Inédita coincidencia: feocromocitoma sobre glándula suprarrenal atrófica por enfermedad de Addison / An unusual association: pheochromocytoma on an atrophied adrenal gland due to Addison´s disease

El síndrome pluriglandular autoinmunitario (SPGA) tipo II es el síndrome inmunoendocrinopatológico más frecuente. Se define por la aparición de2 o más de las siguientes entidades: insuficiencia suparrenal primaria(enfermedad de Addison), enfermedad de Graves, diabetes mellitus tipo1A, tiroiditis autoinmunitaria, hipogonadismo primario, enfermedad celíaca o miastenia grave. Asimismo, es frecuente que aparezcan también vitíligo, alopecia, anemia perniciosa y/o serositis. La insuficiencia suprarrenal primaria con la que cursan estos pacientes afecta a la corteza adrenal, y ésta es destruida por los auto anticuerpos contra la 21-hidroxilasa. A diferencia de las demás etiologías de la insuficiencia suprarrenal (enfermedades infecciosas, enfermedades infiltrativas, hemorragia, enfermedades tumorales), respeta la médula adrenal. Los feocromocitomas son tumores derivados de las células cromafines del sistema nervioso simpático situadas en la médula adrenal. Pueden cursar con manifestaciones clínicas muy variadas, desde una hipertensión arterial (HTA) aislada o acompañada de episodios paroxísticos, que incluyen la clásica tríada de cefalea, palpitaciones y diaforesis, hasta cuadros potencialmente graves, como edema agudo de pulmón, arritmias o muerte súbita. No hay que olvidar que hasta el 40% son asintomáticos. A continuación, presentamos el caso de una paciente diagnosticada de SPGA tipo II, que desarrolla un feocromocitoma. En esta ocasión, en una glándula adrenal cortical atrofiada se desarrolla un tumor dependiente de la médula adrenal. Esta coexistencia de endocrinopatías, sin conexión etiológica alguna, no deja de ser cuando menos un hallazgo sorprendente, no descrito hasta el momento en la literatura actual (AU)

Autoimmune polyendocrine syndrometype II (APS-II) is the most commonimmunoendocrinopathy syndrome. APS-IIis defined by the development of two or more of the following entities: primaryadrenal insufficiency (Addison’s disease),Graves’ disease, type 1A diabetes mellitus,autoimmune thyroiditis, primaryhypogonadism, celiac disease, and myasthenia gravis. Other frequent clinical findings are vitiligo, alopecia, pernicious anemia and/or serositis. Primary adrenal insufficiency in these patients affects the adrenal cortex, which is destroyed byautoantibodies against 21-hydroxylase.Unlike other causes of adrenal insufficiency (infectious diseases, infiltrative diseases, bleeding, tumors), the adrenal medulla is not involved. Pheochromocytomas are tumors arising from the chromaffin cells of the sympathetic nervous system in the adrenal medulla. The clinical symptoms of these tumors vary from isolated hypertension or hypertension accompanied by paroxysmal episodes —including the classical triad of headache, palpitations and diaphoresis—to potentially serious manifestations such as acute pulmonary edema, arrhythmias and sudden death. Nevertheless, up to 40%of affected patients are asymptomatic. We present the case of a patient diagnosed with APS-II who developed apheochromocytoma. In this patient, the adrenal gland cortex was atrophied andthe tumor was attached to the adrenal medulla. This coexistence ofendocrinopathies, with no etiologic connection, is a surprising finding, which has not previously been described in the current literatura (AU)

An unusual association: pheochromocytoma on an atrophied adrenal gland due to addison's disease.

Autoimmune polyendocrine syndrome type II (APS-II) is the most common immunoendocrinopathy syndrome. APS-II is defined by the development of two or more of the following entities: primary adrenal insufficiency (Addison's disease), Graves' disease, type 1A diabetes mellitus, autoimmune thyroiditis, primary hypogonadism, celiac disease, and myasthenia gravis. Other frequent clinical findings are vitiligo, alopecia, pernicious anemia and/or serositis. Primary adrenal insufficiency in these patients affects the adrenal cortex, which is destroyed by autoantibodies against 21-hydroxylase. Unlike other causes of adrenal insufficiency (infectious diseases, infiltrative diseases, bleeding, tumors), the adrenal medulla is not involved. Pheochromocytomas are tumors arising from the chromaffin cells of the sympathetic nervous system in the adrenal medulla. The clinical symptoms of these tumors vary from isolated hypertension or hypertension accompanied by paroxysmal episodes -including the classical triad of headache, palpitations and diaphoresis-to potentially serious manifestations such as acute pulmonary edema, arrhythmias and sudden death. Nevertheless, up to 40% of affected patients are asymptomatic. We present the case of a patient diagnosed with APS-II who developed a pheochromocytoma. In this patient, the adrenal gland cortex was atrophied and the tumor was attached to the adrenal medulla. This coexistence of endocrinopathies, with no etiologic connection, is a surprising finding, which has not previously been described in the current literature.