Urinary excretion of 3-methoxy-4-hydroxymandelic acid and 3-methoxy-4-hydroxyphenylacetic acid by 288 patients with neuroblastoma and related neural crest tumors
LaBrosse, E.H.; Com-Nougué, C.; Zucker, J.M.; Comoy, E.; Bohuon, C.; Lemerle, J.; Schweisguth, O.
Cancer Research 40(6): 1995-2001
1980
ISSN/ISBN: 0008-5472 PMID: 7371035 Document Number: 165997
3-Methoxy-4-hydroxymandelic acid (VMA) and 3-methoxy-4-hydroxyphenylacetic acid (HVA) were assayed in initial urine specimens of 288 patients with neuroblastoma and related tumors of neural crest origin (NCT). The interrelationships between the excretion of these metabolites, age at diagnosis, stage of the disease, site of the primary tumro and prognosis were examined. Excretion by children with Wilms' tumor was examined as a control group. VMA was excreted above the upper limits of controls by 204 (71%) of 288 NCT patients and HVA was excreted by 196 (75%) of 260 NCT patients for whom data were available. VMA excretion of the younger NCT patients was significantly higher than that of NCT patients older than 1 yr. Patients in stages IV and IV-S excreted VMA at levels significantly higher than did those in stages I-III. There was no significant relationship of VMA and HVA excretion to the primary site of the tumor. Age, stage of the disease and site of the primary tumor were each independently related to survival. The patients with extraadrenal abdominal tumors had the poorest prognosis followed by adrenal, thoracic, pelvic and cervical tumors in increasing order of survival. HVA and the VMA/HVA ratio were significantly related to survival, but VMA excretion was not. When data on the stage IV patients were analyzed separately, there was no significant relationship between survival time and VMA or HVA excretion, but the VMA/HVA ratio was significantly related to survival. The initial urinary excretion levels of VMA and HVA are of value as an aid in diagnosis of 75%, or more, of patients with neuroblastoma and related tumors. Elevated levels of VMA and/or HVA in initial urine specimens permit a definitive diagnosis of a neural crest tumor even when histological data are lacking or are nonspecific. The relationships between urinary VMA and HVA and prognosis suggest that there is a common factor between the catecholamine metabolism within the tumor cells and their malignancy as reflected by the survival of the patients.