Gattuso Giovanna, Casanova Michela, Biassoni Veronica, Terenziani Monica, Schiavello Elisabetta, Sironi Giovanna, Collini Paola, Pallotti Federica, Morosi Carlo, Massimino Maura
Pediatric Oncology Unit, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Pathology and Laboratory Medicine, Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy.
Tumori. 2020 Dec;106(6):NP14-NP17. doi: 10.1177/0300891620925532. Epub 2020 May 28.
Peripheral precocious puberty (PPP) may be a paraneoplastic manifestation, associated with beta human chorionic gonadotropin (β-hCG)-secreting tumors. We describe 2 young children with β-hCG-secreting tumors presenting with signs of pubertal activation. In the first patient, a 16-month-old boy with hepatoblastoma, only initial signs of PPP at presentation were identifiable, with concomitant high levels of β-hCG. Although the tumor had good response to therapy, β-hCG levels were fluctuant until the tumor was resected surgically. The second patient, an 18-month-old boy with intracranial germ cell tumor, presented with clear signs of pubertal activation and genitalia enlargement with no initial alteration of sex hormones. In both cases, the oncologic response to therapy was good. In the first case, full remission of the pubertal signs was observed; in the second, pubertal signs were still visible 20 months after the end of treatment.
外周性性早熟（PPP）可能是一种副肿瘤表现，与分泌β人绒毛膜促性腺激素（β-hCG）的肿瘤有关。我们描述了2例患有分泌β-hCG肿瘤并出现青春期激活体征的幼儿。第一例患者是一名16个月大患有肝母细胞瘤的男孩，就诊时仅可识别出PPP的初始体征，同时β-hCG水平较高。尽管肿瘤对治疗反应良好，但在手术切除肿瘤前β-hCG水平一直波动。第二例患者是一名18个月大患有颅内生殖细胞瘤的男孩，表现出明显的青春期激活体征和生殖器增大，初始性激素无变化。在这两个病例中，肿瘤对治疗的反应良好。在第一个病例中，观察到青春期体征完全缓解；在第二个病例中，治疗结束20个月后青春期体征仍然可见。
