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
- The symptoms of pheochromocytoma usually develop in the fifth decade of life.
- Common symptoms are with tachycardia, hypertension, headache, and sweating.
- If left untreated, hyperglycemia and hypertensive emergency. It may lead to heart failure andcerebrovascular strokes.
- If malignant, It can metastasize to lymph nodes, bones, lungs, and liver. [1]
Complications
- Common complications of pheochromocytoma include:
- Damage to cardiac myocytes due to either a compromise of the coronary microcirculation or the direct toxic effects of catecholamines on cardiac myocytes.[2][2]
- Hyperglycemia due to opposition of insulin effect by high doses of adrenaline secreted by the tumor.
- Malignant hypertension that may cause cerebrovascular accidents such as:
- Metastasis to:
Prognosis
- The prognosis of pheochromocytoma is generally good with treatment. The 5-year survival rate in patients with metastatic pheochromocytoma is approximately 50%.[3]
- Approximately 10% recur after being resected.
- Prognosis and survival rate varies with the location of the primary tumor, sites of metastases, Tumor burden, and rate of progression.
- Metastasis to the brain and liver has a worse prognosis than other metastases.
Post-surgical prognosis
- Features leading to a good prognosis include small tumor size, short duration of surgery, systolic blood pressure less than 160 mmHg, and low levels of urinary catecholamines.[4]
- Postoperative hypotension can be avoided by adequate fluid replacement.
- Recurrence is more in patients with familial pheochromocytoma and extra-adrenal tumors.[5]
- Familial pheochromocytomas have a high incidence of bilateral disease.
- Partial adrenalectomy is recommended for these patients with bilateral pheochromocytomas to prevent permanent glucocorticoid deficiency.
- Complete bilateral adrenalectomy is recommended for MEN2B patients. There is a high rate of recurrence compared to sporadic cases. Follow-up for a long duration after surgery is required. Most patients should undergo annual biochemical screening.
References
- ↑ 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. doi:10.1210/jc.2016-3829. PMID 28324046.
- ↑ 2.0 2.1 Goldman 2011, pp. 327
- ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc
- ↑ 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. doi:10.1007/s00464-010-0996-z. PMID 20336320.
- ↑ 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. doi:10.1210/jcem.86.4.7392. PMID 11297571.