title: Malignant hepatic perivascular epithelioid cell tumor (PEComa) - Case report and a brief review.
abstract: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms which can arise from almost any location in the body. Diagnosing them pre-operatively is difficult as they mimic features of other hepatic neoplasms including hepatocellular carcinoma (HCC), fibrolamellar HCC, and focal nodular hyperplasia (FNH) among others. The unique feature of these tumors is the coexpression of muscle and melanocytic markers. These are identified immunohistochemically by the expression of Human Melanin Black-45 (HMB-45), Melan-A and Smooth Muscle Antigen (SMA) which are seen in the majority of tumors. The liver is uncommonly associated with a PEComa and the approach to a patient with hepatic PEComa is not well described. There is no consensus regarding the neo-adjuvant/adjuvant therapy in these patients. The natural history of this condition is not well documented making it an unpredictable disease. Here we have discussed a case and reviewed the literature concerning these rare tumors.
authors.affiliation: P.D. Hinduja National Hospital and Medical Research Centre, Veer Savarkar Marg, Mahim, Mumbai, Maharashtra 400016, India. Electronic address: drabhirupbanerjee@gmail.com.
authors.forename: Banerjee
authors.initials: B
authors.lastname: Abhirup
authors.affiliation: P.D. Hinduja National Hospital and Medical Research Centre, Veer Savarkar Marg, Mahim, Mumbai, Maharashtra 400016, India.
authors.forename: Kundalia
authors.initials: K
authors.lastname: Kaushal
authors.affiliation: Saifee Hospital, No. 15/17, Maharshi Karve Road, Opp Charni Road Railway Station, Mumbai, Maharashtra 400004, India.
authors.forename: Mehta
authors.initials: M
authors.lastname: Sanket
authors.affiliation: P.D. Hinduja National Hospital and Medical Research Centre, Veer Savarkar Marg, Mahim, Mumbai, Maharashtra 400016, India.
authors.forename: Nagarajan
authors.initials: N
authors.lastname: Ganesh
mesh_terms: D000368:Aged; D001706:Biopsy; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D008113:Liver Neoplasms / Q000175:diagnosis*; D009364:Neoplasm Recurrence, Local; D054973:Perivascular Epithelioid Cell Neoplasms / Q000175:diagnosis* / Q000601:surgery; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome
keywords: Malignant hepatic PEComa; Mesenchymal liver neoplasm; PEComa; Perivascular epithelioid cell tumor; Rare liver tumors