Pheochromocytoma natural history, complications and prognosis

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2] Mohammed Abdelwahed M.D[3]

Overview

Pheochromocytoma is an adrenaline secreting tumor, that usually develops in the fifth decade of life. Symptoms start with tachycardia, hypertension, headache and sweating. Massive release of catecholamines may cause hyperglycemia, malignant hypertension and metastasis. The prognosis of pheochromocytoma is generally good but metastatic pheochromocytoma has a 5-year survival rate of approximately 50%.

Natural History, Complications and Prognosis

Natural History

  • Pheochromocytoma is the adrenaline secreting tumor, that usually develop in the fifth decade of life.
  • Fifty percent of pheochromocytomas in children are solitary intra-adrenal lesions, 25% are present bilaterally, and 25% are extra-adrenal.
  • Majority of catecholamine-secreting tumors are sporadic.
  • However, 30% of patients get the tumor as a part of the familial disease. These catecholamine-secreting tumors are more likely to be bilateral.[1]

Symptoms start with tachycardia, hypertension, headache, and sweating. If left untreated, it leads to hyperglycemia and hypertensive emergency that causes heart failure andcerebrovascular strokes. If malignant, It can metastasize to lymph nodes, bones, lungs, and liver.

Complications

Other complications may include:

Prognosis

Post-surgical prognosis

References

  1. Pamporaki C, Hamplova B, Peitzsch M, Prejbisz A, Beuschlein F, Timmers HJLM; et al. (2017). "Characteristics of Pediatric vs Adult Pheochromocytomas and Paragangliomas.". J Clin Endocrinol Metab. 102 (4): 1122–1132. PMID 28324046. doi:10.1210/jc.2016-3829. 
  2. 2.0 2.1 Goldman 2011, pp. 327
  3. National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc
  4. Murphy MM, Witkowski ER, Ng SC, McDade TP, Hill JS, Larkin AC; et al. (2010). "Trends in adrenalectomy: a recent national review.". Surg Endosc. 24 (10): 2518–26. PMID 20336320. doi:10.1007/s00464-010-0996-z. 
  5. Plouin PF, Duclos JM, Soppelsa F, Boublil G, Chatellier G (2001). "Factors associated with perioperative morbidity and mortality in patients with pheochromocytoma: analysis of 165 operations at a single center.". J Clin Endocrinol Metab. 86 (4): 1480–6. PMID 11297571. doi:10.1210/jcem.86.4.7392. 

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