Diagnostic Ultrasound - Abdomen and Pelvis
Myelolipoma TERMINOLOGY Definitions • Rare benign tumor composed of mature fat tissue and hematopoietic elements (myeloid and erythroid cells) IMAGING General Features • Best diagnostic clue ○ Heterogeneous fat-containing adrenal mass • Location ○ Adrenal gland (85%): Thought to arise in zona fasciculata of adrenal cortex ○ Typically unilateral, only very rarely bilateral: 10:1 ○ Extraadrenal (15%): Retroperitoneal (12%) and intrathoracic (3%) ○ Possible right-sided predilection • Size ○ Usually 2-10 cm, rarely 10-20 cm Ultrasonographic Findings • Grayscale ultrasound ○ Well-defined, homogeneous, hyperechoic suprarenal mass (when predominantly composed of fatty tissue) ○ When small, difficult to distinguish from echogenic retroperitoneal fat ○ Apparent diaphragm disruption: Propagation speed artifact; decreased sound velocity through fatty mass leads to this appearance, usually seen when tumor > 4 cm ○ Heterogeneous mass (when myeloid cells predominate), may be isoechoic or hypoechoic ○ Heterogeneous echo pattern may also be due to internal hemorrhage (common), ± calcification • Color Doppler ○ Avascular to hypovascular adrenal mass Radiographic Findings • Radiography ○ Lucent mass with rim of residual normal adrenal cortex ○ May see calcification due to previous hemorrhage CT Findings • CT appearance usually characteristic ○ Typically appears as heterogeneous fat-containing adrenal mass – Macroscopic fat within tumor is diagnostic – Low attenuation of fat density (-30 to -90 HU) – Amount of fat is widely variable: Completely fat, to > 1/2 fat (50%), to only a few tiny foci of fat in soft tissue mass (10%) • Usually well-defined mass with recognizable capsule • Interspersed "smoky" areas of higher CT values than those of retroperitoneal fat because of presence of hematopoietic tissue in myelolipoma • Mass may have attenuation values between fat and water due to diffusely mixed fat and myeloid elements • Higher density areas may be seen with hemorrhage • Punctate calcification in 25-30% of cases • Occasionally the mass may appear to extend into retroperitoneum • Thin sections are recommended (to avoid volume averaging) if fatty tissue is not predominant ○ Or consider pixel mapping MR Findings • Varied MR appearance depending on mixture of elements ○ Fat within mass is hyperintense on T1- and T2WI ○ Hematopoietic elements are T1 hypointense and moderately hyperintense on T2WI • Fat-suppressed sequences best demonstrate intratumoral fat • Opposed-phase sequences can be helpful for characterizing presence of both fat and water ○ India ink artifact at boundary of fat and water confirms diagnosis ○ If mass is predominantly composed of mature fat cells with little intracellular water from soft tissue, little to no loss of signal seen on opposed phase sequence • Soft tissue elements enhance avidly after intravenous administration of gadolinium-based contrast Angiographic Findings • Conventional ○ Differentiate myelolipoma from retroperitoneal liposarcoma by determining origin of blood supply and vascularity of tumors Imaging Recommendations • Best imaging tool ○ Optimally imaged with NECT or MR with fat-suppression sequence • Protocol advice ○ Ultrasonography may be useful in diagnosis of large tumors; however, CT or MR are better in detecting smaller masses DIFFERENTIAL DIAGNOSIS Adrenal Hemorrhage • Usually well defined and round in shape • Acute: Hyperechoic or heterogeneous in echogenicity • Chronic: Well defined, hypoechoic, cystic, or calcified • Often in setting of blunt abdominal trauma, bleeding disorder, stress, or underlying tumor • Best characterized with CT or MR Pheochromocytoma • Variable appearance: Purely solid (68%), complex (16%), and cystic tumor (16%) • Small tumor: Round, solid, well-circumscribed mass with uniform echogenicity • Large tumor may appear as purely solid mass of homogeneous (46%) or heterogeneous (54%) echo pattern • Highly vascular; prone to hemorrhage and necrosis Adjacent Neoplasm • Renal angiomyolipoma ○ Well-defined, fat-containing, hyperechoic lesion arising exophytically from upper pole of kidney ○ Multiplanar CT or MR best demonstrates claw of renal parenchyma around part of lesion • Renal cell carcinoma (RCC) ○ Well-defined mass of variable echogenicity Diagnoses: Adrenal Gland 589
Myelolipoma Diagnoses: Adrenal Gland ○ When large may appear heterogeneous due to areas of necrosis and hemorrhage ○ Multiplanar CT or MR best characterizes origin from adjacent kidney • Liposarcoma ○ Retroperitoneal primary sarcoma in perirenal space ○ Displaces or invades adrenal gland as opposed to arising from it • Retroperitoneal teratoma ○ Uncommon neoplasm composed of mixed dermal elements derived from 3 germ cell layers ○ Fat-fluid (sebum) level and chemical shift between fat and fluid are pathognomonic Adrenal Adenoma • Well-defined, small, solid, round, hypoechoic adrenal mass • Lipid-rich adenoma shows greater signal loss on chemical shift MR, while myelolipoma shows greater signal loss with fat saturated sequences Adrenal Metastases/Lymphoma • Metastases: Usually < 5 cm, variable appearance, may be hypervascular on color Doppler ○ Usually known to have primary malignancy elsewhere • Lymphoma:Discrete or diffuse mass, adrenal shape maintained ○ Often bilateral with retroperitoneal masses or retroperitoneal tumor engulfing adrenal Adrenal Carcinoma • Rare, unilateral, solid mass with heterogeneous echogenicity • Exceedingly rare cases may contain macroscopic fat • Areas of necrosis and hemorrhage ± calcification (> 30%) PATHOLOGY General Features • Etiology ○ Best hypothesis: Metaplasia of adrenal cortical cells resulting from chronic stress or degeneration ○ Secondary hypothesis: Myelolipoma represents site of extramedullary hematopoiesis • Associated abnormalities ○ Adrenal collision tumors: Independently coexisting neoplasms without significant tissue admixture (e.g., adrenal adenoma and myelolipoma) ○ Endocrine disorders in 7%; Cushing syndrome, congenital adrenal hyperplasia (21-hydroxylase deficiency), and Conn syndrome ○ Nonhyperfunctioning adenoma 15% Gross Pathologic & Surgical Features • Mass with pseudocapsule; contains fat and soft tissue components Microscopic Features • Mature fat cells with variable mixture of myeloid cells, erythroid cells, megakaryocytes, and occasional lymphocytes; no malignant cells CLINICAL ISSUES Presentation • Most common signs/symptoms ○ Usually asymptomatic and incidental finding on CT, MR, or US (9% of adrenal incidentalomas) ○ Acute abdomen: Rupture with hemorrhage (rare) • Other signs/symptoms ○ Pain from necrosis, hemorrhage, or compression of structures Demographics • Age ○ Usually in older age group: 50-70 years ○ Uncommon in patients younger than 30 years • Gender ○ Occur with equal frequency in men and women • Epidemiology ○ Autopsy incidence: 0.2-0.4% Natural History & Prognosis • Complication: Rupture with hemorrhage usually when > 4 cm • Prognosis: Excellent • No malignant transformation Treatment • When diagnosis is certain and patient is asymptomatic, surgery is not necessary • If symptomatic, enlarging, or > 7 cm, surgery indicated due to increased risk of bleeding and rupture DIAGNOSTIC CHECKLIST Consider • Differentiate from other fat-containing tumors, most importantly retroperitoneal liposarcoma Image Interpretation Pearls • Well-marginated fat-containing adrenal mass is virtually diagnostic • Diagnosis: CT or MR; biopsy prone to sampling error • Presence of tumoral fat confirms diagnosis of this benign lesion, obviating need for further work-up SELECTED REFERENCES 1. Katabathina VS et al: Adrenal collision tumors and their mimics: multimodality imaging findings. Cancer Imaging. 13(4):602-10, 2013 2. Aron D et al: Adrenal incidentalomas. Best Pract Res Clin Endocrinol Metab. 26(1):69-82, 2012 3. Song JH et al: The incidental adrenal mass on CT: Prevalence of adrenal disease in 1049 consecutive adrenal masses in patients with no known malignancy. AJR 190: 1163-1168; 2008 4. Daneshmand S et al: Adrenal myelolipoma: diagnosis and management. Urol J. 3(2):71-4, 2006 590
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Myelolipoma<br />
Diagnoses: Adrenal Gl<strong>and</strong><br />
○ When large may appear heterogeneous due to areas of<br />
necrosis <strong>and</strong> hemorrhage<br />
○ Multiplanar CT or MR best characterizes origin from<br />
adjacent kidney<br />
• Liposarcoma<br />
○ Retroperitoneal primary sarcoma in perirenal space<br />
○ Displaces or invades adrenal gl<strong>and</strong> as opposed to arising<br />
from it<br />
• Retroperitoneal teratoma<br />
○ Uncommon neoplasm composed of mixed dermal<br />
elements derived from 3 germ cell layers<br />
○ Fat-fluid (sebum) level <strong>and</strong> chemical shift between fat<br />
<strong>and</strong> fluid are pathognomonic<br />
Adrenal Adenoma<br />
• Well-defined, small, solid, round, hypoechoic adrenal mass<br />
• Lipid-rich adenoma shows greater signal loss on chemical<br />
shift MR, while myelolipoma shows greater signal loss with<br />
fat saturated sequences<br />
Adrenal Metastases/Lymphoma<br />
• Metastases: Usually < 5 cm, variable appearance, may be<br />
hypervascular on color Doppler<br />
○ Usually known to have primary malignancy elsewhere<br />
• Lymphoma:Discrete or diffuse mass, adrenal shape<br />
maintained<br />
○ Often bilateral with retroperitoneal masses or<br />
retroperitoneal tumor engulfing adrenal<br />
Adrenal Carcinoma<br />
• Rare, unilateral, solid mass with heterogeneous<br />
echogenicity<br />
• Exceedingly rare cases may contain macroscopic fat<br />
• Areas of necrosis <strong>and</strong> hemorrhage ± calcification (> 30%)<br />
PATHOLOGY<br />
General Features<br />
• Etiology<br />
○ Best hypothesis: Metaplasia of adrenal cortical cells<br />
resulting from chronic stress or degeneration<br />
○ Secondary hypothesis: Myelolipoma represents site of<br />
extramedullary hematopoiesis<br />
• Associated abnormalities<br />
○ Adrenal collision tumors: Independently coexisting<br />
neoplasms without significant tissue admixture (e.g.,<br />
adrenal adenoma <strong>and</strong> myelolipoma)<br />
○ Endocrine disorders in 7%; Cushing syndrome,<br />
congenital adrenal hyperplasia (21-hydroxylase<br />
deficiency), <strong>and</strong> Conn syndrome<br />
○ Nonhyperfunctioning adenoma 15%<br />
Gross Pathologic & Surgical Features<br />
• Mass with pseudocapsule; contains fat <strong>and</strong> soft tissue<br />
components<br />
Microscopic Features<br />
• Mature fat cells with variable mixture of myeloid cells,<br />
erythroid cells, megakaryocytes, <strong>and</strong> occasional<br />
lymphocytes; no malignant cells<br />
CLINICAL ISSUES<br />
Presentation<br />
• Most common signs/symptoms<br />
○ Usually asymptomatic <strong>and</strong> incidental finding on CT, MR,<br />
or US (9% of adrenal incidentalomas)<br />
○ Acute abdomen: Rupture with hemorrhage (rare)<br />
• Other signs/symptoms<br />
○ Pain from necrosis, hemorrhage, or compression of<br />
structures<br />
Demographics<br />
• Age<br />
○ Usually in older age group: 50-70 years<br />
○ Uncommon in patients younger than 30 years<br />
• Gender<br />
○ Occur with equal frequency in men <strong>and</strong> women<br />
• Epidemiology<br />
○ Autopsy incidence: 0.2-0.4%<br />
Natural History & Prognosis<br />
• Complication: Rupture with hemorrhage usually when > 4<br />
cm<br />
• Prognosis: Excellent<br />
• No malignant transformation<br />
Treatment<br />
• When diagnosis is certain <strong>and</strong> patient is asymptomatic,<br />
surgery is not necessary<br />
• If symptomatic, enlarging, or > 7 cm, surgery indicated due<br />
to increased risk of bleeding <strong>and</strong> rupture<br />
DIAGNOSTIC CHECKLIST<br />
Consider<br />
• Differentiate from other fat-containing tumors, most<br />
importantly retroperitoneal liposarcoma<br />
Image Interpretation Pearls<br />
• Well-marginated fat-containing adrenal mass is virtually<br />
diagnostic<br />
• Diagnosis: CT or MR; biopsy prone to sampling error<br />
• Presence of tumoral fat confirms diagnosis of this benign<br />
lesion, obviating need for further work-up<br />
SELECTED REFERENCES<br />
1. Katabathina VS et al: Adrenal collision tumors <strong>and</strong> their mimics:<br />
multimodality imaging findings. Cancer Imaging. 13(4):602-10, 2013<br />
2. Aron D et al: Adrenal incidentalomas. Best Pract Res Clin Endocrinol Metab.<br />
26(1):69-82, 2012<br />
3. Song JH et al: The incidental adrenal mass on CT: Prevalence of adrenal<br />
disease in 1049 consecutive adrenal masses in patients with no known<br />
malignancy. AJR 190: 1163-1168; 2008<br />
4. Daneshm<strong>and</strong> S et al: Adrenal myelolipoma: diagnosis <strong>and</strong> management. Urol<br />
J. 3(2):71-4, 2006<br />
590