Neonatal Bilateral Ovarian Sex Cord Stromal Tumors
Neonatal Bilateral Ovarian Sex Cord Stromal Tumors
复制标题
DOI：
10.1002/pbc.21775
复制
发表时间：
2009-03-01
影响因子：
3.2
通讯作者：
Sarnacki, Sabine
中科院分区：
文献类型：
--
作者：
Capito, Carmen;Flechtner, Isabelle;Sarnacki, Sabine
一个孩子在产前诊断为巨大儿，阴蒂肿大，唇肥大和左卵巢囊肿后被转介评估。核型为46，XX。产后盆腔超声检查正常。高水平的抗苗勒管激素和睾酮导致hCG刺激试验，随后出现同性性早熟和双侧卵巢肿大伴左侧肿瘤肿块。左卵巢肿瘤切除术发现纤维膜瘤。6周后肿瘤复发，完成卵巢切除术后发现为幼年颗粒细胞瘤（JGCT）。而手术后激素水平下降，新的上升与右侧卵巢增大相关，导致右侧JGCT的诊断。父母拒绝接受进一步的手术，医生建议他们进行右卵巢切除术。2个月后，激素水平恢复正常。这个病例说明了新生儿发育生物学和肿瘤生物学之间令人困惑的重叠。中华儿科血癌杂志；2009;32(2):1-4。(C) 2008 Wiley-Liss, Inc。
A child was referred for evaluation after prenatal diagnosis with macrosomia, clitoromegaly, labial hypertrophy, and a left ovarian cyst. The karyotype was 46,XX. The postnatal pelvic ultrasound was normal. High levels of anti-mullerian hormone and testosterone led to hCG stimulation test, which was followed by isosexual precocious puberty and the appearance of a bilateral ovarian enlargement with a left tumoral mass. A left ovarian tumorectomy revealed a fibrothecoma. Six weeks later a tumoral relapse occurred and completion of oophorectomy revealed a juvenile granulosa cell tumor (JGCT). Whereas hormonal levels decreased after surgery, a new rise associated with an enlargement of the right ovary led to the diagnosis of right JGCT. A right oophorectomy was proposed to the parents, who declined further surgery. After 2 months, the hormonal levels normalized. This case illustrates the confusing overlap between developmental and neoplastic biology in neonates. Pediatr Blood Cancer 2009;52:401-401. (C) 2008 Wiley-Liss, Inc.