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• W4250571551 abstract "Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis. The clinical experience acquired, even from a small number of cases, has improved understanding of this condition. The purpose of this study is to characterize patients with ACC followed up at a Portuguese reference center over the past 22 years. Retrospective analysis of clinical records of patients with histopathological diagnosis of ACC followed up from 1992 to 2014. The study sample consisted of 22 patients, 20 of them female. Eleven patients were in stage II, four in stage III, and five in stage IV; 13 patients had functioning lesions. Adrenalectomy was performed in 20 patients, with complete tumor resection in 90% of the cases. During follow-up, eight patients experienced recurrence of local disease, and 12 distant metastases. Fourteen patients received mitotane, 35.7% (n = 5) as adjuvant therapy and 64.3% (n = 9) after recurrence; therapeutic plasma mitotane levels were achieved in 70% of patients. Stage III patients who received adjuvant therapy had longer survival time (13.5 vs. 2.5 months). Two patients were given chemotherapy associated to mitotane. Median survival was 11 months (0–257 months); it was slightly longer in younger patients or patients with non-functioning tumors. Six patients are still alive, four of them with no evidence of disease. Despite the overall poor prognosis, some patients with ACC may have a long survival time. Although complete tumor removal remains the only potentially curative treatment, diagnosis at a younger age, presence of non-functioning tumors, and mitotane treatment also seemed to be associated to longer survival in our patients. El carcinoma de la corteza suprarrenal (ACC) es una enfermedad rara, con mal pronóstico. La experiencia clínica, incluso cuando se obtiene a partir de un número limitado de casos, ha mejorado el conocimiento sobre esta entidad. Este estudio tiene como objetivo caracterizar los pacientes con ACC seguidos en un centro de referencia portugués durante los últimos 22 años. Análisis retrospectivo de la historia clínica de los pacientes con diagnóstico histopatológico de ACC seguidos entre 1992 y 2014. Se incluyeron 22 pacientes, 20 de ellos mujeres. Once pacientes se encontraban en estadio ii, 4 en estadio iii y 5 en estadio iv, mientras que 13 pacientes mostraron lesiones funcionales. La adrenalectomía se realizó en 20 pacientes, con resección completa del tumor en el 90% de los casos. Durante el seguimiento 8 pacientes presentaron recurrencia de la enfermedad local y 12 metástasis a distancia. Catorce pacientes recibieron tratamiento con mitotano: adyuvante en el 35,7% (n = 5) o después de la recidiva de 64,3% (n = 9), alcanzándose niveles plasmáticos terapéuticos en el 70% de los casos tratados. Los pacientes en estadio iii que recibieron tratamiento adyuvante presentaron mayor supervivencia (13,5 frente a 2,5 meses). Dos pacientes fueron sometidos a quimioterapia asociada con mitotano. La mediana de supervivencia fue de 11 meses (0-257 meses), y resultó ligeramente mayor en los pacientes más jóvenes o con tumores no funcionantes. Seis pacientes continúan vivos, 4 de ellos sin evidencia de enfermedad. A pesar del mal pronóstico global, algunos pacientes con ACC pueden presentar una larga supervivencia. Aunque la eliminación completa del tumor sigue siendo el único tratamiento potencialmente curativo, en nuestros pacientes el diagnóstico a una edad más joven, la presencia de tumores no funcionantes y el tratamiento con mitotano también parecen asociarse con una mayor supervivencia." @default.
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• W4250571551 date "2016-05-01" @default.
• W4250571551 modified "2023-09-28" @default.
• W4250571551 title "Adrenocortical carcinoma: Retrospective analysis of the last 22 years" @default.
• W4250571551 doi "https://doi.org/10.1016/j.endoen.2016.05.005" @default.
• W4250571551 hasPublicationYear "2016" @default.
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