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Sacrococcygeal malignant germ cell tumor (SC-MGCT) with intraspinal extension
Rajan Garg1, Sandeep Agarwala1, Sameer Bakhshi2
1Department of Pediatric Surgery, All India Institute of Medical Sciences, New Delhi 110029, India.
Background:
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.
