Pleomorphic leiomyosarcoma of gingivoalveolar sulcus: a case report

Leiomyosarcomas are rare tumors in the oral cavity. Oral leiomyosarcomas may arise primarily from bones (maxilla and mandible) or soft tissues. Pleomorphic leiomyosarcoma (PLMS) is a recently described morphologic variant with aggressive clinical behavior and low survival rate; it is exceptionally rare at intraoral sites.

A 21-year-old male presented with a rapidly progressive growth arising from the gingiva. Fine needle aspiration cytology showed pleomorphic polygonal, plasmacytoid and spindled cells. Immunocytochemistry (IC) showed diffuse positivity for vimentin, desmin and cytokeratin, suggestive of high grade sarcoma. The patient underwent surgery, and total extended maxillectomy was performed; the diagnosis was PLMS on histology and IC. Further, detailed clinical and radiologic findings suggested primary high grade soft tissue sarcoma involving the underlying maxillary bone; thus, the final diagnosis was primary PLMS of the gingivoalveolar sulcus.

Intraoral PLMS arising from gingivoalveolar sulcus in a young patient is an extremely rare lesion with aggressive behavior. Detailed clinicoradiologic findings, its correlation with morphology and IC are mandatory for its final diagnosis and also to exclude metastasis from other sites and primary tumor in bone.