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

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.