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 Vascular Tumours

Pathology of Extrarenal Angiomyolipoma  

Dr Sampurna Roy MD


August 2014

 

Dr Sampurna Roy  MD

Consultant  Histopathologist (Kolkata - India)

 

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Vascular tumours

Angiokeratoma

Lobular capillary hemangioma (pyogenic granuloma

Bacillary angiomatosis

Verruga Peruana

Acro-angiodermatitis / pseudo-Kaposi's  sarcoma

Reactive angioendotheliomatosis

Infantile Hemangioma

Glomeruloid hemangioma 

Acquired tufted angioma

Verrucous hemangioma

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

Angiomyolipoma 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. 

 

Histopathology image of Angiomyolipoma

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. 

Angiomyolipoma 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.

 

                                                              

Further reading:

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.

Atypical pleomorphic epithelioid angiomyolipoma localized to the pelvis: a case report and review of the literature.

Anterior mediastinal presentation of a giant angiomyolipoma.

Angiomyolipoma of the colon - case report and review of the literature.

Angiomyolipoma of the colon: report of a case and review of the literature.

Primary monotypic epithelioid angiomyolipoma of bone.  

Epithelioid angiomyolipoma of the ovary: a case report and literature review.

Expression of KIT (CD117) in Angiomyolipoma.

Uterine angiomyolipoma: case report and review of the literature.

Pleomorphic Angiomyolipoma of Digestive Tract: A Heretofore Unrecognized Entity.

Clinicopathologic analysis of renal and extrarenal angiomyolipomas: report of 44 cases.  

Angiomyolipoma of the parotid gland: a case report.

Angiomyolipoma of the large intestine: report of a case.

Angiomyolipoma of the lung.

Angiomyolipoma arising in the colon.

Fine needle aspiration cytology of renal and retroperitoneal angiomyolipoma. Report of two cases with cytologic findings and clinicopathologic pitfalls in diagnosis.  

Intramyocardial angiomyolipoma.

Extrarenal angiomyolipoma with melanocytic and hibernoma-like features.  

Cutaneous angiomyolipoma. A light-microscopic, immunohistochemical, and electron-microscopic study.


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