8860/0 Angiomyolipoma


Definitions

Liver and intrahepatic bile ducts
ICD-O-3 topography code: C22

A neoplasm with perivascular epithelioid cell differentiation often associated with tuberous sclerosis. It is characterized by a mixture of epithelioid cells, smooth muscle, vessels, and mature adipose tissue. It is currently regarded as a tumour of perivascular epithelioid cells (PEComa) exhibiting dual myomatous and lipomatous differentiation and melanogenesis 1
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Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F (2008)
PEComas: the past, the present and the future.
Virchows Arch 452: 119-32



2
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Tsui WM, Colombari R, Portmann BC, Bonetti F, Thung SN, Ferrell LD, Nakanuma Y, Snover DC, Bioulac-Sage P, Dhillon AP (1999)
Hepatic angiomyolipoma: a clinicopathologic study of 30 cases and delineation of unusual morphologic variants.
Am J Surg Pathol 23: 34-48



3
 
Bosman FT, Carneiro F, Hruban RH, Theise ND (Eds.)
WHO Classification of Tumours of the Digestive System.
4th Edition
International Agency for Research on Cancer: Lyon 2010



. The kidney is the most common site of involvement 4
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



. Other sites of involvement include the liver, lung, lymph nodes, and retroperitoneum. The vast majority of cases follow a benign clinical course. However, cases of metastatic angiomyolipomas with sarcomatoid features have been described.



Kidney
ICD-O-3 topography code: C64

/span>ngiomyolipoma is a benign neoplasm often associated with tuberous sclerosis . It is histologically characterized by a mixture of perivascular epithelioid cells, smooth muscle, vessels, and mature adipose tissue. The kidney is the most common site of involvement
5
 
Eble JE, Sauter G, Epstein JI, Sesterhenn IA (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of the Urinary System and Male Genital Organs.
3rd Edition
IARC Press: Lyon 2004



. Other sites of involvement include the liver, lung, lymph nodes, and retroperitoneum. The vast majority of cases follow a benign clinical course. However, cases of metastatic angiomyolipomas with sarcomatoid features have been described.

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Faraji H, Nguyen BN, Mai KT (2009)
Renal epithelioid angiomyolipoma: a study of six cases and a meta-analytic study. Development of criteria for screening the entity with prognostic significance.
Histopathology 55: 525-34



.



Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49

A neoplasm with perivascular epithelioid cell differentiation often associated with tuberous sclerosis. It is characterized by a mixture of epithelioid cells, smooth muscle, vessels, and mature adipose tissue. The kidney is the most common site of involvement. Other sites of involvement include the liver, lung, lymph nodes, and retroperitoneum. The vast majority of cases follow a benign clinical course. However, cases of metastatic angiomyolipomas with sarcomatoid features have been described.