ORIGINAL ARTICLE MALIGNANT FIBROUS HISTIOCYTOMA OF THE HEAD AND NECK REGION David W. Clark, MD, 1,2 Brian A. Moore, MD, 3 Shreyaskumar R. Patel, MD, 4 B. Ashleigh Guadagnolo, MD, 5 Dianna B. Roberts, PhD, 1 Erich M. Sturgis, MD, MPH 1,6 1 Department of Head and Neck Surgery, The University of Texas M. D. Anderson Cancer Center, Houston, Texas. E-mail: esturgis@mdanderson.org 2 Department of Otolaryngology–Head and Neck Surgery, The University of Texas Health Science Center, Houston, Texas 3 Department of Otolaryngology–Head and Neck Surgery, Eglin Hospital, Eglin Air Force Base, Florida 4 Department of Sarcoma Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 5 Department of Radiation Oncology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas 6 Department of Epidemiology, The University of Texas M. D. Anderson Cancer Center, Houston, Texas Accepted 23 February 2010 Published online 13 July 2010 in Wiley Online Library (wileyonlinelibrary.com). DOI: 10.1002/hed.21449 Abstract: Background. Malignant fibrous histiocytoma (MFH) is 1 of the most common soft tissue sarcomas in the head and neck. Methods. We conducted a retrospective review of patients with MFH of the head and neck region at a large multidiscipli- nary cancer center between 1973 and 2005. Results. Ninety-five patients were included in the study. The median age at diagnosis was 53 years (range, 3–90 years); 69% of the patients were men. The parotid or neck was the most common subsite (35%), and 23% of the cases were associated with prior radiation exposure. Although there were no significant differences in the distribution of age, sex, year of presentation, tumor location, size, local extension, or treatment between patients with and without prior radiation exposure, those with radiation-associated tumors were more likely to have positive or unclear surgical margins (p ¼ .037). With a median follow-up of 34 months, 32 (39%) of the 83 patients treated at M. D. Anderson Cancer Center with curative intent had a recurrence (isolated local recurrence in 18, isolated dis- tant recurrence in 8, both local and distant recurrence in 5, and regional recurrence in 1). For patients treated at our insti- tution with curative intent, 5-year overall, disease-free, and dis- ease-specific survival rates were 55%, 44%, and 69%, respectively. Prior radiation exposure and positive margins were associated with worse survival. Conclusion. MFH of the head and neck region is often aggressive and characterized by local and/or distant recur- rence and poor survival. Radiation-associated tumors seem to have an especially poor prognosis. Based on a significant body of literature, multidisciplinary evaluation and treatment of such high-grade sarcomas is encouraged. V V C 2010 Wiley Periodicals, Inc. Head Neck 33: 303–308, 2011 Keywords: sarcoma; malignant fibrous histiocytoma; head and neck neoplasms; radiation-associated malignancies Malignant fibrous histiocytoma (MFH) is a soft tis- sue sarcoma that was first described in 1964. 1 Histor- ically, it is the most common soft tissue sarcoma. 2,3 Sarcomas can arise because of inherited mutations in tumor suppressor genes, and also because of environ- mental exposures; the most common environmental etiology is exposure to ionizing radiation. 4 In fact, MFH seems to be the most common sarcoma diagno- sis in patients with prior radiation exposure in the head and neck region. 5 The cell of origin for MFH was originally thought to be fibroblastic progenitors. 6 However, diagnosis of MFH has undergone scrutiny as a unique pathologic entity, and today many con- sider MFH to be synonymous with undifferentiated pleomorphic soft tissue sarcoma. 7 Most MFHs are considered high-grade, and their classic behavior is to recur locally. Regional metasta- ses are extremely rare, but distant metastases are common. Approximately 3% to 10% of cases of MFH occur in the head and neck region. 4 However, although MFH is 1 of the more common soft tissue sarcomas in the head and neck region, it remains a rare head and neck malignancy. 4 For most MFHs of the head and neck region, surgery is the mainstay of treatment, and adjuvant chemotherapy and radio- therapy seem to be critical for successful treatment. However, a consistently effective strategy for MFHs of the head and neck remains elusive in part because, given the low numbers of patients with this disease, prospective randomized trials of treatment are diffi- cult to conduct. 3 Furthermore, radiation-associated MFH of the head and neck region seems to be associ- ated with a particularly grave prognosis. 8 The purpose of this study was to review the demo- graphic and clinical characteristics at presentation and the general outcomes of patients with MFH of the head and neck region presenting to a large multi- disciplinary cancer center. Correspondence to: E. M. Sturgis V V C 2010 Wiley Periodicals, Inc. Malignant Fibrous Histiocytoma of the Head and Neck HEAD & NECK—DOI 10.1002/hed March 2011 303