Faculty Bibliography

Cyclotron-produced L-(N-13)glutamate was used to visualize malignant intracranial tumors in 12 pediatric patients who had evidence of recurrent disease as documented by computed transaxial tomography (TCT). Imaging was performed using a rectilinear scanner, gamma camera, or a positron-emission tomograph (PET). The results indicate that N-13 is rapidly taken up by a majority of brain tumors following the administration of L-(N-13)glutamate, and that N-13 uptake is correlated with breakdown of the blood-brain barrier as demonstrated by contrast TCT or pertechnetate (Tc-99m) studies. The feasibility of using this agent in conjunction with PET is established

A 6-year-old child with a brain stem tumor presented with the unusual complaint of intractable facial pain resembling trigeminal neuralgia in the absence of other symptoms or signs referable to the 5th cranial nerve. The radiological evaluation included a computed tomographic scan with intravenous contrast administration, which demonstrated an enhancing intramedullary lesion extending from the obex to C-4. After radiation and chemotherapy had failed to achieve symptomatic relief, the tumor, later proven to be a ganglioglioma, was radically removed with the ultrasonic aspirator. Postoperatively the patient experienced full pain relief.

Dural sinus thrombosis developed in two children with acute lymphoblastic leukemia during induction treatment with vincristine sulfate, prednisone, and asparaginase. Headache, nausea, emesis, and lethargy were the presenting signs. The diagnosis was confirmed by arteriography. The cause is presumed to be secondary to hypercoagulability due to asparaginase-induced antithrombin III deficiency. The patients received anticoagulation therapy and recovered completely. Only two of the six reported patients without heparinization survived

A Phase Ii chemotherapy trial was conducted in 18 children with recurrent brain tumors, using high doses (80 mg/kg or greater) of intravenous cyclophosphamide cyclophosphamide. All eight patients with medulloblastomas responded; two patients with systemic metastases had complete responses and six others had partial responses. In seven patients with gliomas, there were one complete and four partial responses. In a third group, all three patients with intracranial germ-cell tumors had partial responses. The overall response rate was, therefore, 89% (16 of 18 patients), and the mean duration of response was 7 months (range 2 to 24 or more months). The hematological toxicity was considerable, with two deaths possibly related to chemotherapy: one patient, a recipient of unirradiated packed cells, died from a graft versus host reaction, and the other died from an intracranial hemorrhage during a thrombocytopenic episode. Four patients had prior chemotherapy, and 10 patients had prior neuraxis radiation therapy. These patients tolerated aggressive chemotherapy reasonably well. The results are sufficiently encouraging to justify a Phase III trial in patients with newly diagnosed disease

A 16-year-old boy was noted to have an apparent intradural sacral mass 6 weeks after the excision of a 4th ventricle ependymoma. Although spinal axis radiation was considered, it was decided to make a definitive tissue diagnosis. At sacral laminectomy, only segmental arachnoiditis was found. The implications of this finding are the subject of this case report.

Serial echocardiographic (ECHO) studies were obtained on 40 pediatric patients (pts) treated with greater than or equal to 80 mg/kg cyclophosphamide (CPM) (range 80-200 mg/kg) in 1 week. Patients were treated for solid tumors and prior to marrow transplant. Echo changes occurred in 10/13 pts who received CPM greater than or equal to 170 mg/kg over four days, and in 11/19 pts who received 120-140 mg/kg dose over two days and who had previously received greater than or equal to 100 mg/m2 anthracyclines with or without radiation. No changes were seen in eight pts who had 80-160 mg/kg CPM and less than 100 mg/m2 anthracyclines. The observed changes occurred approximately 1 week after CPM and persisted for days to weeks. Pericardial effusion seen in 15 pts was successfully treated with furosemide in 13. Two died with hemorrhagic pancarditis. Other changes seen were increased end diastolic left ventricular diameter, decreased fractional shortening and abnormal left ventricular preejection period/ejection time ratios. Thus, cardiac effects of high dose CPM are not rare in children. Patients receiving greater than 170 mg/kg CPM in 1 week or 120 mg/kg in 1 week after greater than or equal to 100 mg/m2 anthracyclines are at particular risk

Forty-two children with various systemic malignancies in continuous remission for 1 to 3 years after the completion of chemotherapy had CT scans with normal ventricular dimensions, similar to a noncancer 'control' population. Seventeen of these patients had acute lymphocytic leukemia (ALL) treated either with prophylactic cranial irradiation and intrathecal methotrexate [7] or intrathecal methotrexate alone [10] and the remaining 25 patients had soft tissue sarcomas. Sixteen other patients with sarcomatous meningitis had enlarged ventricles while on chemotherapy. Nine had ALL. Seven had soft tissue sarcomas, none of whom received any prior CNS irradiation or intrathecal chemotherapy. In this retrospective study no evidence of hydrocephalus or significant white matter hypodensity was detected in long-term survivors of childhood cancer, regardless of whether prophylactic intrathecal chemotherapy and/or cranial irradiation was given. Direct involvement of the CNS with meningeal cancer was the most important association with ventriculomegaly

Neurofibromatosis includes the common 'peripheral' form and a recently documented 'central' form. We describe the central form in 130 cases from 9 kindreds personally studied and 15 reported kindreds. Central neurofibromatosis with bilateral acoustic neuroma is an autosomal dominant disorder beginning about 20 years of age, accompanied by mild skin changes. In three kindreds with central neurofibromatosis, we measured nerve growth factor in serum by radioimmunoassay and radioreceptor assay. Only the antigenic activity of nerve growth factor was increased. In contrast, in peripheral neurofibromatosis, only the functional activity of nerve growth factor has been reported increased. Central and peripheral forms of neurofibromatosis are closely related but discrete diseases which appear to have separate alterations in nerve growth factor activity