2023 Volume 69 Issue 4 Pages 311-317
Rhabdomyosarcoma is the most frequent soft tissue malignant tumor among children. The head and neck region, including the paraspinal and orbital areas, is regarded as the predominant site of rhabdomyosarcoma, and its occurrence in the hypopharynx is very rare. We herein report an adult case of alveolar rhabdomyosarcoma arising in the hypopharynx. The patient was a 64-year-old man presenting with a tumor in the left piriform sinus of the hypopharynx. A biopsy of the tumor failed to identify the histological type. He underwent total laryngopharyngectomy with cervical esophagectomy and free jejunal reconstruction. The pathological diagnosis was alveolar rhabdomyosarcoma. Adjuvant chemotherapy with vincristine, actinomycin D, and cyclophosphamide (VAC) was administered, and he survived more than three years after the surgery. Recently, TP53 and MYOD1 mutations as well as the FOXO1 fusion gene have been reported as poor prognostic factors. Further risk stratification, including the identification of molecular pathological features and individualization of treatment methods, are expected to improve the prognosis of adult patients with rhabdomyosarcoma.
