Alveoloar Rhabdomyosarcoma in an adult presenting as a sino-orbital mass - a case report

Abstract

Rhabdomyosarcoma (RMS) is predominantly a childhood malignancy, seen rarely in adults. This is a case report of alveolar RMS involving the nasal cavity with               intraorbital extension. A 28 year old gentleman presented with proptosis of the left eye and nasal block for 1 month. Vision was 66 J1 in both eyes. There was proptosis of 5mm in the left eye with restricted adduction and elevation and choroidal folds suggestive of external compression of the globe. MR CT imaging showed a well-defined polypoidal soft tissue mass in the left nasal cavity extending supero-medially into the left orbit. A diagnostic biopsy was done endoscopically which was reported as alveolar rhabdomyosarcoma. After undergoing three cycles of chemotherapy, there was significant reduction in size of the nasal mass. The orbital part of the mass          completely disappeared after chemotherapy on repeat MR CT imaging. Adult RMS is a rare malignant tumor that calls for expert management. It is of paramount importance to             formulate a treatment plan on an individual basis after           considering the patients age, the stage of disease and the tumor size. Although it is found rarely in an adult, RMS should figure in the differential diagnosis of orbital tumors irrespective of age. Chemotherapy itself can bring down the size of the tumor drastically as in this case. Complete surgical removal of the tumor can be ensured in large tumors if surgery is             performed after chemoreduction.