Open AccessRetroperitoneal perivascular epithelioid cell tumours: A case report and review of literature
Author(s) -
Zisis Touloumis,
Niki Giannakou,
Christos Sioros,
Argiro Trigka,
Maria Cheilakea,
Nikoletta Dimitriou,
John Grınıatsos
Publication year - 2019
Publication title -
world journal of clinical cases
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.368
H-Index - 10
ISSN - 2307-8960
DOI - 10.12998/wjcc.v7.i21.3524
Subject(s) - medicine , lymphangioleiomyomatosis , angiomyolipoma , tuberous sclerosis , discovery and development of mtor inhibitors , perivascular epithelioid cell , pathology , epithelioid cell , retroperitoneal space , immunohistochemistry , radiology , kidney , pi3k/akt/mtor pathway , biology , apoptosis , biochemistry
The perivascular epithelioid cell tumour (PEComa) family of tumours mainly includes renal and hepatic angiomyolipomas, pulmonary lymphangioleiomyomatosis and clear cell "sugar" tumour of the lung. Several uncommon tumours with similar morphological and immunophenotypical characteristics arising at a variety of sites (abdominal cavity, digestive tract, retroperitoneum, skin, soft tissue and bones) are also included in the PEComa family and are referred to as PEComas not otherwise specified.