Perivascular epithelioid cell tumor outgrowth from the liver

INTRODUCTION: Perivascular epithelioid celltumor (PEComa) is a rare mesenchymal neoplasia and can be found in various body sites. On the other hand, hepatic PEComa is very rare, with only a few studies having reported hepatic malignant PEComa. There is no gold standard regarding the use of diagnostic imaging studies. The diagnosis of hepatic PEComa is made by a positive immunohistochemical staining for HMB45 and Melan A. Herein, we discussed the therapeutic and follow-up process of a symptomatic hepatic PEComa case. PRESENTATION OF CASE: A 22-year-old woman presented with a palpable mass in abdomen. A computerized tomographic examination showed a giant hepatic mass of left lobe origin, which was excised surgically. The pathology result was reported as a PEComa. DISCUSSION: The diagnostic approach, treatment modalities, and follow-up procedures are not standard. The main treatment modality for PEComa is surgical excision with adequate surgical margin. CONCLUSION: A longer follow-up is required for patients with hepatic PEComa because the nature of the disease is not entirely clear. (C) 2018 The Author(s). Published by Elsevier Ltd on behalf of IJS Publishing Group Ltd