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pathology station/Renal Angiomyolipoma

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Dr. Hamed Dadash Karimi Urologist

Date:
12/20/2018

Views:
169

Catgory:
Kidney

Describtion

Renal Angiomyolipoma

Benign tumor composed of fat, smooth muscle cells and thick-walled vessels; believe to originate from perivascular epithelioid cells (PEC) (aka, PEComa).
Related to other PEComas such as lymphangioleiomyomatosis, "sugar" tumor of lung, cardiac rhabdomyomas, etc.
May arise in association with tuberous sclerosis:
- >50% of patients with TS develop AMLs; <50% of AML has TS.
- Tend to be asymptomatic, bilateral, small, and multiple.
- Associated with genetic alteration in TSC1 (chr 9q34) and TSC2 (chr 16p13.3).
Abundant fat containing tumor recognizable radiologically.
Gross:
Range from 1-20 cm (average 9 cm).
Golden-yellow appearance is common, but depends on the ratio (>fat looks like lipoma; > smooth muscle looks like leiomyoma) (image A).
Usually well-demarcated but not encapsulated.
May show local "invasion" or extension into perinephric or sinus fat.
Consists of varying amounts of smooth muscle cells, mature fat and thick-walled vessels
(image B) and (image C).
Vessels abnormal, often hyalinized and thick with eccentric lumen.
Smooth muscle cells appear to originate and "radiate" off the vessels (image D).
AML with benign epithelial cysts (AMLEC) is a rare variant.
Histology:
IHC: spindle cells reactive with melanoma marker (HMB-45+, Mart1+) and actin+ thereby distinguishing it from other spindle cell lesions of the kidney.
DDX: lipoma (for fat predominant AML), leiomyoma (for smooth muscle predominant AML).
Surgical intervention recommended for tumors >4 cm (retroperitoneal hemorrhage is common complication)

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pathology station/Renal Angiomyolipoma

Describtion

Comments : 18

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