Answer
Unlike other regions of the body where rhabdomyosarcoma can occur, the head and neck region is limited by anatomic constraints; therefore, surgical treatment in this area must be altered accordingly. For superficial nonorbital lesions, wide excision is recommended. A cuff of normal tissue is always resected with the neoplastic tissue; however, narrow margins are accepted because of the anatomic restrictions.
Rhabdomyosarcoma of the orbit does not require surgical exenteration at the time of initial resection. Initial surgery is aimed only at biopsy for diagnosis. Exenteration is reserved for recurrent or locally persistent disease. If evidence suggests microscopic residual disease after initial surgical resection of head and neck rhabdomyosarcoma, a second operation with wide margins before chemotherapy may also improve the prognosis.
Surgery plays a limited role in the treatment of metastatic disease. It is indicated only in the context of persistent pulmonary metastases after chemotherapy and irradiation, and it is considered an option only if pulmonary function can be adequately maintained.
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Embryonal rhabdomyosarcoma is evidenced by a variable cell population consisting of small, round tumor cells with hyperchromatic nuclei and of large, polygonal-shaped tumor cells with abundant eosinophilic cytoplasm, which often contains diagnostic cross striations (arrow). Image provided by Scott Kilpatrick, MD, Department of Pathology, University of North Carolina Hospitals.
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Alveolar rhabdomyosarcoma is evidenced by uniform cell population consisting of cells with a high nuclear-to-cytoplasmic ratio. The cells are arranged in variably sized nests separated by fibrous tissue septa. In places, the cells appear loosely dispersed, mimicking a pulmonary alveolar pattern. Image provided by Scott Kilpatrick, MD, Department of Pathology, University of North Carolina Hospitals.
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Axial CT scan of rhabdomyosarcoma in the left middle ear. Image provided by Suresh Muhkerji, MD, Department of Radiology, University of North Carolina Hospitals.
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Axial CT scan of left orbital rhabdomyosarcoma. Image provided by Suresh Muhkerji, MD, Department of Radiology, University of North Carolina Hospitals.
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Axial CT scan of right masticator space rhabdomyosarcoma. Image provided by Suresh Muhkerji, MD, Department of Radiology, University of North Carolina Hospitals.
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MRI of right masticator space rhabdomyosarcoma. Image provided by Suresh Muhkerji, MD, Department of Radiology, University of North Carolina Hospitals.
