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Low-grade myxofibrosarcoma following a metal implantation in femur: a case report.

Li W, Li D, Zhu X, Lu S, He C, Yang Q - Diagn Pathol (2014)

Bottom Line: 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%.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pathology, Gannan Medical University, No, 1, Yixueyuan Road, Ganzhou, Jiangxi 341000, China. dan.li.liu@hotmail.com.

ABSTRACT

Unlabelled: 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 slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1745984882113605.

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Immunohistochemical studies of the tumor. (a-i) Magnification, ×200. The tumor cells expressed strong immunoreactivity for VIM (a) and negative for CK (b). Labeling index of Ki-67 (c) was 25%. MSA (d), SMA (e), DES (f) and S-100 protein (g) were negative in the tumor cells. (h) CD34 staining was detected in vascular endothelial cells rather than in tumor cells, highlighting the curvilinear capillaries. (i) MDM2 was positive in the tumor cells.
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Figure 3: Immunohistochemical studies of the tumor. (a-i) Magnification, ×200. The tumor cells expressed strong immunoreactivity for VIM (a) and negative for CK (b). Labeling index of Ki-67 (c) was 25%. MSA (d), SMA (e), DES (f) and S-100 protein (g) were negative in the tumor cells. (h) CD34 staining was detected in vascular endothelial cells rather than in tumor cells, highlighting the curvilinear capillaries. (i) MDM2 was positive in the tumor cells.

Mentions: Macroscopically, the excised specimen was a grayish-white or grayish-brown mass measured 7 × 14 × 10 cm. On the cut section, a fish-like appearance with central necrosis area was observed. Microscopic features were identical to that of the biopsy. Immunohistochemical studies showed that the tumor cells expressed strong immunoreactivity for vimentin (VIM) and negative for cytokeratin (CK), suggesting a mesenchymal histogenesis (Figure 3a and b). Labeling index of Ki-67 (the number of Ki-67 positive tumor cells divided by the sum of Ki-67 positive and negative tumor cells) was 25% (Figure 3c). Muscle-specific actin (MSA), smooth muscle actin (SMA) and desmin (DES) were negative in the tumor cells, eliminating the possibility of a muscle-derived tumor (Figure 3d-f)[13]. The negative expression of S-100 protein eliminated a tumor of neural or adipose tissue origin (Figure 3g). The tumor cells were negative for cluster of differentiation 34 (CD34) (Figure 3h), B cell lymphoma/lewkmia-2 and anaplastic lymphoma kinase, which eliminated the possibility of a solitary fibrous tumor or inflammatory myofibroblastic tumor[14]. Interestingly, the positive staining of CD34 in vascular endothelial cells highlighted the curvilinear capillaries, which was an important histological feature of myxofibrosarcoma. In addition, positive expression of murine double minute 2 (MDM2) was observed in the tumor cells. Although MDM2 immunostaining is useful adjunct in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes, overexpression of MDM2 has also been reported in a small number of myxofibrosarcomas[15-17]. The combination of clinical and pathological features revealed a low-grade myxofibrosarcoma.


Low-grade myxofibrosarcoma following a metal implantation in femur: a case report.

Li W, Li D, Zhu X, Lu S, He C, Yang Q - Diagn Pathol (2014)

Immunohistochemical studies of the tumor. (a-i) Magnification, ×200. The tumor cells expressed strong immunoreactivity for VIM (a) and negative for CK (b). Labeling index of Ki-67 (c) was 25%. MSA (d), SMA (e), DES (f) and S-100 protein (g) were negative in the tumor cells. (h) CD34 staining was detected in vascular endothelial cells rather than in tumor cells, highlighting the curvilinear capillaries. (i) MDM2 was positive in the tumor cells.
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC3926977&req=5

Figure 3: Immunohistochemical studies of the tumor. (a-i) Magnification, ×200. The tumor cells expressed strong immunoreactivity for VIM (a) and negative for CK (b). Labeling index of Ki-67 (c) was 25%. MSA (d), SMA (e), DES (f) and S-100 protein (g) were negative in the tumor cells. (h) CD34 staining was detected in vascular endothelial cells rather than in tumor cells, highlighting the curvilinear capillaries. (i) MDM2 was positive in the tumor cells.
Mentions: Macroscopically, the excised specimen was a grayish-white or grayish-brown mass measured 7 × 14 × 10 cm. On the cut section, a fish-like appearance with central necrosis area was observed. Microscopic features were identical to that of the biopsy. Immunohistochemical studies showed that the tumor cells expressed strong immunoreactivity for vimentin (VIM) and negative for cytokeratin (CK), suggesting a mesenchymal histogenesis (Figure 3a and b). Labeling index of Ki-67 (the number of Ki-67 positive tumor cells divided by the sum of Ki-67 positive and negative tumor cells) was 25% (Figure 3c). Muscle-specific actin (MSA), smooth muscle actin (SMA) and desmin (DES) were negative in the tumor cells, eliminating the possibility of a muscle-derived tumor (Figure 3d-f)[13]. The negative expression of S-100 protein eliminated a tumor of neural or adipose tissue origin (Figure 3g). The tumor cells were negative for cluster of differentiation 34 (CD34) (Figure 3h), B cell lymphoma/lewkmia-2 and anaplastic lymphoma kinase, which eliminated the possibility of a solitary fibrous tumor or inflammatory myofibroblastic tumor[14]. Interestingly, the positive staining of CD34 in vascular endothelial cells highlighted the curvilinear capillaries, which was an important histological feature of myxofibrosarcoma. In addition, positive expression of murine double minute 2 (MDM2) was observed in the tumor cells. Although MDM2 immunostaining is useful adjunct in diagnosing well-differentiated and dedifferentiated liposarcoma subtypes, overexpression of MDM2 has also been reported in a small number of myxofibrosarcomas[15-17]. The combination of clinical and pathological features revealed a low-grade myxofibrosarcoma.

Bottom Line: 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%.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Department of Pathology, Gannan Medical University, No, 1, Yixueyuan Road, Ganzhou, Jiangxi 341000, China. dan.li.liu@hotmail.com.

ABSTRACT

Unlabelled: 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 slides: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1745984882113605.

Show MeSH
Related in: MedlinePlus