Singh Charanjeet, Ahmad Sarfraz, Hajjar Fouad M, Holloway Robert W
Department of Pathology & Laboratory Medicine, Florida Hospital, Orlando, FL 32804, USA.
Department of Gynecologic Oncology, Florida Hospital, Orlando, FL 32804, USA.
Gynecol Oncol Rep. 2018 Jun 12;25:70-73. doi: 10.1016/j.gore.2018.06.003. eCollection 2018 Aug.
Ovarian Sertoli-Leydig cell tumors (SLCT) represent <2% of primary ovarian tumors, which range from benign to malignant; majority of the latter are low-grade. We present the case of a 12-year-old pre-pubertal female with poorly differentiated SLCT and heterologous rhabdomyosarcoma (FIGO stage-IA). She presented with worsening abdominal pain, pelvic mass, and elevated pre-operative serum Alpha-fetoprotein (AFP, 77.1 ng/mL). She underwent right salpingo-oophorectomy, omentectomy, and lymphadenectomy. The high-risk histology warranted 4-cycles of adjuvant BEP chemotherapy. There was no clinical evidence of recurrence at the 20-month follow-up. High-grade sex cord-stromal tumors are rare, present with low-stage disease, and have good progression-free survival following chemotherapy. Rarity of these tumors poses challenges in their diagnosis and treatment. Review of literature suggests that the presented case is youngest patient with dedifferentiated SLCT.
卵巢支持-间质细胞瘤（SLCT）占原发性卵巢肿瘤的比例不到2%，其良恶性程度不一；大多数恶性肿瘤为低级别。我们报告了一例12岁青春期前女性，患有低分化SLCT和异源性横纹肌肉瘤（国际妇产科联盟分期为IA期）。她因腹痛加重、盆腔肿块和术前血清甲胎蛋白（AFP）升高（77.1 ng/mL）就诊。她接受了右侧输卵管卵巢切除术、大网膜切除术和淋巴结切除术。高危组织学特征需要进行4个周期的辅助BEP化疗。在20个月的随访中没有复发的临床证据。高级别性索间质肿瘤罕见，多表现为低分期疾病，化疗后无进展生存期良好。这些肿瘤的罕见性给其诊断和治疗带来了挑战。文献回顾表明，本病例是患有去分化SLCT的最年轻患者。
