ABSTRACT
Sclerosing/spindle cell rhabdomyosarcoma (s-scRMS) is a rare variant of striated muscle tumours. It has been recognised as an individual entity, the fourth subtype of rhabdomyosarcoma in the latest WHO classification. In the paediatric population, it occurs more commonly in the paratesticular area, whereas in adults, it occurs more commonly in the head and neck region. It has distinctive characteristics in terms of its histopathological and immunochemistry findings, which help in accurate diagnosis. The mainstay of treatment is a multimodal approach, i.e., surgery, chemotherapy, and radiation therapy. However, no standard care is still being established internationally for adult cases. In adults, this tumour has a poorer prognosis as compared to children. We describe a patient with s-scRMS of the lower limb who has undergone wide local resection of the tumour with surgical reconstruction of the distal tibiofibular joint with autograft and its two-year outcome.
