Desmoplastic Small Round Cell Tumor of the Head and Neck: A Potential Diagnostic Pitfall

Abstract

Primary desmoplastic small round cell tumor (DSRCT) in the head and neck region is extremely rare. There is limited information about its clinicopathological characteristics, prognosis, and treatment modalities. The purpose of this study is to provide a comprehensive review of DSRCT occurring primarily in the head and neck, to demonstrate its peculiar morphology and immunohistochemical expression, and to address the differential diagnoses. A total of 25 cases were collected after a thorough review of the relevant literature. DSRCT was most frequently reported in the major salivary glands, followed by the eyes. Furthermore, some cases were misinterpreted as poorly differentiated carcinoma, Ewing sarcoma, and olfactory neuroblastoma. Diagnosing DSRCTs in the head and neck can be very challenging due to their rarity in this location, overlapping morphology, and immunohistochemistry. In these cases, following a systemic approach helps to solve diagnostic problems.

Multifocal Desmoplastic Small Round Cell Tumor: A Case Report of a Rare Neoplasm

“…DSRCTs in the head and neck are very uncommon, orbital involvements being extremely rare. Less than 10 such cases are discussed in the current literature [5][6][7][8]. Due to the highly aggressive nature of the disease and the short duration of history, it is difficult to locate the primary site of origin of the tumor [2].…”