Journal List > J Korean Bone Joint Tumor Soc > v.19(1) > 1052022

Kong, Lee, Lee, Song, Cho, and Jeon: Inflammatory Myofibroblastic Tumor of Extremities

Abstract

Purpose

We analyzed the oncologic characteristics and outcome of patients with inflammatory myofibroblastic tumor of extremities.

Materials and Methods

Among the soft tissue tumor patients who were treated between 1999 and 2012, 5 patients who were pathologically confirmed as the inflammatory myofibroblastic tumor of extremities were analyzed retrospectively.

Results

There were 1 man and 4 women with mean age of 44 years (37–55 years). The average follow up was 34.6 months (8–87 months). All patients underwent surgical treatment. Only 1 patient had wide resection margin and remaining 4 had marginal (3) or intralesional (1) resection margin. All of 4 patients without wide resection margin developed local recurrence at 10.3 months (8–19 months). Malignant transformation to fibrosarcoma was occurred in 2 patients who developed local recurrence, and 1 patient developed multiple metastases to lung, liver and lymph nodes and expired at 37 months. Three of 5 patients had tumor location abutted to or invasion to major arteries and 1 patient had tumor invading sciatic nerve.

Conclusion

It is observed that inflammatory myofibroblastic tumor of extremities is usually located near the major neurovascular structure. Wide resection should be considered as the initial surgical treatment because this tumor showed a high local recurrence rate and possibility of malignant transformation.

References

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Figure 1.
53-year old woman with inflammatory myofibroblastic tumor on right thigh. (A, B) Initial MRI shows 14×6×11 cm sized poorly marginated high SI on T2WI soft tissue mass abutted to deep femoral vessel and proximal femoral shaft. (C) Spindle cells with abundant plasma cells and lymphocytes infiltrating between collagen fibers were observed (H&E, ×400). (D) Recurrence was found 19 months after marginal excision. MRI shows 8×5×5.3 cm sized infiltrating soft tissue mass which abutted to deep femoral vessels.
jkbjts-19-14f1.tif
Figure 2.
37-year old woman with inflammatory myofibroblastic tumor on left elbow. Incisional biopsy was done at another hospital and patient was referred to our clinic. (A, B) Initial MRI shows 8×4.6×4.6 cm sized poorly marginated, infiltrative soft tissue mass in left elbow surrounding meta-diaphysis of radius and abutted to proximal ulnar. Tumor encases ulnar and interosseous artery with suspicious invasion of ulnar nerve, and abutted to radial neurovascular bundle. (C) Marginal excision including radial head and ulnar artery, alcohol ablation of resected radial head, internal fixation and arteriorrhapy with vein graft were done. (D) Pathologic examination shows scar-like, hypocellular pattern which resembles fibromatosis. Spindle cells are dispersed in background of loose collagen and inflammatory cells (H&E, ×400). (E) Local recurrence was developed 9 months after marginal excision. Tumor abutted to ulnar neurovascular bundle.
jkbjts-19-14f2.tif
Figure 3.
33-year old woman with inflammatory myofibroblastic tumor on right buttock. (A, B) Initial MRI shows 6×6×5.6 cm sized tumor in right sciatic foramen involving sciatic nerve. After intralesional excision, adjuvant chemotherapy and radiation therapy was done. (C) Initial diagnosis was inflammatory myofibroblastic tumor. Loosely arranged spindle or plump cells are noted in a vascular and abundant collagenous background with a few chronic inflammatory cells (H&E, ×400). (D) 8 months later, local recurrence was developed and patient was referred to our clinic. (E) Neoadjuvant chemotherapy was done. MRI shows unchanged size with partial necrosis. Intralesional excision and following adjuvant chemotherapy were done. (F) 10 months later, local recurrence was found and excision was done. (G) Diagnosis was grade 3 spindle cell sarcoma. Tumor shows necrosis and compact fascicles, which consists of spindle cells with pleomorphism and marked hyperchromasia (H&E, ×400).
jkbjts-19-14f3.tif
Table 1.
Demographic and Clinico-Pathologic Data
Case Age Gender Tumor location Involvement of Neurovascular structure Treatment Surgical margin Time to relapse from first surgery (month) Malignant change from first surgery (month) Distant metastasis Patient survival F/U duration (month)
1 33 F Buttock Sciatic nerve Surgery & CTx Intralesional 8 26 Lung, bone DOD 37
2 55 F Thigh   Surgery Wide       NED 8
3 42 M Thigh Femoral artery Surgery Marginal 15 50   NED 87
4 53 F Thigh Deep femoral artery Surgery Marginal 19     NED 30
5 37 F Forearm Ulnar & radial artery Surgery & vessel graft Marginal l 9     NED 11

AWD, alive with disease; CDF, continuous disease free; DOD, dead of disease; NA, not available; CTx, Chemotherapy.

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