An Unusual Presentation of Leiomyosarcoma Posing a Diagnostic Dilemma: A Case Report

Abstract

Leiomyosarcoma (LMS) is a malignant tumour of smooth muscle origin commonly seen in genital and gastrointestinal location. However, its presence in the head and neck region in a young child is extremely rare. Authors present a unique case of LMS in a one year and five-month-old child who presented with a painless mass in the right temporal region of the head. Histopathological examination revealed a highly cellular tumour arranged in intersecting fascicles of spindle cells with brisk mitotic activity and interspersed areas of necrosis. On Immunohistochemistry (IHC), the tumour cells were positive for Smooth Muscle Actin (SMA), Desmin and Vimentin and negative for S100 and Myogenin. A diagnosis of LMS was thus, made. Head and neck sarcoma is a broad entity encompassing plethora of differentials with closely overlapping morphological features which renders them diagnostically challenging, this can be resolved by employing various immunohistochemical stains readily available in all laboratories. This case highlights the combined role played by histopathology and immunohistochemistry in arriving at the correct diagnosis. To the best of our knowledge, this is the first case of LMS reported in the temporal region of head and is distinct with respect to its rare incidence, location and age at presentation. LMS at this site may masquerade as deceptively benign painless mass and may not be suspected initially, however one should bear in mind that these are moderate-to-high grade tumours and any delay in management may portend poor prognosis. Timely and aggressive surgical management is thus, the mainstay of treatment and critical to patient survival.

Keywords

• child
• mass
• temporal region