Polycythemia Secondary to Pheochromocytoma

  • Raheel Raza Holy Family Hospital
  • Saima Ambreen Holy Family Hospital
  • Hassan Mumtaz KRL Hospital
  • Shahzaib Ahmad King Edward Medical University
  • Hadin Darain Khan Shalimar Medical & Dental College
Keywords: polycythemia, pheochromocytoma, tumor management

Abstract

Polycythemia, also known as polyglobulia, is a clinical condition characterized by an increased number of red blood cells (RBC) or haematocrit concentrations in the peripheral blood. It can either be primary (polycythemia vera) or secondary, which can be congenital or acquired; the most common causes include obstructive sleep apnoea, obesity, hypoventilation, Pickwickian syndrome, Chronic obstructive pulmonary disease (COPD), and lastly, pheochromocytoma.

Here we present a case of a 54-year-old male with a four-day history of altered state of consciousness (ASOC), right-sided body weakness, and respiratory difficulty. After a thorough history, examination, and investigation, he was diagnosed as a case of polycythemia secondary to pheochromocytoma. Early diagnosis and intervention are critical to save the patient’s life.

Author Biographies

Raheel Raza, Holy Family Hospital

Post Graduate Resident

Medical Unit-1

Saima Ambreen, Holy Family Hospital

Associate Professor of Medicine

Head of Department- Medical Unit 1

Hassan Mumtaz, KRL Hospital

House physician

Critical Care Medicine

Shahzaib Ahmad, King Edward Medical University

Final Year Medical student

General Secretary: Research Wing

Hadin Darain Khan, Shalimar Medical & Dental College

Final Year Student

General Secretary: Research Wing

References

1. Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and Paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-565. DOI: 10.1056/NEJMra1806651. PMID: 31390501.
2. Aygun N, Uludag M. Pheochromocytoma and Paraganglioma: From Epidemiology to Clinical Findings. Sisli Etfal Hastan Tip Bul. 2020 Jun 3;54(2):159-168. DOI: 10.14744/SEMB.2020.18794. PMID: 32617052; PMCID: PMC7326683.
3. Pang Y, Gupta G, Yang C, et al. A novel splicing site IRP1 somatic mutation in a patient with pheochromocytoma and JAK2V617F positive polycythemia vera: a case report. BMC Cancer. 2018 Mar 13;18(1):286. DOI: 10.1186/s12885-018-4127-x. PMID: 29534684; PMCID: PMC5850917.
4. Yeung SCJ, Gagel RF. Endocrine Paraneoplastic Syndromes ("Ectopic" Hormone Production) In Kufe DW, Pollock RE, Weichselbaum RR, et al., editors. Holland-Frei Cancer Medicine. 6th edition. Hamilton (ON): BC Decker; 2003. Available from: https://www.ncbi.nlm.nih.gov/books/NBK12609/
5. Sourty B, Rousseau A. Hereditary predisposition to tumors of the central and peripheral nervous systems. Ann Pathol. 2020 Apr;40(2):168-179. Epub 2020 Mar 17. PMID: 32192808.
6. Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, et al. Biochemical diagnosis of pheochromocytoma: Which test is best? JAMA. 2002;287:1427–34.
7. Unger N, Pitt C, Schmidt IL, Walz MK, Schmid KW, Philipp T, et al. Diagnostic value of various biochemical parameters for the diagnosis of pheochromocytoma in patients with adrenal mass. Eur J Endocrinol. 2006;154:409–17.
8. Naranjo J, Dodd S, Martin YN. Perioperative Management of Pheochromocytoma. J Cardiothorac Vasc Anesth. 2017 Aug;31(4):1427-1439. doi: 10.1053/j.jvca.2017.02.023. Epub 2017 Feb 4. PMID: 28392094.
9. Guller U, Turek J, Eubanks S, Delong ER, Oertli D, Feldman JM. Detecting pheochromocytoma: Defining the most sensitive test. Ann Surg. 2006;243:102–7.
10. Garg MK, Kharb S, Brar KS, Gundgurthi A, Mittal R. Medical management of pheochromocytoma: Role of the endocrinologist. Indian J Endocrinol Metab. 2011 Oct;15 Suppl 4(Suppl4):S329-36. doi: 10.4103/2230-8210.86976. PMID: 22145136; PMCID: PMC3230088.
11. Shulkin BL, Ilias I, Sisson JC, Pacak K. Current trends in functional imaging of pheochromocytomas and paragangliomas. Ann N Y Acad Sci. 2006;1073:374–82.
12. Clinical Staff Conference. Pheochromocytoma: current concepts of diagnosis and treatment. Combined Clinical Staff Conference at the National Institutes of Health. Ann. Intern. Med. 65: 1302-1326, 1966.
13. Pelosof LC, Gerber DE. Paraneoplastic syndromes: an approach to diagnosis and treatment. Mayo Clin Proc. 2010 Sep;85(9):838-54. doi: 10.4065/mcp.2010.0099. Erratum in: Mayo Clin Proc. 2011 Apr;86(4):364. Dosage error in article text. PMID: 20810794; PMCID: PMC2931619.
Published
2021-12-31
How to Cite
1.
Raza R, Ambreen S, Mumtaz H, Ahmad S, Khan H. Polycythemia Secondary to Pheochromocytoma. JRMC [Internet]. 31Dec.2021 [cited 21Jan.2022];25(4). Available from: https://journalrmc.com/index.php/JRMC/article/view/1655
Section
Case Reports