Myelolipomatous adrenal masses causing Cushing's syndrome.
ABSTRACT Adrenal myelolipomas are uncommon benign tumors, composed of mature adipose tissue and haematopoietic elements in varying proportions. They are usually asymptomatic, non-functioning adrenal incidentalomas, but there have been a few reports of myelolipomatous masses associated with adrenocortical hypersecretion. We report two cases of large mixed adrenal tumors, with heterogeneous appearance and areas of fat density in imaging techniques, and with autonomous cortisol production leading to Cushing's syndrome. Both underwent adrenalectomy and the histological study showed an adrenocortical adenoma with widespread myelolipomatous metaplasia. Hypercortisolism resolved in the one patient that could be evaluated after surgery. We review all the previous reported cases of hypercortisolism associated with adrenal myelolipomas. We also discuss the recommended diagnostic approach and therapeutic management of adrenal masses of lipomatous appearance.
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ABSTRACT: The objective of this study was to describe a case of giant myelolipoma associated with undiagnosed congenital adrenal hyperplasia (CAH) due to 21-hydroxylase (21OH) deficiency. Five seven year-old male patient referred with abdominal ultrasound revealing a left adrenal mass. Biochemical investigation revealed hyperandrogenism and imaging exams characterized a large heterogeneous left adrenal mass with interweaving free fat tissue, compatible with the diagnosis of myelolipoma, and a 1.5 cm nodule in the right adrenal gland. Biochemical correlation has brought concerns about differential diagnosis with adrenocortical carcinoma, and surgical excision of the left adrenal mass was indicated. Anatomopathologic findings revealed a myelolipoma and multinodular hyperplasic adrenocortex. Further investigation resulted in the diagnosis of CAH due to 21OH deficiency. Concluded that CAH has been shown to be associated with adrenocortical tumors. Although rare, myelolipoma associated with CAH should be included in the differential diagnosis of adrenal gland masses. Moreover, CAH should always be ruled out in incidentally detected adrenal masses to avoid unnecessary surgical procedures.Arquivos brasileiros de endocrinologia e metabologia 06/2010; 54(4):419-24. DOI:10.1590/S0004-27302010000400012 · 0.68 Impact Factor
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ABSTRACT: Adrenal myelolipoma is a rare, benign neoplasm that is usually asymptomatic, unilateral and nonsecreting. It develops within the adrenal gland and is composed of mature adipose tissue with elements of the hematopoietic series. We describe the case of what is, to the best of our knowledge, one of the largest secreting adrenal myelolipomas reported in the literature. A 52-year-old Caucasian man of medium build who had had moderate hypertension for three years presented to our hospital. He had no other significant symptoms. His hypertension was pharmacologically treated. He came to our hospital to undergo abdominal ultrasonography during a clinical checkup. The ultrasound scan showed the presence of a voluminous hyperechoic mass interposed between the spleen and the left kidney. It was reported as a myelolipoma of the left kidney on the basis of its structural characteristics and position. Computed tomography confirmed our diagnosis. All preoperative biochemical tests were normal, with the exception of high serum cortisol, which was being overproduced by the lesion and was probably responsible for the patient's hypertension. He underwent successful surgery, and his postoperative course was uneventful. The pathologic examination of the lesion confirmed the diagnosis of adrenal myelolipoma. The patient's blood pressure returned to within the normal range. The "incidental" discovery of an adrenal mass requires careful diagnostic study to plan adequate therapeutic management. Both of the primary investigations at our disposal, ultrasound and blood tests (adrenal hormones), helped in rendering the diagnosis and allowed us to move toward the most appropriate treatment, taking into account the size of the tumor and its probable hormonal production.Journal of Medical Case Reports 07/2011; 5:298. DOI:10.1186/1752-1947-5-298
- European geriatric medicine 04/2011; 2(2):106-107. DOI:10.1016/j.eurger.2011.01.011 · 0.55 Impact Factor