Published online Jan 6, 2021. doi: 10.12998/wjcc.v9.i1.175
Peer-review started: May 25, 2020
First decision: June 12, 2020
Revised: September 15, 2020
Accepted: November 21, 2020
Article in press: November 21, 2020
Published online: January 6, 2021
Processing time: 221 Days and 0.8 Hours
Low grade fibromyxoid sarcoma (LGFMS) is a rare and benign mesenchymal tumor with indolent course, most commonly found in young or middle-aged men. The majority of the LGFMSs are located in the trunk and deep soft tissue of the lower extremities. They appear as well circumscribed, although not encapsulated, which often leads to incomplete surgical resection. Despite their seemingly benign appearance, these tumors have aggressive behavior with high metastatic and recurrence rates. Accurate histopathologic examination of the specimen and its immunohistochemical analysis are mandatory for a precise diagnosis.
We report a case of a 38 year-old-man who presented with jaundice and upper abdominal discomfort. Multi-detector computed tomography and magnetic resonance imaging showed a large left liver tumor mass, extending to the hepatoduodenal ligament. Left hepatectomy was performed with resection and reconstruction of hepatic artery and preservation of middle hepatic vein. Histopathologic examination confirmed the tumor being a low-grade fibromyxoid sarcoma. Three and a half years after surgery, the patient died after being diagnosed with spine metastasis.
Due to poor response to all modalities of adjuvant treatment, we consider that the focus of treatment should be on surgery as the only option for curing the disease.
Core Tip: Low grade fibromyxoid sarcoma (LGFMS) is very rare mesenchymal tumors with indolent course but aggressive biological behavior. There are no effective diagnostic procedures to achieve an accurate preoperative diagnosis. Symptoms are usually caused by compression on adjacent organs and structures. This report describes the case of a large left liver LGFMS in male patient, extending to the hepatoduodenal ligament, which was detected with abdominal ultrasound and confirmed by multi-detector computed tomography and magnetic resonance imaging. Left hepatectomy was performed and the tumor was completely removed at laparotomy.