Soft Tissue Pathology

                         Extrarenal Angiomyolipoma  

                                   Dr Sampurna Roy MD

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January 2014

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Lipoma and variants



Extrarenal Angiomyolipoma

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                                                         Histopathology image of Angiomyolipoma

Angiomyolipomas (AMLs) are hamartomatous lesions containing smooth muscle, blood vessels and mature adipose tissue.

AML occurs most frequently in the kidneys.

This tumour has been reported in several other organs, including the liver, reproductive organs, skin, and retroperitoneum.

Gastrointestinal involvement is rare.

Cases have been reported in the colon, duodenum, stomach, and appendix.

These mesenchymal tumours  arise from the perivascular epithelioid cell (PEC), which may give rise to a variety of tumours and proliferations such as angiomyolipoma, lymphangiomyoma, lymphangiomyomatosis, clear cell myomelanocytic tumour of the falciform ligament/ligamentum teres, and pulmonary clear cell "sugar" tumour. 

Grossly, the tumour is yellow to gray depending on the relative proportion of the various tissue elements.

Histologically, the tumour consist of three components: mature fat cells, blood vessels, and smooth muscle cells.  Immunohistochemically, the proliferating smooth muscle cells are positive for vimentin, alpha-smooth muscle actin, and desmin.  

Angiomyolipomas also express the melanocytic markers HMB-45, MART-1 (Melan A), microphthalmia transcription factor, and tyrosinase. 

AML may be associated with Tuberous Sclerosis .

Although classic renal angiomyolipomas were regarded as universally benign lesions it is becoming increasingly clear that PEComas should be regarded as tumours of uncertain malignant potential.

 Tumours with a poor outcome are usually monotypic myomatous variants, especially those with dominant pleomorphic epithelioid components.

Factors likely to indicate poor outcome include presence of haemorrhage and necrosis, local invasiveness, and high mitotic activity.



Uterine angiomyolipoma with metastasis in a woman with tuberous sclerosis: a case report.

Anterior mediastinal angiomyolipoma.

Cutaneous angiomyolipoma.

Sporadic exophytic hepatic angiomyolipoma.

Extrarenal retroperitoneal angiomyolipoma mimicking metastatic melanoma: CT and FDG PET correlation.

Angiomyolipoma of the right adrenal gland.

Angiomyolipomas of the mediastinum and the lung.

[Extrarenal angiomyolipoma with spontaneous rupture: a case report].

[Angiomyolipoma of the parotid gland].

Colonic angiomyolipoma with a monotypic expression and a predominant epithelioid component .Journal of Clinical Pathology 2005;58:1107-1109

Nasal angiomyolipoma.Acta Otolaryngol. 2005;125(4):446-8.

Atypical pleomorphic epithelioid angiomyolipoma localized to the pelvis: a case report and review of the literature.Histopathology. 2004;44(3):292-5

Anterior mediastinal presentation of a giant angiomyolipoma.Ann Thorac Surg. 2004;78(6):2161-3

Angiomyolipoma of the colon - case report and review of the literature.Z Gastroenterol. 2003 ;41(8):715-8

Angiomyolipoma of the colon: report of a case and review of the literature.Dis Colon Rectum. 2003;46(4):547-9.

Primary monotypic epithelioid angiomyolipoma of bone. Histopathology 2002;40 (3):286-90

Epithelioid angiomyolipoma of the ovary: a case report and literature review.Int J Gynecol Pathol 2002 ;21(1):69-73

Expression of KIT (CD117) in Angiomyolipoma. Am J Surg Pathol 2002;26(4):493-7.

Uterine angiomyolipoma: case report and review of the literature.Pathol Int 2001; 51(11):896-901

Pleomorphic Angiomyolipoma of Digestive Tract: A Heretofore Unrecognized Entity.Int J Surg Pathol. 2000;8(1):67-72.

Clinicopathologic analysis of renal and extrarenal angiomyolipomas: report of 44 cases. Zhonghua Yi Xue Za Zhi (Taipei). 2000;63(1):37-44.

Angiomyolipoma of the parotid gland: a case report.Oral Surg Oral Med Oral Pathol Oral Radiol Endod 1999;87(6):738-41

Angiomyolipoma of the large intestine: report of a case.Mod Pathol. 1999 ;12(12): 1132-6.

Angiomyolipoma of the lung. Arch Pathol Lab Med 1998;122(11):1023-5

Angiomyolipoma arising in the colon. Am J Gastroenterol 1996;91(9):1852-4.

Fine needle aspiration cytology of renal and retroperitoneal angiomyolipoma. Report of two cases with cytologic findings and clinicopathologic pitfalls in diagnosis. Acta Cytol. 1995;39(5):945-50.

Intramyocardial angiomyolipoma. Am J Surg Pathol 1994;18(11):1164-9

Extrarenal angiomyolipoma with melanocytic and hibernoma-like features. Ultrastruct Pathol 1994;18(4):443-8

Cutaneous angiomyolipoma. A light-microscopic, immunohistochemical, and electron-microscopic study. Am J Dermatopathol 1991;13(5):497-502

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