Int Arch Otorhinolaryngol 2014; 18 - a2198
DOI: 10.1055/s-0034-1388763

Embryonal Rhabdomyosarcoma of Temporal Bone: Case Report

Luciana Carolina Peruzzo 1, André Toshio Matsuda 1, Felipe Horst 1, João Marcelo Caldeira Fabiano 1, Ricardo Borges 1, Rosana Heshiki 1
  • 1Hospital Universitário Regional do Norte do Paraná

The rhabdomyosarcoma is a rare malignancy that has its origin in the same primitive mesenchymal cells that originate skeletal muscle tissue. It corresponds to approximately 10% of cases of soft-tissue sarcomas of the head and neck in adults, but is more common in children, reaching up to 50%. When it affects the temporal bone, it can be characterized by various signs and symptoms, such as polypoid tumor in the external auditory canal, otorrhagia, inflammation, or facial paralysis. Histologically, can be divided into embryonic (and its variant botryoid), alveolar, and pleomorphic. The embryonal subtype is most common in children and is usually less aggressive in this group, with a predilection for the orbit and middle-ear segment of the head and neck. Pleomorphic subtype is almost exclusive to adults. The prognosis of rhabdomyosarcoma depends on the tumor size, primary tumor, and invasion of structures. The radiation therapy plus chemotherapy associated with surgical resection has a high cure rate in children. This case reports a female patient who had diagnosed embryonal rhabdomyosarcoma at 9 years old. It began with facial paralysis associated with unilateral otorrhagia and soft tumor extruding the right external auditory meatus. The diagnosis was confirmed by imaging associated with biopsy and immunohistochemistry, having satisfactory response to treatment with chemotherapy and radiation therapy.