Myelolipomas are benign tumors composed of an admixture of matare adip
ose tissue and normal hematopoietic cells, The vast majority occur wit
hin the adrenal glands, but several extra-adrenal myelolipomas (EAMLs)
have been reported, The typical EAML is a solitary, well-defined mass
within the abdomen, most commonly in the retroperitoneal presacral ar
ea, EAMLs may produce symptoms related to their mass effect, but they
are occasionally incidental findings. Most commonly, the patient is ol
der than 40 years and has no hematologic abnormalities. It is importan
t to distinguish EAMLs from other soft tissue tumors, in particular li
posarcomas, myxoid malignant fibrous histiocytomas, and extramedullary
hematopoietic tumors. We discuss two cases of EAML, The first was in
the retroperitoneum of a 76-year-old woman. It is the largest EAML eve
r reported, measuring 26 cm x 15 cm x 11 cm, The second, a presacral m
ass in a 68-year-old man, was diagnosed preoperatively by percutaneous
computed tomography-guided fine needle aspiration biopsy, Preoperativ
e diagnosis facilitated surgical management in this case, We discuss t
he clinical, radiologic, and pathologic characteristics and the differ
ential diagnosis of this rare entity.