Giant Bilateral Symptomatic Adrenal Myelolipomas Associated with Congenital Adrenal Hyperplasia

Abstract

Adrenal myelolipoma is an uncommon, benign, biochemically non-functioning and endocrinologically inactive tumor composed of variable amounts of mature adipose tissue and scattered islands of haemopoietic elements, including erythroid, myeloid and lymphoid series, as well as megakaryocytes. Diagnosis of myelolipomas is based on imaging, with ultrasonography, CT and MRI being effective in more than 90% of cases. Differential diagnosis includes other containing fat adrenal masses such as teratoma, lipoma and liposarcoma. The optimal treatment depends on the size and symptoms of the myelolipoma. For incidentally discovered, asymptomatic adrenal myelolipomas smaller than 4 cm surveillance seems to be enough while symptomatic, complicated, hormonally active and larger than 7 cm myelolipomas, should be surgically removed. We present a case of giant bilateral symptomatic adrenal myelolipomas associated with congenital adrenal hyperplasia. A 34 year old female, with congenital adrenal hyperplasia because of 21-hydroxylase deficiency, presented with diffuse abdominal pain and vomiting. Physical examination revealed hirsutism, pronounced virilization and palpable masses both on the right and left abdominal area. The abdominal CT demonstrated bilateral large masses in the anatomical position of the adrenal glands with densities indicating adipose tissue. The differential diagnosis was between myelolipoma and liposarcoma. For diagnostic and also therapeutical reasons, as the masses were large and symptomatic and causing pressure to the surrounding structures, the patient was submitted to laparotomy for bilateral excision. Histopathological examination established the diagnosis of adrenal myelolipoma.

Giant adrenal myelolipoma: when trauma and oncology collide

“…Adrenal myelolipoma has been associated with congenital adrenal hyperplasia, Cushing syndrome, hypertension, obesity, cholelithiasis, diabetes mellitus and haematological abnormalities in previous reports 4 5. However, the complete aetiology of myelolipoma remains unknown.…”

“…Current literature shows a possible correlation between congenital adrenal hyperplasia, Cushing syndrome, hypertension, obesity, cholelithiasis, diabetes mellitus and haematological abnormalities and the development of adrenal myelolipoma 4 5. We report three patients with the presentation of a giant myelolipoma after a high-energy trauma in the past.…”

Bilateral Adrenal Myelolipoma in a 46 XX DSD patient with Congenital Adrenal Hyperplasia due to 21-hydroxylase Deficiency. case report

“…However, they may attain very large sizes and also be bilateral (23,47). They are benign and usually asymptomatic tumors that usually need not to be resected (13,17,50,59).…”

“…Rarely, myelolipomas have been associated with hormone excess syndromes, such as CAH (16)(17)(18)(19)(20). Giant bilateral adrenal myelolipomas are rare (21)(22)(23)(24)(25)(26).…”

“…There are few reports of bilateral adrenal myelolipomas associated with adrenogenital syndrome caused by 21-OH deficiency made in the adult age in the course of evaluating bilateral large adrenal incidentalomas (17,18,20,23,(26)(27)(28)(32)(33)(34)(35)(36). On the other hand, to our knowledge there are two previus reports of the diagnosis of 46XX DSD patients with CAH due to 21 OHD assigned as male made in adult age in the course of evaluating large adrenal incidentalomas (17).…”