gms | German Medical Science

Objective: Infantile fibrosarcoma (IFS) is a rare malignant mesenchymal tumor. Suggestive clinical features are a palpable and/or visible, painless soft tissue mass with rapid growth. Most cases are diagnosed within the first year of life. Treatment strategies consist of surgery and/or chemotherapy. 5-year overall survival is up to 90%. The majority of cases are located on the limbs and the trunk. There is no literature concerning intraspinal manifestations of IFS.

Method: We present the case of an IFS arising from the neck region showing continuous growth in the cervical spinal canal with severe neurological impairment.

Results: Primary examination of a newborn girl showed complete tetraplegia and severe respiratory insufficiency. Endotracheal intubation was necessary. A soft swelling was visible on the left side of the neck. MRI showed a large, lesion with signs of hemorrhage infiltrating the cervical spinal canal with massive spinal cord compression. Transferred to our pediatric intensive care unit on day 5, we saw spastic paralysis of upper and atonic paralysis of lower limbs with insufficient spontaneous breathing. On day 6 we performed partial hemilaminectomies from C1-C7 and gross-total resection of the intraspinal, extradural tumor; the extraspinal tumor was not resected. Nerve roots C1-C3 had to be sacrificed. After decompression of the spinal cord no pulsation of the thecal sac was visible. Intraoperative histopathology was suggestive for neuroblastoma, however final analysis showed infantile fibrosarcoma. Postoperative MRIs showed no residual intraspinal tumor, and sufficient decompression. Extubation was possible 25 days after the operation. Nine cycles of chemotherapy (doxorubicin, vincristine, cyclophosphamide) were administered. Tetraplegia improved with reduction of spasticity and spontaneous movements of the limbs. Extraspinal tumor manifestation showed complete response to chemotherapy. On the latest follow-up visit 6 years after surgery, we saw a girl in good general condition. She uses both hands, can move her wheelchair and sit with orthosis. Intellectual development is age-appropriate and she attends regular primary school. MRI shows complete response without signs of relevant tumor recurrence.

Conclusions: In the first reported case of intraspinal IFS with spinal cord compression causing tetraplegia and severe respiratory insufficiency, tumor remission and good neurological outcome were achieved after resection of the intraspinal tumor and postoperative chemotherapy.