J Korean Bone Joint Tumor Soc.  2014 Dec;20(2):54-59. 10.5292/jkbjts.2014.20.2.54.

Soft Tissue Malignant Myoepithelioma in the Extremities

• Kong CB
• Lee JW
• Koh JS
• Song WS
• Cho WH
• Jeon DG
• Lee SY

• KMID: 1799893
• DOI: http://doi.org/10.5292/jkbjts.2014.20.2.54

Abstract

PURPOSE
We report the diagnosis, treatment outcomes and prognosis of the patients with soft tissue malignant myoepithelioma in the extremities.
MATERIALS AND METHODS
We retrospectively reviewed 6 patients with soft tissue malignant myoepithelioma in the extremities who were treated at our institution between 2008 and 2014. Two patients received unplanned excision at another hospital and remaining 4 patients underwent the biopsy procedures and received wide excision at our hospital.
RESULTS
There were 3 men and 3 women with mean age of 41 (33-54) years. The average follow up was 28 (9-45) months. Among the 6 patients, only 4 patients underwent biopsy procedures under the impression of malignant soft tissue sarcoma. Surgical margins for these 4 patients were negative. Two patients who had unplanned excision received another re-excision and one of them showed no residual tumor in the resected specimen. Local recurrences were developed in all patients and distant metastasis in 4 patients. All 4 patients who developed distant metastasis died due to disease progression. Among the 2 patients who developed local recurrence only, one patient has another local recurrence after re-operation and remaining one patient is no evidence of disease for 2 years after resection of locally recurred mass.
CONCLUSION
Soft tissue malignant myoepithelioma in the extremities is a rare disease and shows an aggressive behavior. Appropriate biopsy under the impression of soft tissue malignancy is necessary and complete surgical resection with wide margins is the recommended treatment of choice.

Keyword

malignant myoepithelioma; soft tissue; extremities

MeSH Terms

Biopsy
Diagnosis
Disease Progression
Extremities*
Female
Follow-Up Studies
Humans
Male
Myoepithelioma*
Neoplasm Metastasis
Neoplasm, Residual
Prognosis
Rare Diseases
Recurrence
Retrospective Studies
Sarcoma

Figure

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  Figure 1. 40-year-old woman suffered from tingling sensation for two weeks (Case 5). (A) Initial T2-weighted axial MR image shows a large deep-seated soft tissue mass at right posterior thigh. (B) T2-weighted axial MR image which was taken 14 months after the index operation shows a local recurrence. (C) The tumor cells show variegated appearance from chondromyxoid to spindle cell morphology with hypercellularity and nuclear atypism (H&E stain, ×100). (D) High power field view reveals atypical tumor cells showing prominent nucleoli and frequent mitotic figures (H&E stain, ×400). (E) Immunostaining for keratin (PAN-K) is positive (×100).

• 

  Figure 2. 35-year-old man complained about his right arm mass which is developed 3 months ago (Case 2). (A) Initial T2-weighted axial MR image shows a soft tissue mass at right posterior upper arm. (B) After unplanned excision, T2-weighted axial MR image which was taken after referral shows a localized mass lesion. Re-excision was performed and the pathologic specimen revealed that there was no residual tumor cell. However, local recurrence developed at 29 months after re-excision. (C) High power field view reveals nuclear atypism and mitosis (H&E stain, ×400). (D) Immunostaining for keratin (PAN-K) is positive (×200). (E) Immunostaining for smooth muscle actin is also positive (×100).

Reference

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