Mielolipoma adrenal bilateral asociado a disfunción endocrina <span class="so-article-trans-title" dir="auto"> Translated title: Bilateral adrenal myelolipoma associated with an endocrine dysfunction </span>

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Abstract

Se presentó un caso de mielolipoma adrenal bilateral asociado a hiperplasia adrenal congénita por déficit de enzima 21 hidroxilasa en un joven de 27 años de edad, que abandonó tratamiento sustitutivo con acetato de cortisona a los 14 años. Estuvo asintomático hasta su ingreso en el hospital, al cual es remitido por dolor abdominal, vómitos y fiebre, constatándose tumoración abdominal gigante en hemiabdomen izquierdo. Las características clínicas, hormonales y radiológicas halladas son comentadas y correlacionadas con lo registrado en la literatura médica. Hasta donde se revisó, es el primer caso de mielolipoma adrenal bilateral asociado a disfunción endocrina que se publica en Cuba.

Translated abstract

Authors present a case of bilateral adrenal myelolipoma associated with a congenital adrenal hyperplasia by deficit of hydroxilase enzyme 21 in a young aged 27 who leaves the substitution treatment with cortisone acetate at 14 years old. He was asymptomatic until its hospital admission due to abdominal pain, vomiting and fever and a high abdominal tumor in left hemi-abdomen. Clinical hormonal and radiological features founded are discussed and correlated with those registered in medical literature. As far as we know, this is the first case of bilateral adrenal myelolipoma associated with an endocrine dysfunction published in Cuba.

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Adrenal incidentaloma and patients with homozygous or heterozygous congenital adrenal hyperplasia.

S Jaresch, E Kornely, H Kley … (1992)

Adrenal tumors are being detected more frequently in consequence of the wider application of increasingly sensitive radiological investigation techniques. According to the working hypothesis that more silent adenomas could develop from hyperplastic tissue areas under increased stimulation of the adrenal cortex, heterozygous and homozygous patients with congenital adrenal hyperplasia (CAH) were studied. A high incidence of adrenal masses, nearly 82% in homozygous and 45% in heterozygous patients, was found. There was no correlation between tumor size and serum 17-hydroxyprogesterone concentrations. These tumors are, therefore, probably silent adenomas. On the basis of these results, CAH should always be ruled out in the case of incidentally detected adrenal masses. Since CAH is a relatively frequent disease, and the adrenal carcinoma belongs to the rarest malignant tumors, a malignant transformation of these tumors seems to be unlikely.