Sacrococcygeal malignant germ cell tumor (SC-MGCT) with intraspinal extension

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• 作者：Rajan Garg^a ; ^{rajangarg1985@gmail.com" class="auth_mail} ; Sandeep Agarwala^a ; ^{sandpagr@hotmail.com" class="auth_mail} ; Sameer Bakhshi^b ; ^{sambakh@hotmail.com" class="auth_mail} ; M. Srinivas^a ; ^{srinivasem@hotmail.com" class="auth_mail} ; Minu Bajpai^a ; ^{bajpai2@hotmail.com" class="auth_mail} ; Devendra Kumar Gupta^a ; ^{dkgupta@kgmcindia.edu" class="auth_mail} ; Veereshwar Bhatnagar^a ; ^{veereshwarb@hotmail.com" class="auth_mail}
• 关键词：Sacrococcygeal tumors ; Malignant germ cell tumor ; Intraspinal extension
• 刊名：Journal of Pediatric Surgery
• 出版年：July 2014
• 年：2014
• 卷：49
• 期：7
• 页码：1113-1115
• 全文大小：309 K

文摘

Neurological involvement due to intraspinal extension in sacrococcygeal malignant germ cell tumors (SC-MGCTs) has rarely been reported.

Aim

To evaluate the incidence, presentation, management and the outcome of patients of SC-MGCT with intraspinal extension.

Materials and Methods

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.

Results

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.

Conclusions

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.