Abstract
Orbital rhabdomyosarcoma is the commonest primary orbital malignancy in children. A case of alveolar type of orbital rhabdomyosarcoma with unusual presentation in a three and half years male child presented with painless nodular swelling in the left lower lid of one and half years duration and gradual protrusion of the left eyeball. Examination of the patient revealed a mass in the left lower lid region extending to other parts of orbit and lymphnode metastasis in the neck region along with non axial proptosis, rest of the systemic examination being normal. Patient underwent incisional biopsy of the mass and excisional biopsy of the lymphnodes which revealed alveolar type of primary orbital rhabdomyosarcoma with lymphnode metastasis.
Highlights
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children
Weber first described rhabdomyosarcoma in 1854; histological definition was available in 1946, when Stout recognized the distinct morphology of rhabdomyoblasts
Orbital Rhabdomyosarcoma was first reported by Bayer.[3]
Summary
Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children. Bastola et al An Unusual Presentation of Alveolar Type of Rhabdomyosarcoma of Orbit size were present in submandibular region, few were matted, local temperature of the lymph nodes was not raised, and lymph nodes were not attached with overlying skin but were immobile. The section from cervical lymphnode showed almost complete effacement of nodal architecture replaced by tumor cells proliferation was similar to that seen in orbital mass (Figure 4).
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