What determines mortality in malignant pheochromocytoma? : report of a case with eighteen-year survival and review of the literature
Saved in:
| Main Author: | |
|---|---|
| Publication Date: | 2018 |
| Other Authors: | , , , |
| Format: | Article |
| Language: | eng |
| Source: | Repositório Institucional da UnB |
| Download full: | http://repositorio.unb.br/handle/10482/33730 http://dx.doi.org/10.20945/2359-3997000000033 |
Summary: | Pheochromocytoma (PCC) is a tumor derived from adrenomedullary chromaffin cells. Prognosis of malignant PCC is generally poor due to local recurrence or metastasis. We aim to report a case of malignant PCC with 18-year survival and discuss which factors may be related to mortality and long-term survival in malignant pheochromocytoma. The patient, a 45-year-old man, reported sustained arterial hypertension with paroxysmal episodes of tachycardia, associated with head and neck burning sensation, and hand and foot tremors. Diagnosis of PCC was established biochemically and a tumor with infiltration of renal parenchyma was resected. No genetic mutation or copy number variations were identified in SDHB, SDHD, SDHC, MAX and VHL. Over 18 years, tumor progression was managed with 131I-MIBG (iodine-metaiodobenzylguanidine) and 177Lutetium-octreotate therapy. Currently, the patient is asymptomatic and presents sustained stable disease, despite the presence of lung, para-aortic lymph nodes and femoral metastases. Adequate response to treatment with control of tumor progression, absence of significant cardiovascular events and other neoplasms, and lack of mutations in the main predisposing genes reported so far may be factors possibly associated with the prolonged survival in this case. Early diagnosis and life-long follow-up in patients with malignant pheochromocytoma are known to be crucial in improving survival. |
Similar Items: What determines mortality in malignant pheochromocytoma? : report of a case with eighteen-year survival and review of the literature
- Dermoscopy findings in tinea capitis: case report and literature review
- Congenital central hypoventilation syndrome associated with Hirschsprung's disease : case report and literature review
- Aggressive ameloblastic fibrosarcoma in maxilla : case report and new perspectives based on the current literature
- Mortality in Brazil according to gender perspective, years 2000 and 2010
- Malignant paranasal sinus schwannoma
- 3-year survival rates of retained composite resin and ART sealants using two assessment criteria