REVIEW Malignant Fibrous Histiocytoma/Undifferentiated High-Grade Pleomorphic Sarcoma of the Maxillary Sinus Report of a Case and Review of the Literature Drazsen Vuity & Sandor Bogdan & Katalin Csurgay & Zoltan Sapi & Zsolt Nemeth Received: 24 January 2013 / Accepted: 11 April 2013 # Arányi Lajos Foundation 2013 Abstract Malignant fibrous histiocytoma (MFH) also known as undifferentiated high-grade pleomorphic sarcoma (UHPS) is a soft tissue sarcoma, composed of undifferentiated mesenchy- mal tumors possessed fibrohistiocytic morphology without def- inite true histiocytic differentiation. Head and neck localization is very rare, showing an incidence ranging from 4 % to 10 % in different series of investigations. The most frequent involved sites in UHPS are the neck and parotid, followed by the scalp, face, anterior skull base and orbit. Upper aerodigestive tract, lateral skull base and ear are rare locations. The incidence of the lymphatic metastases is also rare. The aim of this article is to report a case of UHPS in the maxillary sinus with palatal, orbital and ethmoidal involvement, with lymphatic metastasis and its surgical treatment. In addition, we review the literature of similar cases of the past 12 years. Keywords Malignant fibrous histiocytoma . Undifferentiated high-grade pleomorphic sarcoma . Orbito- ethmoidal spread . Young age . Lymphatic metastasis Abbreviations CHT Chemotherapy CT Computed tomography FNAB Fine needle aspiration biopsy MFH Malignant fibrous histiocytoma RT Radiotherapy UHPS Undifferentiated high-grade pleomorphic sarcoma WE Wide excision Background Malignant fibrous histiocytoma (also known as undifferentiated high-grade pleomorphic sarcoma) is a soft tissue sarcoma with- out definable line of differentiation. Historically it was first described in 1963 by Ozzello, who obsereved a tumor with storiform (cartwheel-like) growth pattern, with pleomorphic and giant tumor cells from tumoral cell-culture. The tumor displayed ameba-like movement and feature of phagocytosis was also seen. These cells had features similar to histiocytes and fibroblasts (due to their elongated shapes). Based on his observations, Ozzello called these tumor cells “facultative fibro- blasts” [1] because of their overlapping features between histio- cytes and fibroblasts. He used the term ‘malignant fibrous histiocytoma’ to describe this tumor. From the morphologic characteristics, it was divided into five subtypes: storiform- pleomorphic, myxoid (myxofibrosarcoma), giant cell (malignant giant cell tumor of soft parts), inflammatory and angiomatoid [2]. During the mid 1980s, an increasing number of diagnosed MFH began to cause problems to pathologists, because the morphologic patterns of MFH may be similar to many other sarcomas. In 1992, many of previously diagnosed MFH tu- mors were re-analysed using the up to date available immu- nohistochemical and electron microscopic techniques [3–5]. Only one tenth of the cases were re-diagnosed as MFH. In 2002, a similar survey of MFH histological cases showed only a 27 % concordance rate. So it was time to update the nomen- clature and classification of MFH. The 2002 WHO-classification considered the alternative name of old nomenclature of MFH as it gave a more accu- rate description of the origin of the tumor cells [6]. In the new classification the former ‘storiform-pleomorphic MFH’ diagnosis was modified to the term “undifferentiated high-grade pleomorphic sarcoma” (UHPS). The classification has been modified as well along with the nomenclature (Table 1). According to the present criteria in order to D. Vuity (*) : S. Bogdan : K. Csurgay : Z. Nemeth Department of Oro-Maxillofacial Surgery and Stomatology, Semmelweis University, Budapest, Hungary e-mail: drazsen@gmail.com Z. Sapi 1st Department of Pathology and Experimental Cancer Research, Semmelweis University, Budapest, Hungary Pathol. Oncol. Res. DOI 10.1007/s12253-013-9640-2