Al-Hader Ahmad A, Jain Amit, Al-Nasrallah Nawar, Einhorn Lawrence H
*Division of Hematology/Oncology, Melvin and Bren Simon Cancer Center, Indiana University School of Medicine, Indianapolis, IN †Talahassee Memorial Healthcare, Tallahassee, FL.
Am J Clin Oncol. 2015 Aug;38(4):364-6. doi: 10.1097/COC.0b013e31829d1ed7.
Metastatic germ cell cancers are highly chemosensitive and have 80% cure rate with cisplatin-based chemotherapy. Postchemotherapy teratoma can usually be surgically resected. However, teratoma, which is pluripotent tissue, can undergo malignant transformation along mesodermal elements to primitive neuroectodermal tumor (PNET). Unlike teratoma, PNET can metastasize and render a patient unresectable and incurable. We report the results of treatment of patients with malignant transformation to PNET with cyclophosphamide+doxorubicin+vincristine (CAV) alternating with ifosfamide+etoposide (IE).
We reviewed 86 patients with histologically confirmed PNET transformed from testicular teratoma at Indiana University from 1998 to 2012. We identified 18 patients who were treated with chemotherapy comprising cyclophosphamide (1000 to 1200 mg/m), doxorubicin (50 to 75 mg/m), and vincristine (2 mg) alternating with ifosfamide (1.8 g/m) plus etoposide (100 mg/m) for 5 consecutive days. Treatment was given every 3 weeks with a maximum of 6 cycles or until progression or undue toxicity. Hematopoietic growth factors were usually incorporated. The remaining 68 patients underwent surgical resection.
Twelve patients had unresectable disease and 6 were treated in an adjuvant setting. Median age was 29 years (range, 20 to 53 y). Nine of the 12 metastatic patients achieved objective response by RECIST criteria. Six of those were rendered with no evidence of disease (NED) with further surgery. Although 4 of the 6 patients subsequently relapsed, 1 patient remains alive and NED at 78 months. The 6 patients who received adjuvant treatment are alive with NED at 9 to 90 months with a median duration of 32.7 months.
CAV and IE alternating chemotherapy has high objective response rate for PNET transformed from teratoma and results in occasional long-term disease-free survival when combined with subsequent resection. We recommend adjuvant CAV alternating with IE chemotherapy for patients with PNET after RPLND due to the high probability of recurrent disease and their high chemosensitivity to this regimen.
转移性生殖细胞癌对化疗高度敏感，基于顺铂的化疗治愈率达80%。化疗后畸胎瘤通常可通过手术切除。然而，畸胎瘤作为多能组织，可沿中胚层成分发生恶性转化，形成原始神经外胚层肿瘤（PNET）。与畸胎瘤不同，PNET可发生转移，导致患者无法切除且无法治愈。我们报告了采用环磷酰胺+阿霉素+长春新碱（CAV）与异环磷酰胺+依托泊苷（IE）交替治疗发生PNET恶性转化患者的结果。
我们回顾了1998年至2012年在印第安纳大学组织学确诊为睾丸畸胎瘤转化而来的PNET的86例患者。我们确定了18例接受化疗的患者，化疗方案为环磷酰胺（1000至1200mg/m²）、阿霉素（50至75mg/m²）和长春新碱（2mg）与异环磷酰胺（1.8g/m²）加依托泊苷（100mg/m²）交替，连续5天。每3周进行一次治疗，最多6个周期，或直至病情进展或出现严重毒性反应。通常会使用造血生长因子。其余68例患者接受了手术切除。
12例患者疾病无法切除，6例接受辅助治疗。中位年龄为29岁（范围20至53岁）。12例转移性患者中有9例根据RECIST标准达到客观缓解。其中6例经进一步手术达到无疾病证据（NED）。尽管6例患者中有4例随后复发，但1例患者在78个月时仍存活且处于NED状态。6例接受辅助治疗的患者在9至90个月时存活且处于NED状态，中位持续时间为32.7个月。
CAV和IE交替化疗对畸胎瘤转化而来的PNET具有较高的客观缓解率，与后续切除联合使用时偶尔可实现长期无病生存。由于复发疾病的可能性较高且对该方案化疗敏感性高，我们建议对行腹膜后淋巴结清扫术后的PNET患者采用辅助性CAV与IE交替化疗。
