Hyperostosis in orbital rhabdomyosarcoma

Abstract

Rhabdomyosarcoma is the most common soft-tissue sarcoma in paediatric patients and may arise as a primary orbital neoplasm. Imaging studies show a unilateral solitary orbital lesion. With larger tumours, erosion of the adjacent orbital walls is a common accompanying bony change. We present an unusual case of rhabdomyosarcoma in a preschool girl with proptosis and temporal bossing. Imaging studies showed a homogeneous, well-delineated, extraconal mass of the right orbit with hyperostosis of the adjacent sphenoid bone. After excision of the mass and debulking of the sphenoid bone, histopathology showed embryonal rhabdomyosarcoma with no malignant infiltration of the adjacent bone. This is the first reported case of hyperostosis associated with rhabdomyosarcoma. Periosteal reaction is the proposed underlying mechanism.

Late dental and bone alterations in patients after orbital rhabdomyosarcoma treatment

“…[12] In imaging studies of orbital rhabdomyosarcoma, there is usually a single unilateral orbital lesion. In larger tumors, there may be bone alteration, such as erosion of the adjacent orbital walls [13], most commonly in the retrobulbar region. [8][9][10][11][12][13][14] When bone erosion is present, the primary site of tumor is considered parameningeal.…”

“…[14] Despite technological advances, children with orbit rhabdomyosarcoma submitted to multimodal treatment (surgery, chemotherapy and radiotherapy) still present signi cant alterations in teeth and facial bones in development. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18][19][20][21] In this scenario, the most prevalent dental changes are root shortening (14.6%), agenesis (14.3%), and microdontia (13.6%). Risk factors have been associated with these alterations such as younger age at diagnosis (< 3 years), irradiated area, and dose-dependent alkylating agent therapy.…”