Biswas Ahitagni, Puri Tarun, Goyal Shikha, Gupta Ruchika, Eesa Muneer, Julka Pramod Kumar, Rath Goura Kishor
Department of Radiotherapy, All India Institute of Medical Sciences, New Delhi, India.
Acta Neurochir (Wien). 2009 Mar;151(3):277-84. doi: 10.1007/s00701-009-0200-1. Epub 2009 Feb 25.
Primary spinal cord germ cell tumour is a rare tumour. We herein review the tumour characteristics, associated risk factors, treatment policy, and patterns of failure of primary intradural germ cell tumour.
We conducted a PUBMED search using a combination of keywords such as "spinal germ cell tumor," "germinoma," "extradural," "intradural," "intramedullary," "extramedullary," and identified 19 cases of primary spinal germ cell tumour. Clinical features, pathologic characteristics, and treatment details of these patients including status at follow-up were noted from respective case reports. We also describe a case of a young Indian patient of intradural extramedullary germ cell tumour treated with a combination of surgery, chemotherapy, and radiotherapy.
The median age at presentation was 24 years. The most common location of the tumour was thoracic (40%). Beta-HCG overproduction was noted in 40% of the patients. Most patients were treated with a combination of surgery, radiation therapy, and systemic chemotherapy. Median follow-up was 16.5 months. Recurrence was observed in 10% of the patients, all in beta-HCG over-producing tumours. The illustrative case was a 28-year male, presenting with pain in lower back and both lower limbs for 2 months. Magnetic resonance imaging spine showed an inhomogeneous hyperintense soft tissue mass at L(2)-L(4) spinal level. He was treated with complete surgical excision and four cycles of chemotherapy with BEP regimen following a histological diagnosis of non-seminomatous germ cell tumour. Palliative irradiation to the lumbar spine was given on progression at 3 months. The patient eventually succumbed to his condition, due to compressive transverse myelitis possibly due to cervical cord metastasis.
Limited surgery followed by upfront radiation therapy and adjuvant chemotherapy is the optimal management of this rare group of tumour. Omission of radiation therapy from the treatment armamentarium might engender local recurrence and spinal dissemination at first failure.
原发性脊髓生殖细胞瘤是一种罕见肿瘤。我们在此回顾原发性硬脊膜内生殖细胞瘤的肿瘤特征、相关危险因素、治疗策略及失败模式。
我们在PUBMED上进行检索，使用“脊髓生殖细胞瘤”“生殖细胞瘤”“硬膜外”“硬膜内”“髓内”“髓外”等关键词组合，确定了19例原发性脊髓生殖细胞瘤病例。从各自的病例报告中记录了这些患者的临床特征、病理特征及治疗细节，包括随访状态。我们还描述了一例年轻的印度硬膜内髓外生殖细胞瘤患者，采用手术、化疗和放疗联合治疗。
发病时的中位年龄为24岁。肿瘤最常见的部位是胸部（40%）。40%的患者出现β-HCG过度产生。大多数患者接受了手术、放疗和全身化疗联合治疗。中位随访时间为16.5个月。10%的患者出现复发，均为β-HCG过度产生的肿瘤。举例病例为一名28岁男性，出现下背部和双下肢疼痛2个月。脊柱磁共振成像显示L2-L4脊髓水平有一个不均匀的高信号软组织肿块。在组织学诊断为非精原性生殖细胞瘤后，他接受了完整的手术切除，并采用BEP方案进行了四个周期的化疗。3个月病情进展时对腰椎进行了姑息性放疗。患者最终因可能因颈髓转移导致的压迫性横贯性脊髓炎而病情恶化。
有限手术，随后进行 upfront 放疗和辅助化疗是这类罕见肿瘤的最佳治疗方法。治疗方案中遗漏放疗可能会在首次失败时导致局部复发和脊髓播散。
