Please cite this article in press as: Boaz K, et al. Rapidly enlarging swelling of the palate: A case report. J Oral Maxillofac Surg Med Pathol (2017), http://dx.doi.org/10.1016/j.ajoms.2017.01.008 ARTICLE IN PRESS G Model JOMSMP-602; No. of Pages 5 Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology xxx (2017) xxx–xxx Contents lists available at ScienceDirect Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology j o ur nal ho me pa ge: www.elsevier.com/locate/jomsmp Case report Rapidly enlarging swelling of the palate: A case report K. Boaz a , A. Sharma a , N. Srikant a,∗ , R.M. Kumar b , A. Kumar b , B. Shakthi Dorai a a Department of Oral Pathology and Microbiology, Manipal College of Dental Sciences, Light House Hill Road, Manipal University, Mangalore, Karnataka, India b TCR Multispecialty Hospital, Krishnagiri, Tamilnadu, India a r t i c l e i n f o Article history: Received 26 October 2016 Received in revised form 25 January 2017 Accepted 31 January 2017 Available online xxx Keywords: Malignant fibrous histiocytoma Undifferentiated High Grade Pleomorphic Sarcoma CD68 Vimentin a b s t r a c t Undifferentiated high-grade pleomorphic sarcoma (UHPS)/pleomorphic sarcoma NOS (earlier MFH) is a soft tissue sarcoma which shows fibro-histiocytic morphology without a definitive lineage. These tumours are extremely rare in the head and neck region and account for only 0.1% of all sarcomas in the oral cavity. A rare case of UHPS of palate is hereby reported in a 55 year old female patient with emphasis on sequential diagnosis, pathogenesis, and evolution of current terminology. We also aim to address the role of immunohistochemical profiling in head and neck sarcomas and the impact of histo-differentiation on the prognosis of such patients. © 2017 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved. 1. Introduction Sarcomas are rare neoplasms which account for only about 1% of all tumours (overall). These tumours are uncommon with 5–15% occurrence in the head and neck in adults, whereas in the paediatric population 1 in 3 sarcomas are seen in the head and neck region [1] and represent 7% of all paediatric neoplasms [2]. Adult soft tissue sarcomas comprise a heterogeneous group of tumours of diverse histological backgrounds amongst which the most frequently encountered variants in the head and neck region are the Malignant fibrous histiocytoma (now termed as undiffer- entiated high-grade Pleomorphic sarcoma as adapted by the 2012 update by WHO on soft tissue sarcomas), fibrosarcoma, angiosar- coma and malignant peripheral nerve sheath tumour [3]. Based on histological subtyping, 50% of sarcomas in head and neck region are either osteosarcoma, rhabdomyosarcoma, malignant fibrous histi- ocytoma, fibrosarcoma or angiosarcoma [2]. Malignant fibrous histiocytoma (now known as undifferentiated high-grade pleomorphic sarcoma; 2002/Pleomorphic Sarcoma, NOS) is a soft tissue sarcoma without a definitive line of differ- entiation. It was first described by Ozzello (1963) as a tumor with pleomorphic and giant tumor cells that were thought to be derived from histiocytes and could assume the form and function of fibrob- lasts (“facultative fibroblasts”) in storiform (cartwheel-like) growth ∗ Corresponding author. E-mail address: srikant.n@manipal.edu (N. Srikant). pattern. This evidence was based principally on tissue culture stud- ies of tumor explants [4]. O’Brien and Stout (1964) coined the term ‘malignant fibrous histiocytoma’ for the tumour derived from these cells. From the morphologic characteristics, it was divided into five subtypes: storiform-pleomorphic, myxoid, giant cell, inflammatory and angiomatoid [5]. We hereby present a case of UHPS (Undiffer- entiated High-grade Pleomorphic Sarcoma) affecting the palate in an adult and highlight the sequence of diagnosis along with dis- cussion of evolution of present day terminology and underlying concepts of origin of this tumor. 2. Case report A 55 year old female patient reported to an oral and maxillo- facial surgeon with an intra-oral nodular growth on the surface of the palate crossing the midline. There was no significant his- tory of habit, or medical or familial history. Extra-oral examination did not reveal any abnormality. The fungating growth was firm in consistency with ulceration. Radiographically, the CECT (Contrast enhanced computed tomography) showed a moderately enhanced multilobulated soft tissue mass measuring 8.2 × 5.5 × 4.5 cm which was seen to arise from the left anterior aspect of hard palate and left side of body of maxilla with extension into the nasal cavity, infratemporal fossa and maxillary sinus. Erosion of the alveolar pro- cess, body of the maxilla and left side of the hard palate was noted with displacement of the inferior turbinate and masticatory mus- cles on the left side. Multiple enlarged lymph nodes in bilateral submandibular and upper deep cervical regions were also noted http://dx.doi.org/10.1016/j.ajoms.2017.01.008 2212-5558/© 2017 Asian AOMS, ASOMP, JSOP, JSOMS, JSOM, and JAMI. Published by Elsevier Ltd. All rights reserved.