Hepatic Angiomyolipoma and Lipoma

 Only 50% of hepatic angiomyolipomas have substantial fat component



• Arterial phase: Prominent enhancement of nonfatty portion of lesion
image Central vessels within lesion if mass is large

• Fatty component of tumor results in hyperintense (high signal) foci on T1WI and T2WI

• MR, fat suppression ± opposed-phase GRE imaging




TOP DIFFERENTIAL DIAGNOSES




• Hepatocellular carcinoma

• Postoperative state, liver

• Focal steatosis

• Hepatic adenoma

• Hepatic lipoma

• Metastases 
image Teratoma or liposarcoma


PATHOLOGY




• Associated with tuberous sclerosis in < 10% of cases
image But some patients likely have forme fruste tuberous sclerosis


DIAGNOSTIC CHECKLIST




• Small, fat density hepatic mass in patient with tuberous sclerosis is almost certainly benign

• Angiomyolipoma that is primarily myeloid or angioid may be indistinguishable from other hepatic tumors, including hepatocellular carcinoma

image
(Left) Gross photograph of a fixed specimen shows a heterogeneous mottled tan, yellow, and brown tumor with areas of hemorrhage and degeneration image. Note that the background liver is not cirrhotic. (Courtesy J. Misdraji, MD.)


image
(Right) H&E-stained section shows a tumor composed of 3 elements: Adipose tissue image, vessels image, and plump spindle cells image. (Courtesy J. Misdraji, MD.)

image
(Left) Axial T2WI MR shows a heterogeneously bright mass with a fatty component image that is nearly isointense to subcutaneous fat image. The rest of the tumor has the moderate hyperintensity typical of most neoplasms on T2WI MR.


image
(Right) Axial T1WI MR in the same patient shows that most of the mass image is hyperintense, an unusual feature of most neoplasms and generally indicative of the presence of fat or hemorrhage within the mass. This tumor was resected and proved to be an isolated angiomyolipoma (AML).


TERMINOLOGY


Abbreviations




• Hepatic angiomyolipoma (AML)


Synonyms




• Benign hepatic hamartoma


Definitions




• Benign mesenchymal tumor composed of variable amounts of smooth muscle (myoid), fat (lipoid), and proliferating blood vessel (angioid) components


IMAGING


General Features




• Best diagnostic clue
image Well-circumscribed, mostly fatty mass in liver

• Location
image Liver is 2nd most common site (kidney is 1st)

• Size
image Variable; 0.3-36 cm in diameter

• Key concepts
image Round or lobulated solitary mass or multiple lesions with variable shape

image Only 50% of hepatic angiomyolipomas have substantial fat component
– Those without much fat are difficult to distinguish from other hepatic tumors


CT Findings




• NECT
image Well-defined mass with heterogeneous attenuation values due to presence of fat and soft tissue densities
– May be almost completely fat or soft tissue density mass

• CECT
image Arterial phase: Prominent enhancement of nonfatty portion of lesion

image Portal phase: Lesion shows hypoattenuation throughout mass

• CTA
image Central vessels within lesion if mass is large


MR Findings




• T1WI
Nov 16, 2016 | Posted by in GASTROINTESTINAL IMAGING | Comments Off on Hepatic Angiomyolipoma and Lipoma

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