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Myelolipoma of the Adrenal Gland: Case Report and Review of the Literature

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Myelolipoma of the Adrenal Gland: Case Report and Review of the Literature Myelolipoma of the adrenal gland: Case report and review of the literature RICHARD A. SCHELLIN, D.O. Fort Worth, Texas dentally via CT, especially when scanning was Myelolipoma is a rare, benign, performed for an unrelated problem. 8 With the in- nonfunctioning neoplasm of the creased use of the newer high-resolution, thin-sec- adrenal gland and consists of fatty tion scanners, undoubtedly more asymptomatic, and hematopoietic tissue. It is usually benign adrenal lesions of even smaller size will be found only at autopsy; the present discovered. patient represents only the 33rd This report presents the 33rd reported clinical reported clinical case of the tumor. case of adrenal myelolipoma, as well as a review of The clinicoradiologic features of the literature regarding its clinicoradiologic diag- myelolipoma are described, and it is nosis and treatment. expected that with the wider application of newer computed Report of case tomographic scanners, more tumors A 40-year-old white woman was admitted for evaluation of this nature will be encountered in of global weakness, aphasia, vertigo, amnesia, and a clinical cases. Because it is usually an near syncopal episode. She had been experiencing ce- phalalgia for the past week and stated that that was ex- asymptomatic, incidentally found, tremely unusual for her. Her past medical history in- nonmalignant tumor, it is important cluded questionable hypoglycemia and two episodes of for the radiologist to include it in acute labyrinthitis, the most recent being 6 months pri- differential diagnosis, especially or to this admission. when computed tomography Physical examination revealed an obese woman with demonstrates a fat-containing tumor. a supine blood pressure of 140/80 mm. Hg. There was Criteria of benign versus malignant questionable muscle weakness of the left upper extrem- adrenal lesions are also presented. ity. Pale striae of the abdomen were also noted. No other Percutaneous biopsy is physical signs were identified. Usual admission labora- recommended when the diagnosis is tory test results were normal. Electroencephalographic doubtful. Surgery should be reserved and brain CT scanning findings also were normal. only for definite malignancy or During her hospitalization, the patient was noted to have labile hypertension, with the diastolic pressure ris- questionable biopsy cases. In ing to 110 mm. Hg. Intravenous pyelography demon- agreement with other authors, a strated normal renal function and a right suprarenal conservative management approach mass of low density measuring 6 cm. in diameter (Fig. in questionable cases, especially in 1). Some amorphous calcification was noted along the elderly patients who cannot tolerate medial margin of the mass, and the mass appeared to be surgery, is also presented. flattening the superior pole of the right kidney. Ultra- sonography was performed, and it revealed a 5 x 6-cm. right adrenal mass with an echodense pattern through- out. At its medial margin, hard echoes with associated shadowing indicated the presence of calcification. The overall pattern was consistent with a fatty tumor con- taining calcification (Fig. 2). Myelolipoma is a rare, nonfunctioning, benign tu- Adrenal CT showed a 6 x 6-cm. hypodense mass of the mor of the adrenal gland and consists of adipose right adrenal gland. The mass was homogeneous, and tissue and hematopoietic elements. 1 '2 Most of while no specific density numbers were obtained, it ap- these tumors are described at autopsy as inciden- peared slightly more dense than adjacent retroperito- tal findings. A small number have been discovered neal fat. Calcification along the medial margin was again clinically and resected. seen (Fig. 3). Some small (1 cm.) left para-aortic nodes were also noted. The appearance was thought to be most Computed tomography (CT) has become the di- consistent with adrenal carcinoma with necrosis or with agnostic modality of choice for imaging the adren- pheochromocytoma. A myelolipoma was not considered al glands." Adrenal masses have been found inci- in the differential diagnosis. Myelolipoma of the adrenal gland 26/63 neovascularity or tumor blush (Fig. 4). A simple right adrenalectomy was performed; it revealed a yellow to reddish-brown, homogeneous fatty tumor containing some hard foci of calcification. Microscopic examination showed a well-delineated but unencapsulated tumor with an adjacent rim of cortical tissue. The mass was composed of mature adipose tissue and hematopoietic elements. Foci of calcification and a focus of osseous met- aplasia were also noted. Final diagnosis was adrenal myelolipoma. The patients postoperative course was uneventful. She is being followed for her labile hyper- tension. Discussion Fig. 1. A tomographic cut of kidneys shows a low-density mass Patients ages at time of discovery range from 17 to above the right kidney, which causes some flattening of the supe- 93 years9; the majority of symptomatic cases oc- rior pole. Amorphous calcification is present within the mass at its medial aspect. curred between the fourth and seventh decades39- 12 No sex predilection was found by some authors,9" 11 while other investigators reported a 1.75:1 to 2:1 male to female predominance.212 Pathology Myelolipoma is usually a circumscribed tumor of the adrenal cortex or medulla. 19 The tumor is re- ported as an incidental autopsy finding in 0.2 to 0.8 percent of cases. 11,13-15 Size of the tumor varies from a few millimeters to 8 cm. in autopsy cases, 11 "6 while symptomatic resected tumors vary from 6 cm. and 50 gm. 11 to 30-34 cm. and 5,500-5,900 gm. 1,12 A myelolipoma is composed of mature fat and bone marrow tissue in varying amounts. Certain authors report the presence of a capsule, 13•14 while others describe no capsule. Most reports state that the tumor is well-circumscribed or encapsulated, but on microscopic evaluation they describel91315 a pseudocapsule of adjacent compressed adrenal cortical tissue, as was present in the reported case. In other areas of the tumor, some collagen layers are seen between the interface of the mass and normal cortical tissue. Some areas reveal fibrosis with fat necrosis and hemorrhage, thus indicating 1,15 Figs. 2A and 2B. Longitudinal ultrasonograms reveal echogenic infarction ; hemorrhage was usually found in mass between the right kidney and the liver. The echogenic pat- symptomatic tumors. 15 Calcification in areas of tern is the same as the retroperitoneal fat located posterior to the kidney. In Fig. 2B, note the two hard echoes at the anterior sur- hemorrhage was also described.1415 face of the mass, with apparent acoustic shadowing suggesting the presence of calcification within the mass. Etiology The cause of adrenal myelolipomas still is un- known. The following possibilities have been pro- posed: (1) embryonic rests of primitive mesenchym- al tissue; (2) emboli of bone marrow cells; and (3) Samples were obtained for serum catecholamine and urinary vanillylmandelic acid testing, and the patient metaplasia of adrenocortical cells in response to was allowed to go home. These endocrine determina- some still unknown stimulus3 9-15'17 The last the- tions were found to be normal, and she was readmitted 2 ory is the most widely supported. Multiple reports 2,10-13,17 weeks later for angiography and exploratory surgery. have suggested that the stimulus for the Angiography revealed a hypovascular, 6-cm. right ad- metaplasia may be associated with systemic stress renal mass with calcification, but without evidence of from chronic illnesses or necrotic tissue. 27/64 Jan. 1986/Journal of AOA/vol. 86/no. 1 Clinical features As earlier stated, most myelolipomas are asymp- tomatic and are incidental discoveries. Of the symptomatic cases reported, most patients were obese or were described as robust or well nour- ished. The majority also had high blood pres- sure.1,2,12 Our patient was obese and had labile hy- pertension. In fact, evaluation of her hypertension led to the discovery of the adrenal mass. The most common presenting complaint was abdominal pain; this is believed to be due to hemorrhage within the tumor. 15 Hematuria is another fairly common finding, but its association with myeloli- poma is unclear. Noble and associates suggest that the hypertension and hematuria are probably not related to the myelolipoma. In almost all cases, endocrinologic work-up has revealed no endocrine function of the tumor. Our case further supports this finding. Bennett and co- workers 7 reported a single case of functioning myelolipoma. His paper describes a large myeloli- poma that was found in a previous subtotal adren- alectomy site of Cushings disease. The patient had a recurrence of his cushingoid symptoms, and the myelolipoma was found to have collections of adrenocortical cells disbursed throughout rather than a rim of cortical tissue. Radiographic studies Plain radiography of the abdomen usually is not helpful in demonstrating myelolipoma. Sometimes Fig. 3A. Computed tomogram just superior to the kidneys reveal a radiolucent mass or a mottled appearing mass a smoothly marginated 6 x 4-cm. hypodense mass of the right adrenal gland. Calcification of the anteromedial margin of the can be seen in the areas of the kidney. s If the mass mass is present. The density of the mass is just slightly greater contains calcification, this also may be identified. than the adjacent retroperitoneal fat and is quite homogeneous These findings are not definitive, however. Usual- except for the calcified margin. Fig. 3B. A more caudal cut re- veals the relationship of the mass to the liver and the right kid- ly, nephrotomography will be helpful in screening ney. A fleck of calcium is seen along the anterolateral margin of for an adrenal tumor. A radiolucent or soft-tissue the adrenal mass. The density of the mass appears more hetero- mass along with calcification is better visualized geneous, but its center is still only slightly greater than fat.
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