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]
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Overview
The prognosis of pheochromocytoma is generally good but metastatic pheochromocytoma has a 5-year survival rate of approximately 45%.[1] Massive release of catecholamines can causes damage to myocytes.
Natural History, Complication and Prognosis
Natural History
Complication
The massive release of catecholamines in pheochromocytoma can cause damage to myocytes.[2] This damage may be due to either a compromise of the coronary microcirculation or the direct toxic effects of catecholamines on myocytes.[2] Other complications may include:
- Hyperglycemia
- Refractory hypertension
- Malignant hypertension
- Metastasis to lymph nodes, bones, lungs, and liver
Prognosis
five-year survival rates that range widely from 12 to 84 percent [3,5-15]. Some of this variability has to do with differing definitions of malignancy.
Among malignant tumors, the survival rate may depend on the primary tumor site and sites of metastases. Outcomes are most variable for patients with malignant paragangliomas of the skull base and neck, most of which are nonsecretory. The following represents the range of findings:
●In one single institution series of 19 patients with malignant skull base and neck paraganglioma treated between 1970 and 2005, the five-year survival rate was 84 percent, despite the fact that 14 had distant metastases [10].
●On the other hand, in another report of 86 cases of malignant head and neck paraganglioma reported to the National Cancer Institute Surveillance, Epidemiology, and End Results (SEER) database from 1973 to 2009, the five-year survival rate was 65 percent overall; for those with regionally confined metastases (n = 47), it was 82 percent, compared with 41 percent for those with distant metastases (n = 39) [15]. Outcomes were more favorable for carotid body tumors than for malignant tumors at other sites (five-year survival 87 versus 48 percent).
●An earlier report of data on 59 cases of malignant paraganglioma of the skull base and neck extracted from the National Cancer Database demonstrated a five-year survival rate of 77 percent for regionally-confined metastases, but only 12 percent for patients with distant metastatic disease [3].
● patients with metastatic pheochromocytoma five-year survival rates are 34 to 60 percent, averaging approximately 50 percent [5-9,11-14,16]. Even 10-year survival rates of 25 percent are reported [16]. However, others report that outcomes are poorer with pheochromocytomas compared with paraganglioma, regardless of functionality [17]. In this study, pheochromocytomas presented more often with distant metastases and with larger tumors; overall survival at five years was 58 percent compared with 80 percent for paraganglioma.
Prognosis is impacted by tumor burden, location of metastases, and rate of progression;
patients with brain, liver, and lung metastases tend to have a worse prognosis than do those with isolated bone lesions [18].
- Approximately 10% recur after being resected.
References
- ↑ National Cancer Institute. Physician Data Query Database 2015. http://www.cancer.gov/types/pheochromocytoma/hp/pheochromocytoma-treatment-pdq#link/_25_toc
- ↑ 2.0 2.1 Goldman 2011, pp. 327