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A rare case of malignant fibrous histiocytoma (pleomorphic undifferentiated sarcoma NOS) of the vocal fold.


Malignant fibrous histiocytoma, also known as pleomorphic undifferentiated sarcoma not otherwise specified, arises in numerous organs. The first-line treatment is complete excision of the mass, but in some cases postoperative chemo- and/or radiotherapy is recommended. Only a few cases of malignant fibrous histiocytoma of the vocal fold have been previously reported in the literature. We report a new case, which originated in the true vocal fold of a 65-year-old man. The mass was removed via C[O.sub.2] laser excision with preservation of the vocal fold and without the need for further treatment. At 3 years of follow-up, the patient remained recurrence-free.


Malignant fibrous histiocytoma (MFH), also known as pleomorphic undifferentiated sarcoma not otherwise specified (NOS), originates in the interstitial cells that differentiate fibroblasts and histiocytes. (1) These tumors are the most common of all the soft-tissue sarcomas seen in adults. Their incidence is highest in the trunk and the upper and lower limbs. They usually affect patients between the ages of 50 and 70 years. (2)

The treatment of MFH depends on the specific location of the tumor and the tumor grade. Surgery is generally the first-line option for soft-tissue sarcomas, with or without postoperative chemo- and/or radiotherapy. (3) Recurrence rates are fairly high, and metastases develop occasionally. (4)

MFHs in the head and neck are relatively uncommon, and tumors of the larynx are very rare. (5-7) Only a few cases of MFH of the vocal fold have been previously reported in the literature. (8,9) In this article, we report a new case of vocal fold MFH, which was initially diagnosed as a polyp.

Case report

A generally healthy 65-year-old man presented for evaluation of a change in his voice. The patient reported no relevant precipitating factors in his personal or family history. Examination of the nasal cavity and oral cavity and palpation of the neck detected no mass or other anomaly. However, an endoscopic examination of the larynx revealed the presence of a 0.5 x 0.5-cm polyp-shaped mass on the right vocal fold in the immediate posterior area of the anterior commissure (figure 1). Movement of the vocal fold was normal.

We initially diagnosed the mass as a polyp, and we performed a microscopic resection with general anesthesia and a C[O.sub.2] laser. However, surgical exploration revealed that the mass was hard, round, and firmly attached to the vocal fold. Since these characteristics were not typical of most vocal fold polyps, we suspected that it was a carcinoma. The mass was dissected with a 3-mm safety margin and a partial resection of the vocal fold ligaments. Frozen-section biopsy identified the mass as a sarcoma. The tumor had not invaded the muscle layer of the vocal fold. The surgery was completed after confirming the absence of tumors in the margin.

Postoperative pathologic examination confirmed that the surgical margin was negative. Findings on histopathology of the excised specimen were consistent with MFH (figure 2, A, B, and C).

Immunohistochemistry was negative for cytokeratin, positive for vimentin (figure 2, D) and CD68, and partially positive for smooth-muscle actin.

A subsequent examination for metastasis revealed no abnormalities. Three years postoperatively, endoscopy found no evidence of recurrence (figure 3).


According to the latest World Health Organization update on sarcoma, MFH can be classified as a pleomorphic undifferentiated sarcoma NOS after other forms of differentiation have been ruled out. (10) However, the term malignant fibrous histiocytoma is still widely used.

MFH is an aggressive tumor that requires radical surgery. Reported recurrence rates are as high as 44%, and rates of metastasis to the lungs and the lymph nodes are 82 and 32%, respectively. (11,12) The major predictors of outcome are size, depth of involvement, and the inflammatory component of the tumor. (11,12)

In addition to an MFH, the more common lesions encountered frequently in ENT clinics that should be differentiated from vocal fold polyps include laryngeal cysts, laryngeal papillomas, vocal fold hematomas, and early malignant tumors. Since malignant tumors that originate in the mesenchyme are very rare, the differential diagnosis is difficult. In most cases, therefore, they are diagnosed pathologically after local resection.

Radiologic findings in MFH of the head and neck area are nonspecific. The boundaries of the tumor are not clear, as they resemble a moth-eaten pattern with irregular bone margins and erosion of the cortex. Such findings are also observed frequently in squamous cell carcinoma. In our case, the value of imaging was limited because the tumor was so small. Since our case did not feature the typical clinical patterns of an MFH, we initially diagnosed it as a benign polyp.

Histologically, MFH exhibits a diverse cellular composition, as the fibrosarcomatous and histiocytomatous elements of the tumor are present in a mixed fashion. The most characteristic feature is the arrangement of spindle cells in a whorled or cartwheel pattern. Microscopic examination demonstrates a vigorous proliferation of fibroblasts and the appearance of polymorphic cells, many mitoses, bizarre cells, and large tumor cells.

Achieving a wide surgical margin without sacrificing important structures is the preferred treatment method. The risk of recurrence can be decreased by complete extraction during excision. (9) Most recurrences of MFH are attributable to insufficient resection. Sabesan et al reported that resection with close margins was associated with a local recurrence rate of up to 85%, whereas the recurrence rate with radical resection was only 27%. (4) Therefore, wide surgical margins are recommended.

The extent of the resection should not be determined simply by macroscopic observation during surgery. The margins should be confirmed to be safe by histopathology. If an incomplete resection is performed, postsurgical radiotherapy should be considered. (12,13) In several reported cases of MFH of the larynx, the patient was treated with total laryngectomy. (5-7) In our case, we were able to excise the tumor while preserving the vocal fold because the tumor affected only the surface of the fold.

While local radiotherapy and adjuvant chemotherapy can significantly increase survival rates and reduce the risk of metastasis in head and neck tumors, outcomes have varied depending on the tumor's specific location. (13-16) In our case, it appears that surgery alone was sufficient. This is consistent with reports of the treatment of squamous cell cancer of the vocal fold; if the vocal fold is sufficiently exposed and a surgical margin of at least 3 mm can be achieved without invading the anterior commissure, C[O.sub.2] laser resection alone is adequate. (17)

Since very few cases of vocal fold MFH have been reported, we can make no definitive statement about the prognosis. However, based on studies of other types of vocal fold tumors, we might be able to extrapolate that positive indicators include tumor size less than 5 cm, low tumor grade, localization of disease, and a complete resection. (3) If tumor cells are absent in the pathologic surgical margin, the incidence of local recurrence can be considered to be almost nothing.


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Jin Pyeong Kim, MD; Jin Yong Kim, MD; Gyung Hyuck Ko, MD; Seung Hoon Woo, MD

From the Department of Otorhinolaryngology-Head and Neck Surgery (Dr. J.P. Kim, Dr. J.Y. Kim, and Dr. Woo), the Institute of Health Sciences (Dr. J.P. Kim and Dr. Woo), and the Department of Pathology (Dr. Ko), College of Medicine, Gyeongsang National University, Jinju, Republic of Korea.

Corresponding author: Seung Hoon Woo, MD, Department of Otorhinolaryngology-Head and Neck Surgery, Gyeongsang National University Hospital, 90 Chilam-dong, Jinju, Republic of Korea 660-702. Email:

Funding/support: The preparation of this article was supported by the Basic Science Research Program through the National Research Foundation (NRF) of Korea and funded by the Ministry of Science, ICT, and Future Planning (2013R1A1A1012542). It was also supported by the Leading Foreign Research Institute Recruitment Program through the NRF and funded by the Ministry of Education, Science, and Technology (2012K1A4A3053142).
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Article Details
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Author:Kim, Jin Pyeong; Kim, Jin Yong; Ko, Gyung Hyuck; Woo, Seung Hoon
Publication:Ear, Nose and Throat Journal
Article Type:Case study
Geographic Code:1USA
Date:Jul 1, 2015
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