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What determines mortality in malignant pheochromocytoma? - Report of a case with eighteen-year survival and review of the literature
Andrade, Matheus de Oliveira; Cunha, Vinícius Santos da; Oliveira, Dayana Carla de; Moraes, Olívia Laquis de; Lofrano-Porto, Adriana.
Afiliación
  • Andrade, Matheus de Oliveira; Universidade de Brasília. Faculdade de Ciências da Saúde. Laboratório de Farmacologia Molecular. Brasília. BR
  • Cunha, Vinícius Santos da; Universidade de Brasília. Faculdade de Ciências da Saúde. Laboratório de Farmacologia Molecular. Brasília. BR
  • Oliveira, Dayana Carla de; Universidade de Brasília. Faculdade de Ciências da Saúde. Laboratório de Farmacologia Molecular. Brasília. BR
  • Moraes, Olívia Laquis de; Universidade de Brasília. Faculdade de Ciências da Saúde. Laboratório de Farmacologia Molecular. Brasília. BR
  • Lofrano-Porto, Adriana; Universidade de Brasília. Faculdade de Ciências da Saúde. Laboratório de Farmacologia Molecular. Brasília. BR
Arch. endocrinol. metab. (Online) ; 62(2): 264-269, Mar.-Apr. 2018. tab, graf
Article en En | LILACS | ID: biblio-887643
Biblioteca responsable: BR1.1
ABSTRACT
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.
Asunto(s)

Texto completo: 1 Colección: 01-internacional Base de datos: LILACS Asunto principal: Feocromocitoma / Neoplasias de las Glándulas Suprarrenales Tipo de estudio: Prognostic_studies / Screening_studies Límite: Humans / Male Idioma: En Revista: Arch. endocrinol. metab. (Online) Asunto de la revista: ENDOCRINOLOGIA / METABOLISMO Año: 2018 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Brasil

Texto completo: 1 Colección: 01-internacional Base de datos: LILACS Asunto principal: Feocromocitoma / Neoplasias de las Glándulas Suprarrenales Tipo de estudio: Prognostic_studies / Screening_studies Límite: Humans / Male Idioma: En Revista: Arch. endocrinol. metab. (Online) Asunto de la revista: ENDOCRINOLOGIA / METABOLISMO Año: 2018 Tipo del documento: Article País de afiliación: Brasil Pais de publicación: Brasil