The perioperative course and outcome of 17 children who underwent surgical resection of an intra-axial cervicomedullary tumor between 1980-1992 are reported from the Departments of Pediatrics and Neurology, University of Michigan Medical Center, Ann Arbor, MI, and New York University Medical Center, New York, NY. One group of 11 with no treatment before surgery was compared to 6 who had signs of progression following previous radiotherapy. Surgical resection was total in 2 and partial in 15. Low-grade glial tumors were found in 15 and anaplastic gangliogliomas in 2. Symptoms were present for a mean of 2.1 years before diagnosis. They were medullary in 11, including nausea, vomiting, head tilt, dysarthria, and dysphagia; and consistent with cervical cord involvement in 8. Four-year progression-free rates for newly diagnosed and radiation-relapsed patients were 70% and 41%, respectively. The prognosis was superior to that of typical pontine brainstem tumors not amenable to surgery. [1]

COMMENT. Cervicomedullary tumors are a distinct sub-group of brainstem tumors with a slowly progressive course and low-grade malignancy. They appear to be amenable to surgery, and a favorable prognosis is expected in two thirds. Patients who have relapsed following prior radiotherapy have a higher morbidity. Preliminary results of chemotherapy in recurrent intrinsic brainstem gliomas of childhood are encouraging.

Five children with brainstem gliomas (1 cervicomedullary type) were treated with tamoxifen (80 mg/m2), a nonsteroidal, antiestrogen chemotherapy, at Children’s Mercy Hospital, Kansas City, MO. Four have shown objective shrinkage as measured by MRI during treatment from 6 to 30 months. Side-effects were described as minimal. [2]