Pheochromocytoma natural history, complications and prognosis
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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
- 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.
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.
- The massive release of catecholamines in pheochromocytoma can cause damage to cardiac myocytes. This damage may be due to either a compromise of the coronary microcirculation or the direct toxic effects of catecholamines on cardiac myocytes.
Other complications may include:
- 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:
- The prognosis of pheochromocytoma is good with treatment.
- Approximately 10% recur after being resected.
- Without treatment, patients with metastatic pheochromocytoma have a five-year survival rate of approximately 50%. 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. Metastasis to the brain and liver has a worse prognosis than other metastases.
- 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.
- Postoperative hypotension can be avoided by adequate fluid replacement.
- Recurrence is more in patients with familial pheochromocytoma and extra-adrenal tumors.
- 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.
- 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.
- 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. PMID 20336320. doi:10.1007/s00464-010-0996-z.
- 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.