creators_name: Challa, VR
creators_name: Swamyvelu, K
creators_name: Amirtham, U
creators_name: Rangappa, P
creators_name: Goud, BYG
creators_id: vasureddyc@gmail.com
editors_name: Kakkilaya, Srinivas
editors_id: Kakkilaya BS
type: journale
datestamp: 2013-05-04 23:09:51
lastmod: 2013-05-04 23:09:51
metadata_visibility: show
title: Epitheloid Variant of Angiomyolipoma in a Patient with Tuberous Sclerosis.
ispublished: pub
subjects: OJHAS
full_text_status: public
keywords: Epitheloid angiomyolipoma; Tuberous sclerosis; Hepatic angiomyolpoma; Pulmonary lymphangioleiomyomatosis.
abstract: Epitheloid angiomyolipoma of kidney is a type of Perivascular endothelial cell derived tumor with an aggressive behaviour with specific pathological, immunohistochemistrical and genetic characteristics. They can occur in a pure form or in assosciation with classical angiomyolipoma. It can be assosciated with tuberous sclerosis in 50% of cases. Our case is a possible case of tuberous sclerosis with epitheloid angiomyolipoma, hepatic angiomyolipoma and lymphangioleiomyomatosis with normal MRI brain and no cutaneous features. Radical nephrectomy with biopsy of hepatic lesion was performed. Histopathological examination revealed epitheloid variant with features of angiomyolipoma. It is six months post surgery and patient is doing well with no evidence of recurrence. Epitheloid angiomyolipoma is a rare malignancy with only 120 cases reported in literature.
date: 2013-01-25
date_type: published
publication: Online Journal of Health and Allied Sciences
volume: 11
number: 4(18)
publisher: Kakkilaya BS
refereed: TRUE
referencetext: 1.	Nese N, Martignoni G, Fletcher CD, Gupta R, Pan CC, et al Pure epithelioid PEComas (so-called epithelioid angiomyolipoma) of the kidney: A clinicopathologic study of 41 cases: detailed assessment of morphology and risk stratification. Am J Surg Pathol. 2011 Feb;35(2):161-176.
2.	Roach ES, Gomez MR, Northrup H. Tuberous sclerosis complex consensus conference: Revised clinical diagnostic criteria. J Child Neurol. 1998;13:624-628.
3.	Logue LG, Acker RE, Sienko AE. Best cases from the AFIP: Angiomyolipomas in tuberous sclerosis. Radiographics. 2003;23:241-246.
4.	Bonetti F, Pea M, Martignoni G,Zamboni G. PEC and sugar. Am J Surg Pathol 1992;16:307-308.
5.	Zamboni G, Pea M, Martignoni G et al. Clear cell ‘sugar’ tumour of the pancreas. A novel member of the family of lesions characterised by the presence of perivascular epitheloid cells. Am J Surg Pathol 1996;20:722-730.
citation:   Challa, VR and Swamyvelu, K and Amirtham, U and Rangappa, P and Goud, BYG  (2013) Epitheloid Variant of Angiomyolipoma in a Patient with Tuberous Sclerosis.  [Journal (On-line/Unpaginated)]     
document_url: http://cogprints.org/8947/1/2012-4-18.pdf