GASTRIC GANGLIONEUROBLASTOMA - A RARE FINDING IN AN INFANT WITH MULTIFOCAL GANGLIONEUROBLASTOMA

Purpose: This report describes a female infant with stage 4 multifocal ganglioneuroblastoma with gastric involvement. Patient: The patient h ad a right cervical tumor, a left posterior mediastinal. tumor, bilate ral adrenal tumors, and bony and bone marrow metastases. The tumor cel ls were diploid acid lacked N-myc gene amplification. The gastric invo lvement, which did not produce clinical symptoms, was only detected by meticulous exploration during laparotomy. Results: Our patient achiev ed only a partial response to alternating cycles of cyclophosphamide, vincristine, and adriamycin; and etoposide and cisplatin. She currentl y has stable, unresectable disease with elevated catecholamines. Concl usions: Multifocal ganglioneuroblastomas may arise from either neurobl astic rests or aberrant deposits of neuroblasts. The latter mechanism may have accounted for our patient's gastric tumor. Patients with mult ifocal ganglioneuroblastomas warrant meticulous radiographic and surgi cal evaluation to completely document the full extent of disease, and to ensure appropriate staging and therapy.