eMedicine Specialties > Radiology > Genitourinary
Adrenal Myelolipoma
Updated: Feb 15, 2007
Introduction
Background
Adrenal myelolipoma is a rare benign neoplasm composed of mature adipose tissue and a variable amount of hematopoietic elements. Most lesions are small and asymptomatic, discovered incidentally at autopsy or on imaging studies performed for other reasons. Most tumors are unilateral but show no predilection to one particular side. Tumor size varies from several millimeters to more than 30 cm.
Pathophysiology
Several explanations of this neoplasm's development have been offered. One theory by D. C. Collins suggests that a myelolipoma represents a site of extramedullary hematopoiesis. The most widely accepted theory, as cited by Meaglia and Schmidt in a 1992 study of the natural history of adrenal myelolipoma, is the existence of metaplasia of the reticuloendothelial cells of blood capillaries in the adrenal gland in response to stimuli such as necrosis, infection, or stress.
Frequency
United States
Incidence varies from 0.08-0.4% at autopsy.
Mortality/Morbidity
No death rate has been reported in the literature because of the rarity of this lesion. Myelolipomas do not undergo malignant transformation.
The primary complication, which is uncommon, is spontaneous rupture of the mass, resulting in retroperitoneal hemorrhage. Rupture and hemorrhage also can occur following blunt trauma.
Race
As reported by Han et al in 1997 in a series of 20 patients, 85% of patients with adrenal myelolipoma were white. No other generalizations have been reported in the literature.
Sex
Mostly, the male-to-female ratio is 1:1; however, a study conducted by Han et al in 1997 reported a male-to-female ratio of 2:3.
Age
Lesions most commonly occur in patients in their fifth to seventh decades, although they have been seen in patients aged 12-93 years.
Anatomy
Most myelolipoma lesions occur within the adrenal gland. Rarely, myelolipomas can exist in extraadrenal locations, such as the presacral retroperitoneum, perirenal and pararenal retroperitoneum, mediastinum, liver, and muscle fascia. Kammen et al reported a rare case of an extra-adrenal myelolipoma that presented as a fat-containing retroperitoneal mass not contiguous with the adrenal gland.
Presentation
Most myelolipomas are asymptomatic and hormonally nonfunctional. Occasionally, patients present with nonspecific abdominal or flank pain secondary to intratumoral or peritumoral hemorrhage, tumor necrosis, or mechanical compression from tumor bulk. Other rare presenting symptoms include hematuria and abdominal mass.
In a review of the literature, Hisamatsu et al documented 25 cases of endocrine dysfunction associated with adrenal myelolipomas, including conditions such as Cushing syndrome, Conn syndrome, and congenital adrenal hyperplasia. In 85% of these patients, the abnormality involves the pituitary adrenal axis. In patients with associated endocrine dysfunction, stimulation with cortisol or adrenocorticotropin hormone has been suggested to be involved in the pathogenesis of myelolipomas.
Patients with small asymptomatic myelolipomas are monitored clinically for symptoms, and routine follow-up radiologic tests appear unnecessary for such lesions. Symptomatic tumors are treated by adrenalectomy. Transcatheter embolization prior to surgical resection has been used successfully to achieve hemostasis in cases of ruptured myelolipomas leading to retroperitoneal hemorrhage. Occasionally, large silent tumors are excised to prevent the occurrence of rupture.
Preferred Examination
The fatty component of a myelolipoma is macroscopic in most patients and is diagnostic when discovered on cross-sectional imaging.
The preferred imaging modality is CT, which shows focal fatty density within the mass. MRI also accurately depicts both microscopic and macroscopic fat using chemical shift imaging and explicit fat saturation technique, respectively. Myelolipomas may be discovered incidentally on ultrasound (US), which otherwise is not used routinely to characterize adrenal neoplasms.
Limitations of Techniques
Occasionally, myelolipomas can enlarge enough to make the organ of origin difficult to discern on CT, resulting in a differential diagnosis that includes renal angiomyolipoma and retroperitoneal liposarcoma or lipoma. In these patients, the multiplanar capability of MRI can help define the tissue planes and confirm that the mass is adrenal in origin.
Some myelolipomas may have a larger amount of hematopoietic tissue and no recognizable fat, making them impossible to distinguish from well-differentiated retroperitoneal malignancies or other adrenal tumors on CT or MRI. Percutaneous biopsy may be necessary to establish a diagnosis.
Differential Diagnoses
Adrenal Adenoma
Adrenal Carcinoma
Angiomyolipoma, Kidney
Liposarcoma, Soft Tissue
Radiography
Findings
If large enough, a suprarenal mass may cause inferior displacement of the kidney. The mass may be radiolucent if it is predominantly fatty and, occasionally, can contain calcifications.
Degree of Confidence
Plain radiographs are nonspecific and not part of the imaging workup of these lesions. Perform cross-sectional imaging using CT or MRI to document the presence of fat.
False Positives/Negatives
Smaller myelolipomas are unlikely to exert enough mass effect to be visible on conventional radiographs, thereby constituting false-negative results. Similarly, inferior displacement of the kidney on radiographs can be a result of other adrenal or extra-adrenal lesions.
Computed Tomography
Findings
CT appearance of myelolipomas depends on their histologic composition. Large amounts of fat often are seen interspersed with higher-attenuation myeloid tissue. The attenuation values are low (-20 to -30 Hounsfield units [HU]), reflecting the mixture of adipose and myeloid elements. The density of the mass may be slightly higher than that of the adjacent retroperitoneal fat secondary to the presence of the myeloid tissue.
The masses usually have a recognizable capsule and may contain calcification in as many as 20% of patients. Calcification may be related to previous hemorrhage. After contrast administration, the mass enhances. If intratumoral or peritumoral hemorrhage has occurred, high-attenuation or low-attenuation fluid may be present, depending on the age of the blood.
Degree of Confidence
CT is sensitive in depicting macroscopic fat in myelolipomas. The key to a CT diagnosis is to find a true focal fat collection in the adrenal mass. This modality is specific and diagnostic if the focal fat density is revealed.
CT can be problematic if the mass is large and the organ of origin is not obvious using axial imaging. MRI may be warranted for further evaluation as a result of its multiplanar capability. Additionally, if little or no macroscopic fat is present, the lesion has soft tissue attenuation.
False Positives/Negatives
Adrenal adenomas may have low-attenuation values because of a large amount of intracellular lipid, but the density usually is not less than –20 HU. In almost all myelolipomas, some regions have densities of less than –30 HU and as low as –100 HU. If the mass contains more hematopoietic tissue, it may appear more heterogeneous and mimic a retroperitoneal liposarcoma. A definitive diagnosis requires percutaneous-guided cytologic or histologic sampling.
Magnetic Resonance Imaging
Findings
The fat in an adrenal myelolipoma is of high signal intensity on T1- and T2-weighted sequences and is key to diagnosis. The hematopoietic tissue has low signal intensity on T1-weighted images and moderate signal intensity on T2-weighted images. The nonuniform admixture of fat and marrow elements also may result in a heterogeneous appearance on T2-weighted sequences.
Explicit (chemical) fat-suppressed T1-weighted sequences show a focal loss of signal intensity in the fatty part of the mass, which confirms the diagnosis. The presence of myeloid tissue or hemorrhage results in persistent areas of increased signal intensity on fat-suppressed images. A water-saturated T1-weighted sequence shows the fat as high signal intensity on a background of low signal intensity.
Chemical shift gradient-echo imaging depicts microscopic (intracellular) fat that is contained in the same voxel as a water proton. Two scans are obtained through the mass using parameters that are identical except for the echo time (TE), which is selected so that the fat and water protons are either in phase or out of phase. Out-of-phase images show signal loss, since the fat and water protons in the same voxel cancel each other out. This decrease in signal intensity indicates the presence of microscopic fat. Adrenal myelolipomas enhance after gadolinium administration as a result of the presence of hematopoietic tissue.
Gadolinium-based contrast agents (gadopentetate dimeglumine [Magnevist], gadobenate dimeglumine [MultiHance], gadodiamide [Omniscan], gadoversetamide [OptiMARK], gadoteridol [ProHance]) have recently been linked to the development of nephrogenic systemic fibrosis (NSF) or nephrogenic fibrosing dermopathy (NFD). For more information, see the eMedicine topic Nephrogenic Fibrosing Dermopathy. The disease has occurred in patients with moderate to end-stage renal disease after being given a gadolinium-based contrast agent to enhance MRI or MRA scans.
As of late December 2006, the FDA had received reports of 90 such cases of NSF/NFD . Worldwide, over 200 cases have been reported, according to the FDA. NSF/NFD is a debilitating and sometimes fatal disease. Characteristics include red or dark patches on the skin; burning, itching, swelling, hardening, and tightening of the skin; yellow spots on the whites of the eyes; joint stiffness with trouble moving or straightening the arms, hands, legs, or feet; pain deep in the hip bones or ribs; and muscle weakness. For more information, see the FDA Public Health Advisory or Medscape.
Elsayes et al described 3 categories of myelolipomas based on their MRI features. These categories are (1) homogeneous, hyperintense masses on T1-weighted imaging indicating that they are composed mainly of fat; (2) heterogeneous masses composed of an admixture of fatty and myeloid elements; and (3) focal enhancing masslike areas composed mainly of myeloid cells.
Degree of Confidence
MRI is as sensitive and specific as CT in depicting macroscopic fat and confirming the diagnosis. In evaluating an adrenal myelolipoma, explicit (chemically selective) fat saturation sequences show more signal loss than chemical shift imaging, since the presence of macroscopic fat is typical of a myelolipoma. In contrast, a lipid-rich adrenal adenoma would reveal greater loss of signal intensity on chemical shift imaging as it contains intracellular (microscopic) fat. An added advantage of MRI is the ability to image directly in the coronal and sagittal planes, confirming that the origin of a large mass is adrenal and not hepatic or renal.
False Positives/Negatives
Lesions, such as adrenal adenomas, metastases, and primary cortical carcinomas, rarely have been reported to contain focal fat and can be misdiagnosed as myelolipoma. However, these lesions are rare enough that the detection of macroscopic fat should not prevent the diagnosis in almost all patients. Conversely, some adrenal myelolipomas (eg, giant myelolipomas) have been shown to contain minimal fat, resulting in difficulty in making a definitive diagnosis by MRI. As with CT, markedly heterogeneous masses may not be distinguished easily from well-differentiated retroperitoneal malignancies, such as liposarcoma, and require tissue sampling for definitive diagnosis.
Ultrasonography
Findings
Sonographic appearance varies with the composition of the neoplasm. If the tumor contains predominantly fatty components, it appears uniformly hyperechoic. If the tumor contains a predominance of myeloid cells, it may appear heterogeneous or hypoechoic.
The classic appearance is an echogenic suprarenal mass, which suggests the diagnosis. The ipsilateral hemidiaphragm artifactually appears as a disrupted line, a finding unique to fat-containing masses. The margins of the lesion are difficult to define because of the lack of contrast with the adjacent retroperitoneal fat.
Degree of Confidence
Although a predominantly hyperechoic adrenal mass strongly suggests the diagnosis of myelolipoma, US is not as specific as CT or MRI, either of which should be obtained for further evaluation. US is more nonspecific if the tumor contains only a small quantity of fat and appears hypoechoic, thus mimicking other benign or malignant adrenal neoplasms.
False Positives/Negatives
Small lesions are more difficult to diagnose by US, since they may be masked by adjacent echogenic retroperitoneal fat. The finding of apparent disruption of the diaphragmatic echoes is appreciated only for tumors larger than 4 cm; however, the absence of this finding does not mean that a hyperechoic mass does not contain fat.
Lesions containing a predominance of myeloid cells appear more hypoechoic and mimic an adenoma, hematoma, metastasis, or primary cortical carcinoma. The presence of hemorrhage or calcification within the tumor alters the sonographic appearance and further confounds the issue.
Angiography
Findings
As a result of vascular specificity, an angiogram can confirm that a mass is adrenal in origin rather than arising from the liver or kidney. The mass is predominantly avascular, with a peripheral rim of vascularity from branching vessels.
Degree of Confidence
The procedure is fairly specific in determining the organ of mass's origin but is otherwise nonspecific regarding its tissue characteristic. With the advent of cross-sectional imaging modalities, little indication exists for routine angiography.
False Positives/Negatives
Other adrenal masses, such as adenomas, lipomas, cysts, and metastases, can have a similar angiographic appearance.
Intervention
Since myelolipomas contain different proportions of fat and myeloid tissue, a definitive diagnosis using CT or MRI may be difficult, although rarely so, if only a small amount of fat is present.
To distinguish the mass from a well-differentiated liposarcoma, a percutaneous fine needle aspiration via US or CT guidance can confirm the diagnosis. The presence of mature adipose tissue intermixed with hematopoietic elements, including megakaryocytes on cytology or histology, is diagnostic of myelolipoma.
Multimedia
Media file 1: Intravenous urogram performed in a 64-year-old man reveals inferior displacement of the left kidney by a left suprarenal mass containing a large focus of calcification (indicated by *). Courtesy of P. Ramchandani, MD.
Media file 2: Contrast-enhanced CT in a 64-year-old man (same patient as in Image 1) confirms the presence of chunky calcification and a large amount of macroscopic fat in the left adrenal mass, which is diagnostic of a myelolipoma. Courtesy of P. Ramchandani, MD.
Media file 3: Intravenous urogram performed in a 63-year-old man reveals a round left suprarenal mass (indicated by *) without mass effect on the left kidney. Courtesy of P. Ramchandani, MD.
Media file 4: Contrast-enhanced CT in a 63-year-old man (same patient as in Image 3) reveals several scattered foci of macroscopic fat in the left adrenal mass, confirming a diagnosis of myelolipoma. Courtesy of P. Ramchandani, MD.
Media file 5: Contrast-enhanced CT in a 72-year-old woman reveals a 6-cm right adrenal mass containing large amounts of macroscopic fat, consistent with an adrenal myelolipoma. The attenuation of the fat is slightly higher than that of the adjacent retroperitoneal fat, likely as a result of the presence of hematopoietic elements in the mass. Courtesy of P. Ramchandani, MD.
Media file 6: Unenhanced CT in an asymptomatic man reveals a 6-cm right adrenal mass with density measurements that range from -14 to -27 Hounsfield units, consistent with myelolipoma. Courtesy of P. Ramchandani, MD.
Media file 7: Contrast-enhanced CT reveals a left adrenal mass with a large deposit of macroscopic fat, measuring -97 Hounsfield units, diagnostic of adrenal myelolipoma.
Media file 8: A more inferior axial image of the patient in Image 7 shows areas of soft tissue density and discrete foci of macroscopic fat, measuring approximately -90 Hounsfield units. Courtesy of P. Ramchandani, MD.
Media file 9: A 70-year-old asymptomatic man with a left adrenal myelolipoma noted incidentally. Axial T1-weighted in-phase gradient-echo image reveals a left adrenal soft tissue mass with 2 small foci of high signal intensity in the posterior aspect of the mass, which can represent hemorrhage or fat. Courtesy of S. Adusumilli, MD.
Media file 10: Axial T1-weighted out-of-phase gradient-echo image at the same level as in Image 9. Overall, the mass demonstrates slight loss of signal intensity with respect to the spleen compared to the in-phase image in Image 9, reflecting the presence of intracellular lipid. The 2 foci of high signal intensity in the posterior aspect of the mass do not lose signal, since they represent macroscopic fat (confirmed on the sequences in Images 11 and 12). Courtesy of S. Adusumilli, MD.
Media file 11: Axial T1-weighted out-of-phase gradient-echo image with explicit fat saturation at the same level as Images 9 and 10. The 2 foci of high signal intensity in the posterior aspect of the mass have completely lost signal because of the fat saturation technique, thus represent macroscopic fat. This is in contrast to the intracellular or microscopic fat demonstrated in the rest of the mass in Images 9 and 10. The loss of signal intensity also proves that these foci do not represent hemorrhage, which remains high in signal intensity. Courtesy of S. Adusumilli, MD.
Media file 12: Axial fat-saturated T2-weighted fast spin-echo image at the same level as Images 9-11. The left adrenal mass is minimally heterogeneous as a result of the presence of hematopoietic tissue. The 2 foci of dark signal intensity in the posterior aspect of the mass represent macroscopic fat, since an explicit fat saturation technique was used. Courtesy of S. Adusumilli, MD.
Media file 13: An 87-year-old woman with a left adrenal myelolipoma. Axial T1-weighted in-phase gradient-echo image reveals a heterogeneous left adrenal mass with a discrete area of high signal intensity in the anterior medial aspect of the mass, which can represent fat or hemorrhage. Courtesy of S. Adusumilli, MD.
Media file 14: Axial T1-weighted out-of-phase gradient-echo image at the same level as Image 13 shows that most of the mass loses signal intensity with respect to the spleen, which is consistent with intracellular or microscopic lipid. A persistent area of high signal intensity is seen in the anterior medial aspect of the mass, which can represent either macroscopic fat or hemorrhage. Courtesy of S. Adusumilli, MD.
Media file 15: Axial T1-weighted in-phase gradient-echo image with water saturation at the same level as Images 13 and 14. This water saturation sequence detects macroscopic fat that remains high in signal intensity, while the rest of the abdominal structures lose signal intensity, since they contain water protons. A part of the left adrenal mass loses signal intensity as a result of the presence of hematopoietic tissue; however, a persistent focus of high signal intensity is seen in the anterior medial aspect of the mass, which represents macroscopic fat. Courtesy of S. Adusumilli, MD.
Media file 16: Axial T1-weighted gradient-echo image with explicit fat saturation at the same level as Images 13-15 demonstrates that the entire mass loses signal intensity, which is reflective of the presence of macroscopic fat throughout the mass. Courtesy of S. Adusumilli, MD.
Media file 17: Axial fat-saturated T2-weighted fast spin-echo image at approximately the same level as Images 13-16 shows areas of brighter signal intensity in the mass which reflects the presence of hematopoietic tissue. Courtesy of S. Adusumilli, MD.
Media file 18: Coronal fat-saturated gadolinium-enhanced T1-weighted gradient-echo image of the patient in Images 13-17 shows that the left adrenal myelolipoma is avascular, as reflected by its lack of enhancement. Courtesy of S. Adusumilli, MD.
Media file 19: Axial fat-saturated gadolinium-enhanced T1-weighted gradient-echo image of the patient in Images 13-18 shows low signal intensity (reflecting the presence of macroscopic fat) and lack of contrast enhancement in the left adrenal myelolipoma. Courtesy of S. Adusumilli, MD.
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Keywords
myelolipoma, adrenal gland myelolipoma, adrenal tumors, adrenal gland tumors, adrenal neoplasms, adrenal gland neoplasms
Contributor Information and Disclosures
Author
Parvati Ramchandani, MD, Associate Professor of Radiology, University of Pennsylvania School of Medicine; Consulting Staff, Section of Genitourinary Radiology, Department of Radiology, Hospital of the University of Pennsylvania at Philadelphia
Parvati Ramchandani, MD is a member of the following medical societies: American Association for Women Radiologists, American College of Radiology, American Roentgen Ray Society, Radiological Society of North America, and Society of Uroradiology
Disclosure: Nothing to disclose.
Pharmacy Editor
Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
Managing Editor
Arnold C Friedman, MD, FACR, Associate Chairman, Department of Radiology, University of Florida Health Science Center; Chief, Department of Radiology, Shands-Jacksonville Hospital
Arnold C Friedman, MD, FACR is a member of the following medical societies: American College of Radiology, American Institute of Ultrasound in Medicine, American Roentgen Ray Society, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.
CME Editor
Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.
Chief Editor
Eugene C Lin, MD, Consulting Staff, Department of Radiology, Virginia Mason Medical Center
Eugene C Lin, MD is a member of the following medical societies: American College of Nuclear Medicine, American College of Radiology, Radiological Society of North America, and Society of Nuclear Medicine
Disclosure: Nothing to disclose.