Affiliation:
1. Department of Oncopathology, The Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India
2. Department of Community Oncology, The Gujarat Cancer and Research Institute, Ahmedabad, Gujarat, India
Abstract
Abstract
Introduction Sarcomas are a heterogeneous group of malignancies originating from the transformed cells of mesenchymal lineage, representing only 1% of all the primary tumors arising within the head and neck region with a prevalence range of 4 to 10%. They are biologically different from the sarcomas of other sites because of the smaller size of the tumor at the time of presentation and higher chances of local recurrences. Due to the rarity in adults, there are very few studies on sarcomas of the head and neck. The aim of this study was to analyze the frequency and spectrum of primary sarcomas in the head and neck region.
Materials and Methods This is a retrospective descriptive study. The authors analyzed 40 cases of histologically and immunohistochemically proven primary sarcomas arising in the head and neck region according to three age groups, pediatric (<12 years), adolescent (12–19 years), and adults (> 19 years).
Results Primary sarcomas represented 0.06% and 2.4% of total cancer cases and total sarcoma cases, respectively. Embryonal rhabdomyosarcoma was the most common histologic type, and nasopharynx the most common location in the pediatric population. Osteoblastic osteosarcomas and Ewing’s sarcoma were typical variants in adolescent age, affecting the hemimandible predominantly. In adults, osteosarcomas were commonest followed by conventional grade 1 chondrosarcoma. Treatment was planned according to the stage and grade of the tumor. Out of the 40 patients, 19 (47.5%) had a disease relapse within a period of 6 months from the completion of therapy.
Conclusion Head and neck sarcomas are notorious to recur. A timely and accurate diagnosis, proper staging, and multidisciplinary treatment plan can yield good patient outcomes.