URINARY-EXCRETION OF METHYLATED CATECHOLAMINE METABOLITES IN A CHILD WITH NEUROBLASTOMA MATURING INTO GANGLIONEUROMA

Neuroblastomas are malignant tumors derived embryonically from the neu tral crest. Biological diagnosis relies on assay of urinary excretion of homovanillic acid (HVA), vanillylmandelic acid (VMA), and dopamine (DA). Spontaneous regression of these neoplasms has been reported by n umerous investigators. The authors report the case of a child with neu roblastoma that illustrates the relationship between catecholamine met abolites and tumor maturation. At 1 month of age, this infant presente d an adrenal neuroblastoma with multiple metastases (stage IV); the in itial histological diagnosis based on examination of cutaneous metasta ses was neuroblastoma. At the age of 6 months, after chemotherapy, the primary tumor was resected; hepatic metastases were discovered at lap arotomy. The histological diagnosis for all lesions was highly differe ntiated, mature ganglioneuroma-like tissue. The main biochemical abnor mality at the time of diagnosis was an elevation in normetanephrine (N MN). HVA was only slightly increased but rose progressively during che motherapy; it dropped back to normal levels after the sixth course. Th is case illustrates the potential benefits of separate assays of urina ry methylated catecholamine metabolites for biochemical diagnosis and therapeutic management of neuroblastoma in addition to assays of HVA, VMA, and DA. Case findings suggest existence of a transformation proce ss with maturation of the tumor involving enzymatic regulation and exp ression of MAO. (C) 1996 Wiley-Liss, Inc.