鼻腔より発生した横紋筋肉腫の一剖検例

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Rhabdomyosarcoma is rare in the head and neck region. Although combinations of surgery, radiotherapy and chemotherapy have improved the survival rate in recent years, the prognosis for patients with rhabdomyosarcoma in the nasal cavity is still poor.A 16-year-old male with alveolar type rhabdomyosarcoma originating in the right nasal cavity visited a hospital for the evaluation of rapidly growing right exophthalmos, nasal obstruction and bloody nasal secretion. Roentogenographic examination revealed a large right nasal mass with ipsilateral orbital and intracranial invasion. The first biopsy specimen from the nasal mass was pathologically suspected to be olfactory neuroblastoma.He was treated first with radical surgery, combined with frontal craniotomy. The pathological diagnosis of the resected specimen was rhabdomyosarcoma after immunohistochemical and electronmicroscopic examinations. One month after the radical operation, the intracranial tumor recurred. During radiotherapy for the intracranial tumor, there was an abrupt onset of leucocytopenia and thrombocytopenia and pathological evidence of bone marrow invasion. Although the hematological and radiological abnormalities responded to combination chemotherapy with mitoxantrone, etoposide, cisplatinum and prednisolone, cytological abnormality of the spinal fluid continued. Extra high dose general treatment with methotrexate in combination with weekly intradural injection of methotrexate and steroid were only partially effective, and he died of respiratory failure due to brain stem involvement. Autopsy revealed no residual or metastastic lesions in the head and neck or other organs except for the central nervous system.
耳鼻咽喉科臨床学会の論文

鼻腔より発生した横紋筋肉腫の一剖検例

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