Sclerosing Perivascular Epitheloid Cell Tumor of the Falciform Ligament in a 34-year Old Woman

Case Report

Austin J Surg. 2014;1(3): 1013.

Sclerosing Perivascular Epitheloid Cell Tumor of the Falciform Ligament in a 34-year Old Woman

Andreas Puntschart1*, Katharina Meszaros1,2, Peter Kornprat1, Bernadette Liegl-Atzwanger2 and Hans Joerg Mischinger1

1Department of General Surgery, Medical University of Graz, Austria

2Department of Pathology, Medical University of Graz, Austria

*Corresponding author: Andreas Puntschart, Department of General Surgery, Medical University of Graz, Auenbruggerplatz 29, A-8036 Graz, Austria

Received: May 09, 2014; Accepted: May 30, 2014; Published: June 02, 2014

Abstract

PEComas represent rare, heterogeneous mesenchymal neoplasms derived from perivascular epitheloid cells exprimating smooth muscle as well as melanocytic markers. Most of described cases showed a benign clinical course; nevertheless, rare cases with development of metastases were described. The extremely rare sclerosing PEComa subtype with strong hyalinization of stroma was mainly reported in the retroperitoneum.

We report a 34–year old female patient with a sclerosing PECOMA of the falciform ligament, who was primarily admitted with an intra–abdominal mass. Radiologic examination revealed a partially cystic tumor near to the hilum of the liver and led to the radiologic diagnosis of a GIST (gastrointestinal stromal tumor).

The tumor could be resected completely; definitive histopathologic examination showed a sclerosing subtype of PEComa. The further postoperative course was uneventful; the patient could be discharged home on postoperative day eight. One year postoperatively, the patient is under close clinical and radiological follow–up proving absence of tumor recurrence.

The extreme rarity and the uncertain malignant potential of sclerosing PEComa subtype occurring outside the retro peritoneum represents a clinical challenge because of lacking oncological guidelines for the ideal treatment and follow–up protocol. The presented case shows that in such cases, a tailored multi–disciplinary approach and close follow–up intervals are mandatory.

Keywords: Sclerosing PEComa; Mesenchymal tumor; Falciform ligament

Introduction

Perivascular Epitheloid Cell tumors (PEComas) represent a rare tumor entity deriving from perivascular epitheloid cells exprimating smooth muscle as well as melanocytic markers in immunohistochemical assay [1,2]. Most of described cases showed a benign clinical course; nevertheless, rare cases with development of metastases were described [3]. The extremely rare sclerosing PEComa subtype associated with strong stroma hyalinization was mainly reported in the retroperitoneum [4,5].

We present a case of a 34–year old female patient with a sclerosing PEComa of the falciform ligament.

Case Presentation

A 34–year old female primarily admitted with a palpable solid intra–abdominal mass. Magnetic resonance imaging of the abdomen revealed a partially cystic mass of 4.5 x 5.0 centimeters located near to the hilum of the liver, immediately ventrally of the pancreatic head without infiltration of the adjacent structures (Figure 1A,1B).Especially in the T1 weighted images a solitary, marginal component of the tumor with contrast enhancement became obvious. Becauseof the central cystic transformation of the tumor with radiomorphologic signs of gastrointestinal stromal tumors, a PET–scan(positron emission tomography) using FDG (Fluordesoxyglucose)was performed. The PET–scan showed a missing tracer uptake of the cystic tumor but proved absence of multifocal tumor occurrence and⁄or suspected metastatic lesions.