Low-grade fibromyxoid sarcoma of the liver: A case report

Abstract

Background: 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.

Case summary: 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.

Conclusion: 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.

Keywords: Case report; Fibromyxoid sarcoma; Histopathology; Liver; Resection.

Figure 1

Multi-detector computed tomography of the abdomen. 1: Tumor; 2: Infiltration of proper hepatic artery from the origin of the gastro-duodenal aretry up to the right second branching 3-complete infiltration of the left portal vein.

Figure 2

Histopathology. A: Mix of heavily collagenizedhypocellular zones -giant rosettes and cell-rich part of tumor [hematoxylin eosin staining (HE), 40 ×]; B: Short fascicular and characteristic whorling growth patterns are often seen. There are arcades of curvilinear blood vessels accompanied by perivascular hyaline degeneration (HE, 100 ×).

Figure 3

Immunohistochemistry. Tumor cells were diffusely and strongly positive for vimentin and MUC4, CD99 and epithelial membrane antigen were diffuse and slight expressed, and cytokeratin, smooth muscle actin, S-100 protein and neuron specific enolase were negative.

Figure 4

Intraoperative finding. T: Tumor; PV: Portal vein; CHA: Common hepatic artery; GDA: Gastroduodenal artery; CBD: Common bile duct.

Figure 5

Reconstruction of the common hepatic artery with saphenous vein graft. CHA: Common hepatic artery.

Figure 6

Abdominal and pelvic computed tomography after left hepatectomy-axial images. A: There is no tumor reccurence on surgical margin (white star) and no focal lesions in right liver lobe; B: An ill-defined lytic lesion (white star) of the L5 vertebral body is seen without periosteal reaction, representing solitary osseous metastasis of liver sarcoma.

Figure 7

Histopathology. Prominent vascularity in myxoid areas and perivascular hypercellularity seen in metastatic tumor corelate with changes in primary tumor.