Lawlor E R, Murphy J I, Sorensen P H, Fryer C J
Division of Pediatric Hematology-Oncology & Bone Marrow Transplant, British Columbia's Children's Hospital, Vancouver, Canada.
Med Pediatr Oncol. 1997 Oct;29(4):308-12. doi: 10.1002/(sici)1096-911x(199710)29:4<308::aid-mpo13>3.0.co;2-6.
Primitive neuroectodermal tumours (PNET) of the ovary are rare, aggressive tumours which are associated with high morbidity and mortality. Previously reported cases have shown limited response to therapy in patients presenting with metastatic disease and survival rates have been discouragingly low. We report the case of a 13-year-old girl who presented with a primary ovarian PNET and extensive metastatic disease. Pathologic studies confirmed the neural origin of the tumour and its morphologic appearance of neuroblastoma. Incomplete surgical resection was followed by treatment with aggressive multi-agent chemotherapy including cis-platinum, etoposide, cyclophosphamide, anddoxorubicin as per a neuroblastoma treatment protocol. Complete clinical remission ensued and she received consolidative therapy with myeloablative doses of thiotepa, melphalan, and carboplatin followed by autologous peripheral blood progenitor cell rescue. All therapy was well tolerated and the patient remains in complete remission with no evidence of disease 18 months from presentation. Mega-dose chemotherapy followed by progenitor cell rescue may provide optimal therapy for patients presenting with metastatic ovarian PNET.
卵巢原始神经外胚层肿瘤（PNET）罕见且具有侵袭性，与高发病率和死亡率相关。先前报道的病例显示，患有转移性疾病的患者对治疗反应有限，生存率低得令人沮丧。我们报告了一名13岁女孩的病例，她患有原发性卵巢PNET并伴有广泛的转移性疾病。病理研究证实了肿瘤的神经起源及其神经母细胞瘤的形态学表现。手术切除不完全后，根据神经母细胞瘤治疗方案，采用包括顺铂、依托泊苷、环磷酰胺和多柔比星在内的积极多药化疗。随后实现了完全临床缓解，她接受了大剂量噻替派、美法仑和卡铂的巩固治疗，随后进行自体外周血祖细胞救援。所有治疗均耐受性良好，自就诊以来18个月，患者仍处于完全缓解状态，无疾病证据。大剂量化疗后进行祖细胞救援可能为患有转移性卵巢PNET的患者提供最佳治疗。
