Garg Rajan, Agarwala Sandeep, Bakhshi Sameer, Srinivas M, Bajpai Minu, Gupta Devendra Kumar, Bhatnagar Veereshwar
Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi 110029, India.
Department of Medical Oncology, BRAIRCH, All India Institute of Medical Sciences, New Delhi 110029, India.
J Pediatr Surg. 2014 Jul;49(7):1113-5. doi: 10.1016/j.jpedsurg.2013.11.063. Epub 2013 Nov 28.
Neurological involvement due to intraspinal extension in sacrococcygeal malignant germ cell tumors (SC-MGCTs) has rarely been reported.
To evaluate the incidence, presentation, management and the outcome of patients of SC-MGCT with intraspinal extension.
Case records of all cases of SC-MGCT from 2001 to 2008, were reviewed to identify cases with vertebral involvement and intraspinal extension. They were evaluated in terms of their presentation, response to therapy, extent of surgical resection, recovery of neurological symptoms and outcome.
Of the 31 cases of SC-MGCT, 5 (16%) had intraspinal extension. Age ranged from 12 to 84 months (median 24 months). Four patients had Altman type 4 disease (stage 4) and 1 had Altman type 3 (stage 3) disease. The intraspinal extension in all patients was detected on contrast CT scan. Patients presented with neurological symptoms in the form of lower limb paresis (80%), bowel and bladder (20%) incontinence. All the tumors responded to pre-operative chemotherapy. Gross complete local resection could be achieved in 4(80%). Neurological recovery was complete in all except for persisting neurogenic bladder in one. During follow up of 3-32 months, all were alive with no recurrence.
SC-MGCT presenting with neurological deficits due to intraspinal extension is usually advanced disease. These patients respond to chemotherapy and surgical resection and most have complete neurological improvement.
骶尾部恶性生殖细胞肿瘤(SC-MGCTs)脊髓内扩展导致的神经受累鲜有报道。
评估脊髓内扩展的SC-MGCT患者的发病率、临床表现、治疗及预后。
回顾2001年至2008年所有SC-MGCT病例记录,以确定有椎体受累及脊髓内扩展的病例。对其临床表现、治疗反应、手术切除范围、神经症状恢复情况及预后进行评估。
31例SC-MGCT中,5例(16%)有脊髓内扩展。年龄范围为12至84个月(中位年龄24个月)。4例患者为阿尔特曼4型疾病(4期),1例为阿尔特曼3型(3期)疾病。所有患者的脊髓内扩展均在增强CT扫描时被发现。患者表现为下肢轻瘫(80%)、肠道及膀胱失禁(20%)等神经症状。所有肿瘤对术前化疗均有反应。4例(80%)可实现大体完全局部切除。除1例持续性神经源性膀胱外,其余患者神经功能均完全恢复。在3至32个月的随访中,所有患者均存活且无复发。
因脊髓内扩展而出现神经功能缺损的SC-MGCT通常为晚期疾病。这些患者对化疗和手术切除有反应,且大多数神经功能有完全改善。