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

CASE REPORT

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).


Abstract

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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  1. The incidence and epidemiologic characteristics of neuroblastoma in the United States. Am J Epidemiol 1987;126(6):1063-1074.
  2. Neuroblastoma in adults: incidence and survival analysis based on SEER data. Paediatric blood and cancer 2007;49(1):41-46.
  3. Cerebral ganglioneuroblastoma of adult onset: two patients and review of literature. Clinical neurology and neurosurgery 2012;114(6):529-534.
  4. Adult neuroblastoma: report of three cases and review of the literature. Cancer 1986;57(12):2419-2421.
  5. Neuroblastoma in adults. Pathologic findings and clinical outcomes. Arch Pathol Lab Med 1985;109(4):352-356.
  6. Ganglioneuroblastoma in Adults. Cancer 1976;37(2):974-983.
  7. Noradrenaline-secreting neuroblastomata. Br Med J 1959;1(5119):401-404.
  8. Catecholamine-secreting tumours. Proc R Soc Med 1967;60(8):795-797.
  9. Medulloblastoma and Neuroblastoma in adults. Neth J Med 1982;25(4):94-99.
  10. Abdominal sympathoblastoma in adult with lung abscess. J Ind Medicine 1975;64(9):240-242.
  11. A case of neuroblastoma in an adult with raised catecholamines. Br J Surg 1970;57(7):551-553.
  12. Neuroblastoma in adults. Post Grad Med J 1979;55(646):579-580.
  13. Neuroblastoma in adults and adolescents. An indolent course with poor survival. Cancer 1997;79(10):2028-2035.
  14. Neuroblastoma in Adulthood. Turk J Med Sci 2008;38(3):281-283.
  15. Adult neuroblastoma in pregnancy: report of a case. Surgery Today 1993;23(8):742-746.
  16. Adult type neuroblastoma originated in retroperitoneum beginning with obstructive jaundice. J Clin Imaging 2001;25(4):284-287.
  17. Neuroblastoma occurring in a 38-year-old Nigerian man: a rare finding. Rare Tumours 2009;1(1):42-43.
  18. Treatment of adult neuroblastoma. Cancer 1980;45(5):840-844.
  19. Neuroblastoma in the adult. Effective combination chemotherapy. Cancer 1978;41(1):56-63.
  20. Adult neuroblastoma of the retroperitoneum and abdomen: clinicopathologic distinction from primitive neuroectodermal tumor. Am J Surg Pathol 2001;25(7):918-924.
  21. Adult neuroblastoma. Electron microscopic observations in 9 cases. Cancer 1976;37(3):1334-1351.
  22. Treatment results among adults with childhood tumours: a 20 year experience. Int J radiat Oncol Biol Phys 1989;17(3):507-514.
  23. Neuroblastoma presenting as renal cell carcinoma in an adult. Urology 1975;6(3):376-378.
  24. Neuroblastoma in adolescents. Cancer 2006;106(6):1409-1417.
  25. Primary retroperitoneal ganglioneuroblastoma in an adult. Int J Urology 2001;8(3):130-132.
  26. Adult type ganglioneuroblastoma in the adrenal glan treated by a laparoscopic resection: report of case. Surgery Today 2003;33(10):785-790.
  27. High catecholamine-producing retroperitoneal ganglioneuroblastoma in adult. Orv Hetil 1971;112(36):2162-2164.
  28. Primary renal ganglioneuroblastoma in adult. Post Grad Med J 1990;66(780):869-971.
  29. Nodular ganglioneuroblastoma in adults. Canadian J Surgery 2009;52(4):E111-E113.
  30. Life-treatening complications of extremely rare tumour of the kidney: adult-type primary renal ganglioneuroblastoma. Sourth Med J 2007;100(8):852-853.
  31. Retroperitoneal ganglioneuroblastoma in an adult with itratumoural bleed. Ind J Radiolog Image 2006;16(2):193-196.
  32. Bilateral intra-abdominal ganglioneuroblastoma in adult. Br J Radiology 1997;70:96-98.
  33. Adrenaline-secreting neuroblastoma in infant. Lancet 1957;273(6990):322-325.
  34. Further observations on the determination of catecholamine metabolites in tumours of sympathetic nervous system. Arch Dis Childh 1964;39(204):168-171.
  35. Studies of Catecholamine excretion by children with neural tumours. J clin Endocrinol 1962;22:126-133.
  36. The excretion of dixydrophenylanine, dopamine and dihydroxyphenylacetic acid in neuroblastoma. Pediatrics 1963;31:660-668.
  37. Spectrum of catechol amine biochemistry in patients with neuroblastoma. NEJM 1963;269:232-235.
  38. Methylated catecholamine metabolites for diagnosis of neuroblastoma. Med Paed Oncology 1992;20(3):215-220.
  39. Urinary catecholamines and their metabolites in management of neuroblastoma. Paed Haem Oncol 1988;5(3):229-237.
  40. Ewing's tumours and neuroblastomas. J clin Path 1964;17(4):411-417.
  41. An adrenaline-secreting ganglioneuroblastoma with elevated urinary HMMA but normal metanephrine secretion. Ann Clin Biochem 1992;29(pt 6):678-680.
  42. Secretion of catecholamines in malignant paechromocytoma. BMJ 1964;1(5395):1422-1424.
  43. Spectrum of catecholamine biochemistry in patients with neuroblastoma. N Engl J Med 1963;269:232-235.
  44. Neuroblastoma. Biochemical studies, in recent results in cancer research. In: Bohuon, editor. Berlin; 1966. p. 55.
  45. Intraoperative hypertension during surgical excision of neuroblastoma case report and review of 20 years experience. Anesth Analg 1992;75(5):854-858.
  46. 123I-meta-iodobenzylguanidine scintigraphy for the detection of neuroblastoma and pheochromocytoma: results of a meta-analysis. J Clin Endocrinol Metabol 2010;95(6):2596-2606.
  47. Revisions of the international criteria for neuroblastoma diagnosis, staging, and response to treatment. J clin Oncol 1993;11(8):1466-1467.
  48. Primary adrenal ganglioneuroblastoma in adult. Internal Medicine 1995;34(12):1168-1173.
  49. Neuroblastoma. Principles and practice of paediatric oncology. Philadelphia: Lippincott Williams and Wilkings; 2002. p. 895-937.
  50. Neuroblastoma. The joint centre for radiation therapy/dana-farber cancer institute/children's hospital experience. J Clin Oncol 1984;2(7):719-732.
  51. Improved survival in stage II and improved response rate in stage IV neuroblastoma. Proc Am Soc Clin Oncol 1984;2(7):799-803.
  52. Neuroblastoma in adults-three case reports and review of the literature. Cancer 1986;58(5):1149-1157.
  53. Phase II study on the effect of disease sites, age, and prior therapy on response to iodine- 131-metaiodobenzylguanidine therapy in refractory neuroblastoma. J Clin Oncol 2007;25(9):1054-1060.
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