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, usually develop in the fifth decade of life. Symptoms start with tachycardia, hypertension, headache and sweating. Massive release of catecholamines can 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%[1]
Natural History, Complication and Prognosis
Natural History
- Pheochromocytoma is the adrenaline secreting tumor, 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.
- Most catecholamine-secreting tumors are sporadic.
- However, 30% of patients got the tumor as a part of the familial disease, the catecholamine-secreting tumors are more likely to be bilateral.[2]
Symptoms start with tachycardia, hypertension, headache and sweating. If left untreated it causes hyperglycemia, hypertension emergency that causes heart failure, cerebrovascular strokes. If malignant, It can metastasize to lymph nodes, bones, lungs, and liver.
Complication
- The massive release of catecholamines in pheochromocytoma can cause damage to myocytes.[3] This damage may be due to either a compromise of the coronary microcirculation or the direct toxic effects of catecholamines on myocytes.[3]
Other complications may include:
- Hyperglycemia due to opposing insulin effect by high doses of adrenaline secreted by the tumor.
- Malignant hypertension that may cause cerebrovascular accidents such as intracranial hemorrhage, coronary syndrome, aortic dissection and heart failure.
- Metastasis to lymph nodes, bones, lungs, and liver.
Prognosis
- Pheochromocytoma prognosis is good if treated.
- Approximately 10% recur after being resected.
- Patients with metastatic pheochromocytoma five-year survival rates are approximately 50%.[1]Survival rate may depend on the primary tumor site and sites of metastases.
- Tumor burden, the location of metastases, and rate of progression are the main factors affecting prognosis. Metastases in the brain, the liver has a worse prognosis than other metastases.
- Prognosis of surgery
- Good prognosis patients 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 MEN2 patients. There is a high rate of recurrence than in sporadic cases. Follow up for a long duration after surgery is needed. Most patients should have annual biochemical screening.
References
- ↑ 1.0 1.1 National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc
- ↑ 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.
- ↑ 3.0 3.1 Goldman 2011, pp. 327
- ↑ 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.