Low-grade myofibroblastic sarcoma of the mandible: A rare tumour of childhood

Abstract

Low-grade myofibroblastic sarcoma is a rare and indolent tumour of soft tissue. This tumour is relatively common in the head and neck region followed by extremities. Primary low-grade myofibroblastic sarcoma of the mandible is very rarely reported and the occurrence of this tumour in a child is very unusual. A 7-year-old male child presented with a swelling in right angle of mandible. X-ray and computed tomography scan showed a lytic lesion. The lesion was excised and the tissue was sent for histopathological evaluation, which revealed a cellular spindle cell neoplasm arranged in fascicles. The tumour was partly circumscribed and lobulated. On immunohistochemistry (IHC), these tumour cells showed cytoplasmic positivity for vimentin, and smooth muscle actin showed ‘tram-track’ pattern of positivity. The case was diagnosed as low-grade myofibroblastic sarcoma. There are no definite clinical features or pathognomonic radiological appearances of this tumour that can differentiate this rare tumour from other commonly encountered gnathic bone tumours, such as osteosarcoma, inflammatory myofibroblastic tumour, etc., Histopathological diagnosis coupled with ancillary investigations such as IHC is important to establish a definite diagnosis and rule out the differentials. The exact biological behaviour of this tumour is not known.