Abstract
Myxofibrosarcoma is a myxoid variant of malignant fibrous histiocytoma that most commonly involves the extremities of elderly people. However, a primary myxofibrosarcoma with bone invasion in young adults is extremely rare. Herein, we report the case of a 31-year-old male with a gradually enlarging left thigh mass, who had a history of left femur fracture and received an open reduction and internal fixation with titanium alloy plates and screws 33 months previously. Imaging investigations revealed an irregularly shaped soft tissue mass around the left femur shaft and a partial bone defect in the middle one-third of the left femur. Pathological examination of the resected specimen showed a multi-nodular appearance, abundant myxoid matrix and elongated curvilinear capillaries. Immunohistochemical studies revealed that the tumor cells was positive for VIM and MDM2, and was negative for CK, MSA, SMA, DES, S-100 and CD34. Labeling index of Ki-67 was 25%. Based on the morphological finding and immunostaining, it was diagnosed as a low-grade myxofibrosarcoma. The clinical and imaging examinations did not reveal the evidence of a primary cancer elsewhere, and the patient had no personal or family history of malignancy. To our knowledge, this is the first case of a primary myxofibrosarcoma developed following a fracture and metal implantation in young adults.Virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1745984882113605
Highlights
Myxofibrosarcoma is a myxoid variant of malignant fibrous histiocytoma (MFH), characterized by a nodular appearance, prominent myxoid matrix, elongated curvilinear capillaries, and location mostly in dermal and subcutaneous tissues in the extremities of elderly people [1,2,3]
It is noteworthy that the patient had a history of left femur fracture and received an open reduction and internal fixation with titanium alloy plates and screws 33 months previously
This is the first report on a primary myxofibrosarcoma that developed following a fracture and metal implantation in young adults
Summary
Myxofibrosarcoma is a myxoid variant of malignant fibrous histiocytoma (MFH), characterized by a nodular appearance, prominent myxoid matrix, elongated curvilinear capillaries, and location mostly in dermal and subcutaneous tissues in the extremities of elderly people [1,2,3]. It is noteworthy that the patient had a history of left femur fracture and received an open reduction and internal fixation with titanium alloy plates and screws 33 months previously To our knowledge, this is the first report on a primary myxofibrosarcoma. A high-resolution volumetric CT scanning revealed a 9.7 × 11 × 19 cm irregularly shaped soft tissue mass in the anteromedial left thigh (yellow arrow) and a partial bone defect approximately 9.7 cm in length on the medial border of the left femur shaft (light blue arrow). Tissue biopsy was performed and histopathological examination showed that the tumor exhibited the classical histological features of myxofibrosarcoma, including a nodular appearance, prominent myxoid matrix and elongated curvilinear capillaries. The patient eventually underwent left hip disarticulation due to the inability to control the tumor progression
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