Sandoval C, Oiseth S, Slim M, Tugal O, Ozkaynak M F, Brudnicki A, Beneck D, Bostwick H, Jayabose S
Department of Pediatrics, Pathology, Pediatric Surgery and Radiology, New York Medical College, Valhalla 10595, USA.
J Pediatr Hematol Oncol. 1996 Nov;18(4):409-12. doi: 10.1097/00043426-199611000-00016.
This report describes a female infant with stage 4 multifocal ganglioneuroblastoma with gastric involvement.
The patient had a right cervical tumor, a left posterior mediastinal tumor, bilateral adrenal tumors, and bony and bone marrow metastases. The tumor cells were diploid and lacked N-myc gene amplification. The gastric involvement, which did not produce clinical symptoms, was only detected by meticulous exploration during laparotomy.
Our patient achieved only a partial response to alternating cycles of cyclophosphamide, vincristine, and adriamycin; and etoposide and cisplatin. She currently has stable, unresectable disease with elevated catecholamines.
Multifocal ganglioneuroblastomas may arise from either neuroblastic rests or aberrant deposits of neuroblasts. The latter mechanism may have accounted for our patient's gastric tumor. Patients with multifocal ganglioneuroblastomas warrant meticulous radiographic and surgical evaluation to completely document the full extent of disease, and to ensure appropriate staging and therapy.
本报告描述了一名患有4期多灶性神经节神经母细胞瘤并累及胃部的女婴。
该患者有右颈部肿瘤、左后纵隔肿瘤、双侧肾上腺肿瘤以及骨骼和骨髓转移。肿瘤细胞为二倍体，且无N - myc基因扩增。胃部受累未产生临床症状，仅在剖腹手术时通过细致探查才被发现。
我们的患者对环磷酰胺、长春新碱和阿霉素；以及依托泊苷和顺铂交替化疗周期仅产生部分反应。她目前患有病情稳定、无法切除且儿茶酚胺升高的疾病。
多灶性神经节神经母细胞瘤可能起源于神经母细胞残余或神经母细胞的异常沉积。后一种机制可能是导致我们这位患者胃部肿瘤的原因。多灶性神经节神经母细胞瘤患者需要进行细致的影像学和手术评估，以全面记录疾病的完整范围，并确保进行恰当的分期和治疗。
