World Journal of Endocrine Surgery

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VOLUME 6 , ISSUE 2 ( May-August, 2014 ) > List of Articles


Functional Retroperitoneal Neuroblastoma and Ganglioneuroblastoma in Adults

Kamilla Mahkamova, Barney J Harrison

Citation Information : Mahkamova K, Harrison BJ. Functional Retroperitoneal Neuroblastoma and Ganglioneuroblastoma in Adults. World J Endoc Surg 2014; 6 (2):89-95.

DOI: 10.5005/jp-journals-10002-1144

Published Online: 01-04-2008

Copyright Statement:  Copyright © 2014; The Author(s).


Ganglioneuroblastoma and neuroblastoma are malignant catecholamine-secreting tumors arising from neural crest tissue. Thirty-eight cases of functional retroperitoneal extra-adrenal neuroblastoma and ganglioneuroblastoma in adults are reported in English literature. These tumors behave more aggressively in adults compared to children and have very poor prognosis. We report four adults who presented with retroperitoneal extraadrenal ganglioneuroblastoma and neuroblastoma between 2008 and 2012. Median age at presentation was 28.5 years (21-40 years). One patient was in late stages of pregnancy. Three patients presented with local pressure effects from the tumor and one patients manifested clinical features of catecholamine excess. Biochemical analysis showed raised urinary dopamine excretion in all our patients. Urinary noradrenaline and metanephrines were elevated in three cases. Computed tomography/magnetic resonance imaging/meta-iodobenzylguanidine (CT/MRI/MIBG) confirmed stage IIB disease in one patient, stage III disease one patient and stage IV in two cases. Preoperative biopsy confirmed diagnosis in two cases. Complete surgical resection was performed in patient with stage II B disease and partial surgical resection was feasible in other cases. Histological diagnosis was ganglioneuroblastoma in two cases and neuroblastoma in two cases. Meta-iodobenzylglunidine/radiotherapy and chemotherapy were used for residual disease. Urinary catecholamines and metanephrines normalized after complete tumor resection but remained elevated in residual disease. Survival for neuroblastoma was 15 and 21 months after the surgery and 29 months for ganglioneuroblastoma. One patient remains free of disease at 6 months. Functioning ganglioneuroblastoma and neuroblastoma are rare in adults and only one other case ganglioneuroblastoma in pregnancy is reported in the world literature. Measurement of plasma/urinary excretion of catecholamines and their precursors and metabolites is helpful for diagnosis of neuroblastoma and ganglioneuroblastoma. Due to late presentation surgical resection is unlikely to be curative. Three of our patients developed local recurrence of disease. Urinary/plasma assay are used for monitoring disease relapse. Long-term survival is unusual.

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