Cacciari E, D'Errico A, Balsamo A, Grigioni F W, Pascucci M G, Bacci F, Tacconi M, Mancini A M
Department of Pediatrics, University of Bologna, Italy.
Acta Endocrinol (Copenh). 1990 Oct;123(4):416-22. doi: 10.1530/acta.0.1230416.
Gonadal histology was investigated by means of conventional microscopy in 6 patients with complete androgen insensitivity syndrome, in 11 with incomplete androgen insensitivity syndrome, and in 3 with 5 alpha-reductase syndrome. Twelve subjects were prepubertal and 8 pubertal. In all patients gonadal tissue was removed as a prophylactic measure and no patients gave rise to any clinical suspicion of a tumour. Eight patients with incomplete androgen insensitivity syndrome, 5 of whom (62.5%) were prepubertal, showed intratubular germ cell neoplasia and in 6 of them it was bilateral. Histochemical and immunohistochemical analysis showed considerable agreement between atypical morphological aspects and positive response to Schiff's periodic acid and to staining with the anti-placenta alkaline phosphatase antibody. Our patients were characterized by one of the highest reported incidences of intratubular germ cell neoplasia, particularly at prepubertal age. These findings would seem to indicate that a rethink is needed concerning the general opinion that patients with androgen intensivity syndrome have practically no risk of developing malignancy, and that orchidectomy is not advisable before puberty is completed.
通过传统显微镜检查对6例完全性雄激素不敏感综合征患者、11例不完全性雄激素不敏感综合征患者和3例5α-还原酶综合征患者的性腺组织学进行了研究。12名受试者处于青春期前，8名处于青春期。所有患者均作为预防性措施切除性腺组织，且没有患者出现任何肿瘤的临床疑似情况。8例不完全性雄激素不敏感综合征患者，其中5例（62.5%）处于青春期前，表现为管内生殖细胞瘤，其中6例为双侧。组织化学和免疫组织化学分析显示，非典型形态学特征与对高碘酸希夫反应以及抗胎盘碱性磷酸酶抗体染色的阳性反应之间存在相当程度的一致性。我们的患者以管内生殖细胞瘤的报道发病率最高为特征之一，尤其是在青春期前年龄。这些发现似乎表明，需要重新思考关于雄激素敏感性综合征患者几乎没有发生恶性肿瘤风险的普遍观点，以及在青春期结束前不建议进行睾丸切除术的观点。
