Soft Tissue Malignant Myoepithelioma in the Extremities

Chang-Bae Kong; Jung-Wook Lee; Jae-Soo Koh; Won Seok Song; Wan Hyeong Cho; Dae-Geun Jeon; Soo-Yong Lee

doi:10.5292/jkbjts.2014.20.2.54

Journal List > J Korean Bone Joint Tumor Soc > v.20(2) > 1052049

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Kong, Lee, Koh, Song, Cho, Jeon, and Lee: Soft Tissue Malignant Myoepithelioma in the Extremities

Original Article

J Korean Bone Joint Tumor Soc 2014;20(2):54-59.

Published online: 10 January 2014

DOI: https://doi.org/10.5292/jkbjts.2014.20.2.54

Soft Tissue Malignant Myoepithelioma in the Extremities

Chang-Bae Kong, Jung-Wook Lee, Jae-Soo Koh^*, Won Seok Song, Wan Hyeong Cho, Dae-Geun Jeon, Soo-Yong Lee

^*Pathology, Korea Cancer Center Hospital, Seoul, Korea

Departments of Orthopedic Surgery, Korea Cancer Center Hospital, Seoul, Korea

Correspondence to: Wan Hyeong Cho Department of Orthopedic Surgery, Korea Cancer Center Hospital, 75, Nowon-ro, Nowon-gu, Seoul 139-706, Korea TEL: +82-2-970-1243 FAX: +82-2-970-2403 E-mail: chowanda@naver.com

Received 30 October 2014 Revised 26 November 2014 Accepted 30 November 2014

This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/by-nc/3.0) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited.

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 reoperation 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.

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Keywords: malignant myoepithelioma, soft tissue, extremities

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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).

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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).

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Table 1.

Demographic and Clinico-pathologic Data

Case No.	Gender/ age	Tumor location	Symptom	Tumor size (cm)	Biopsy	Initial treatment	Surgical margin	Histologic Grade	Patient status	F/U duration (Month)
1	M/48	Shoulder	Mass	10	PCNB	W/E & Adj. CTx	Negative	Gr 2	NED	42
2	M/35	Upper arm	Mass	5	None	U/E	NA	Gr 3	AWD	36
3	M/33	Buttock	Mass	14	PCNB	W/E	Negative	Gr 2	DOD	15
4	F/38	Thigh	Mass	10	None	U/E	NA	Gr 2	DOD	45
5	F/40	Thigh	Mass & tingling sense	11	PCNB	W/E	Negative	Gr 2	DOD	22
6	F/54	Thigh	Mass	13	Frozen	W/E	Negative	Gr 2	DOD	9

PCNB, percutaneous core needle biopsy; W/E, wide excision; U/E, unplanned excision; Adj. CTx, adjuvant chemotherapy; NED, no evidence of disease; AWD, alive with disease; DOD, dead of disease; NA, not assessed.

Table 2.

Treatments for Local Recurrence and/or Metastasis

Case No.	Time to relapse from index surgery (months)	Treatment for local recurrence	Surgical margin of operation for local recurrence	Time to metastasis from index surgery (months)	Metastasis location	Treatment for metastasis
1	20	W/E & postop. RT	Positive	None	–	–
2	29	W/E	Negative	None	–	–
3	6	None	–	6	Lung	–
4	5	W/E	Negative	14	Abdomen, chest wall, pelvis	Excision
5	14	Preop. CTx & W/E	Negative	3	Lung	Wedge resection
6	3	W/E	Negative	5	Lung, Brain	CTx, brain RT

W/E, wide excision; RT, radiation therapy; CTx, chemotherapy.

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