Histiocytic sarcoma of paranasal sinus; report of the first case with emphasis on potential diagnostic pitfalls and differential diagnosis

Abstract

Introduction: Histiocytic sarcoma is a very rare hematopoietic malignancy of mature histiocytes with an aggressive clinical course, arising as a primary neoplasm or less commonly as a secondary neoplasm from pre-existing indolent lymphoma or rarely after therapy for other neoplasms. Most patients present at advanced stage with metastasis and hence have poor prognosis. Case Report: A 70 year old female presented with difficulty in breathing and epistaxis. There was no pain, fever, weight loss and other systemic manifestations. On examination, there was a tumour in the nasopharynx. Based on the characteristic morphological features and an extensive panel of Immunohistochemistry markers, a diagnosis of histiocytic sarcoma was rendered. Discussion: It usually occurs in extra nodal sites and the most common locations being the gastrointestinal tract, skin, various soft tissue sites, liver and spleen. To the best of our knowledge, rare case arising in nasal cavity has been reported, but tumour from paranasal sinus has not been reported in literature, so far. We report the first case of Histiocytic sarcoma arising from Ethmoid in an elderly female, also discuss the differential diagnosis, potential pitfalls and highlight the importance of immunohistochemistry. © This is an open access article distributed under the terms of the Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/) which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.