Indian Journal of Endocrine Surgery and Research

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VOLUME 16 , ISSUE 1 ( Jan-Jun, 2021 ) > List of Articles


Composite Pheochromocytoma—A Rare Clinical Entity: A Case Series

Pushkar Sharma, Paul M Jacob, Santosh Raj, Elanthenral Sigamani, Marie T Manipadam

Keywords : Adrenal, Composite Pheochromocytoma, Ganglioneuroma, Multiple endocrine neoplasia

Citation Information : Sharma P, Jacob PM, Raj S, Sigamani E, Manipadam MT. Composite Pheochromocytoma—A Rare Clinical Entity: A Case Series. 2021; 16 (1):28-31.

DOI: 10.5005/jp-journals-10088-11151

License: CC BY-NC 4.0

Published Online: 26-08-2021

Copyright Statement:  Copyright © 2021; The Author(s).


Background: Composite pheochromocytoma is a rare entity with tumor comprising both pheochromocytoma and neuroblastic elements. The clinical outcome of such a composite tumor is not fully understood. In this case series, we report three cases of composite pheochromocytoma managed at our institute from 2016 to 2021. Results: We had three patients with MEN2A syndrome with composite tumor who underwent adrenalectomy, all of which were ganglioneuroma with pheochromocytoma. None of the three cases had recurrence or metastasis associated with composite adrenal tumor on 13-month mean follow-up. Conclusion: We noticed a higher incidence of syndromic association in composite adrenal tumors. The clinical and radiological presentation was dominated by the pheochromocytoma component.

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  1. Guilmette J, Sadow PM. A guide to pheochromocytomas and paragangliomas. Surg Pathol Clin 2019;12(4):951–965. DOI: 10.1016/j.path.2019.08.009.
  2. Tischler AS, deKrijger RR. 15 years of paraganglioma: pathology of pheochromocytoma and paraganglioma. Endocr Relat Cancer 2015; 22(4):T123–T133. DOI: 10.1530/ERC-15-0261.
  3. Weston JA. The migration and differentiation of neural crest cells. Adv Morphog 1970;8:41–114. DOI: 10.1016/b978-0-12-028608-9.50006-5.
  4. Hu J, Wu J, Cai L, et al. Retroperitoneal composite pheochromocytoma-ganglioneuroma: a case report and review of literature. Diagn Pathol 2013;8(1):63. DOI: 10.1186/1746-1596-8-63.
  5. Dhanasekar K, Visakan V, Tahir F, et al. Composite phaeochromocytomas—a systematic review of published literature. Langenbecks Arch Surg 2021. DOI: 10.1007/s00423-021-02129-5.
  6. Lam K-Y, Lo C-Y. Composite pheochromocytoma-ganglioneuroma of the adrenal gland: an uncommon entity with distinctive clinicopathologic features. Endocr Pathol 1999;10(4):343–352. DOI: 10.1007/BF02739777.
  7. Gupta S, Zhang J, Erickson LA. Composite pheochromocytoma/paraganglioma-ganglioneuroma: a clinicopathologic study of eight cases with analysis of succinate dehydrogenase. Endocr Pathol 2017;28(3):269–275. DOI: 10.1007/s12022-017-9494-3.
  8. Shawa H, Elsayes KM, Javadi S, et al. Clinical and radiologic features of pheochromocytoma/ganglioneuroma composite tumors: a case series with comparative analysis. Endocr Pract 2014;20(9):864–869. DOI: 10.4158/EP14010.OR.
  9. Trump DL, Livingston JN, Baylin SB. Watery diarrhea syndrome in an adult with ganglioneuroma-pheochromocytoma: identification of vasoactive intestinal peptide, calcitonin, and catecholamines and assessment of their biologic activity. Cancer 1977;40(4):1526–1532. DOI:10.1002/1097-0142(197710)40:4<1526::aid-cncr2820400425>;2-7.
  10. Nigawara K, Suzuki T, Onodera T, et al. [Watery diarrhea, hypokalemia, achlorhydria syndrome due to recurrent malignant pheochromocytoma]. Nihon Naibunpi Gakkai Zasshi 1987;63(8):923–933. DOI: 10.1507/endocrine1927.63.8_923.
  11. Onozawa M, Fukuhara T, Minoguchi M, et al. Hypokalemic rhabdomyolysis due to WDHA syndrome caused by VIP-producing composite pheochromocytoma: a case in neurofibromatosis type 1. Jpn J Clin Oncol 2005;35(9):559–563. DOI: 10.1093/jjco/hyi139.
  12. Salmi J, Pelto-Huikko M, Auvinen O, et al. Adrenal pheochromocytoma-ganglioneuroma producing catecholamines and various neuropeptides. Acta Med Scand 1988;224(4):403–408. DOI: 10.1111/j.0954-6820.1988.tb19603.x.
  13. Mahajan H, Lee D, Sharma R, et al. Composite phaeochromocytoma-ganglioneuroma, an uncommon entity: report of two cases. Pathology (Phila) 2010; 42(3):295–298. DOI: 10.3109/00313021003636451.
  14. George DJ, Watermeyer GA, Levin D, et al. Composite adrenal phaeochromocytoma-ganglioneuroma causing watery diarrhoea, hypokalaemia and achlorhydria syndrome. Eur J Gastroenterol Hepatol 2010;22(5):632–634. DOI: 10.1097/MEG.0b013e328311a697.
  15. Khan AN, Solomon SS, Childress RD. Composite pheochromocytoma-ganglioneuroma: a rare experiment of nature. Endocr Pract 2010;16(2):291–299. DOI: 10.4158/EP09205.RA.
  16. Lam K-Y, Lo C-Y. Composite pheochromocytoma-ganglioneuroma of the adrenal gland: an uncommon entity with distinctive clinicopathologic features. Endocr Pathol 1999; 10(4):343–352. DOI: 10.1007/BF02739777.
  17. Gj L, Cm S, Es S, et al. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation. Radiographics 2002;22(4):911–934. DOI: 10.1148/radiographics.22.4.g02jl15911.
  18. Tran L, Fitzpatrick C, Cohn SL, et al. Composite tumor with pheochromocytoma and immature neuroblastoma: report of two cases with cytogenetic analysis and discussion of current terminology. Virchows Arch 2017;471(4):553–557. DOI: 10.1007/s00428-017-2225-9.
  19. Wang J, Zheng W, Qin P, et al. Composite pheochromocytoma/ganglioneuroblastoma of the adrenal gland: a case report and review of literature. Int J Clin Exp Med 2017;10(3):4740–4747.
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