Giant adrenal myelolipoma with minimal fat content in a patient with homozygous beta‐thalassemia: Appearance on MRI

Abstract

Giant adrenal myelolipoma is an uncommon entity. We present the atypical MR imaging findings of a giant adrenal myelolipoma in a patient with homozygous beta-thalassemia with histopathology correlation. The tumor showed a drop in signal on the opposed-phase images, with no evidence of macroscopic fat contents, and demonstrated very high signal intensity on T2-weighted images. Giant adrenal myelolipoma should be considered in the differential diagnosis of tumors with the combination of the above MR imaging characteristics.

Adrenal glands in beta-thalassemia major: magnetic resonance (MR) imaging features and correlation with iron stores

“…On MR imaging, myelolipomas are shown as well-circumscribed masses of varying size, usually hyperintense on T1, due to fat [28]. They may occasionally appear heterogenous, if hematopoietic tissue predominates, with signal drop in the opposed-phase images and variable SI on T2-weighted images [28]. We did not notice any discrete adrenal mass among our series of 60 abdominal MR imaging studies reviewed in total, thus confirming the rarity of these entities among thalassemic patients.…”

“…Myelolipomas are rare, benign, encapsulated adrenal tumors containing mature adipose tissue interspread with hematopoieitic tissue and hemorrhagic components [27]. On MR imaging, myelolipomas are shown as well-circumscribed masses of varying size, usually hyperintense on T1, due to fat [28]. They may occasionally appear heterogenous, if hematopoietic tissue predominates, with signal drop in the opposed-phase images and variable SI on T2-weighted images [28].…”

“…Adrenal extramedullary hemopoiesis presents as unilateral or bilateral silent adrenal masses [24][25][26][27]. There have been also sporadic case reports of myelolipomas in thalassemic patients although there is no documented association between thalassemia and myelolipomas [28][29][30]. Myelolipomas are rare, benign, encapsulated adrenal tumors containing mature adipose tissue interspread with hematopoieitic tissue and hemorrhagic components [27].…”

“…Adrenal gland involvement in beta-thalassemia has been previously described both pathologically and in terms of adrenal function [2,3,[15][16][17][18][19][20][21][22][23]. Cases of myelolipomas and extramedullary hemopoiesis in the adrenal glands have been sporadically described on cross-sectional imaging studies of patients with beta-thalassemia [24][25][26][27][28][29][30]. However, the presence and incidence of hypointense, normal-sized adrenals has not been previously reported in the medical literature.…”

A giant adrenal myelolipoma in a beta‐thalassemia major patient: Does ineffective erythropoiesis play a role?

“…Myelolipoma is a rare tumor that accounts for approximately the 4% of adrenal tumors. Some cases are associated with chronic hemolytic anemias or ineffective erythropoiesis, including both transfusion dependent and transfusion independent thalassemia , sickle cell anemia , and hereditary spherocytosis . In a few cases the diagnosis of myelolipoma allowed the retrospective diagnosis of the underlying congenital anemia .…”