J Korean Assoc Oral Maxillofac Surg.  2011 Feb;37(1):67-71. 10.5125/jkaoms.2011.37.1.67.

Low-grade myxofibrosarcoma in the mandible: a case report

• Park JH
• Choi SY
• Kwon TG
• Kim CS

• KMID: 2189809
• DOI: http://doi.org/10.5125/jkaoms.2011.37.1.67

Abstract

Myxofibrosarcoma, also known as a myxoid variant of a malignant fibrous histiocytoma (MFH), is one of the most common sarcomas in the extremities of elderly people. The lesion is characterized by a high frequency of local recurrence but is uncommon in the head and neck regions. Low-grade myxofibrosarcoma, which is commonly misinterpreted as being benign, has a tendency for histological and biological progression in local recurrences, highlighting the importance of an accurate diagnosis and wide surgical excision of the primary lesion. We report a rare case of low-grade myxofibrosarcoma of the mandible located in the left mandibular body and angle area. The tumor was first diagnosed as a myxofibroma and was resected initially. After the final biopsy the patient underwent combined chemo-radiotherapy. The progress of the patent was uneventful until the one year follow up.

Keyword

Myxofibrosarcoma; Mandible; Recurrence; Biologic grading

MeSH Terms

Aged
Biopsy
Extremities
Fibroma
Follow-Up Studies
Head
Histiocytoma, Malignant Fibrous
Humans
Mandible
Neck
Recurrence
Sarcoma

Figure

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  Fig. 1. A. Extraoral photograph. Left facial swelling. B. Intraoral photograph. A well-circumscribed bony and gingival swelling of the left mandible, which measured 4 cm at its greatest diameter, suggesting benign tumor.

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  Fig. 2. A. Preoperative Panorama. A destructive process with osteolytic changes near the angle, effacement of adjacent cortical outline on these lesion. B. Preoperative CT view. Ill-defined multilocular radiolucent lesion with buccolingual, inferior cortical thinning and buccolingual cortical disruption.(CT: computed tomography)

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  Fig. 3. A. An intra/extraoral resection of the left mandible from the premolar to the angle was carried out. B. The specimen consisted of a smooth, lobulated mass, the edge of which was well defined and covered with periosteum, there was no evidence of a capsule. C. After the patient underwent extirpation of the lesion, the defect area was reconstructed by iliac bone and reconstruction plate.

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  Fig. 4. A. The tumor is composed of spindle or stellate cells lying in an abundant amorphous myxoid stroma that also contains isolate coarse and fine collagen fiber bundles.(H&E staining, original magnification ×10) B. The tissue is not very vascular, and many of the small vessels are surrounded by a zone of hyalinization.(arrows, H&E staining, original magnification ×40) C. The tumor is composed of elongated and angular cells lying in an abundant amorphous stroma that also contains isolate coarse and fine collagen fiber bundles.(H&E staining, original magnification ×40) D. In sone parts, there is little pleomorphism or hyperchromatism. Mitotic figures are very infrequent, and the appearances are like those of a myxofibroma. In other parts, however, there is greater variation in the cells, mitotic figures are frequent, and these include occasional abnormal forms.(arrows, H&E staining, original magnification ×40)

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  Fig. 5. Postoperative panorama. Dental implant were installed on both canine a premolar areas.

Reference

References

1. Gugatschka M, Beham A, Stammberger H, Schmid C, Friedrich G. First case of a myxofibrosarcoma of the vocal folds: case report and review of the literature. J Voice. 2010; 24:374–6.

2. Nishimura G, Sano D, Hanashi M, Yamanaka S, Tanigaki Y, Taguchi T, et al. Myxofibrosarcoma of the hypopharynx. Auris Nasus Larynx. 2006; 33:93–6.

3. Wada T, Hasegawa T, Nagoya S, Kawaguchi S, Kaya M, Ishii S. Myxofibrosarcoma with an infiltrative growth pattern: a case report. Jpn J Clin Oncol. 2000; 30:458–62.

4. Huang HY, Lal P, Qin J, Brennan MF, Antonescu CR. Low-grade myxofibrosarcoma: a clinicopathologic analysis of 49 cases treated at a single institution with simultaneous assessment of the efficacy of 3-tier and 4-tier grading systems. Hum Pathol. 2004; 35:612–21.

5. Angervall L, Kindblom LG, Merck C. Myxofibrosarcoma. A study of 30 cases. Acta Pathol Microbiol Scand A. 1977; 85A:127–40.

6. Mentzel T, Calonje E, Wadden C, Camplejohn RS, Beham A, Smith MA, et al. Myxofibrosarcoma. Clinicopathologic analysis of 75 cases with emphasis on the low-grade variant. Am J Surg Pathol. 1996; 20:391–405.

7. Sturgis EM, Potter BO. Sarcomas of the head and neck region. Curr Opin Oncol. 2003; 15:239–52.

8. Nascimento AF, Bertoni F, Fletcher CD. Epithelioid variant of myxofibrosarcoma: expanding the clinicomorphologic spectrum of myxofibrosarcoma in a series of 17 cases. Am J Surg Pathol. 2007; 31:99–105.

9. Kummoona R. Central myxofibrosarcoma of the mandible treated by radical resection. Oral Surg Oral Med Oral Pathol. 1975; 39:713–7.

10. Lam PK, Trendell-Smith N, Li JH, Fan YW, Yuen AP. Myxofibrosarcoma of the sphenoid sinus. J Laryngol Otol. 2002; 116:464–6.

11. Pomerantz JM, Sanfacon DG, Dougherty TP, Hanson S. Myxofibrosarcoma of the maxillary sinus. Del Med J. 1982; 54:147–52.

12. Weiss SW, Enzinger FM. Myxoid variant of malignant fibrous histiocytoma. Cancer. 1977; 39:1672–85.

13. Soper CP, Andrews PA, Bending MR, Singh L, Fisher C. Cervical myxofibrosarcoma in a renal allograft recipient treated with murine anti-CD3 monoclonal antibody therapy. Nephrol Dial Transplant. 1998; 13:1902–3.

14. Merck C, Angervall L, Kindblom LG, Ode′n A. Myxofibrosarcoma. A malignant soft tissue tumor of fibroblastic-histiocytic origin. A clinicopathologic and prognostic study of 110 cases using multivariate analysis. Acta Pathol Microbiol Immunol Scand Suppl. 1983; 282:1–40.

15. Kearney MM, Soule EH, Ivins JC. Malifnant fibrous histiocytoma: a retrospective study of 167 cases. Cancer. 1980; 45:167–78.