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Background  Leiomyosarcoma of the head and neck is a rare cancer with high local aggressiveness. Radical surgery and adjuvant treatment offer the best chance for cure, nonetheless 5-years recurrence rate remains high. Despite international guidelines are available for soft tissue sarcomas, no recommendations are specifically endorsed for leiomyosarcoma of the head and neck, due to the rarity of its presentation and consequently scarcity of data on long-term outcome.    Case presentation  A 50-year old woman, operated 10 years before for leiomyosarcoma of the nasal pit and with negative 5-years follow-up, was admitted to our ward for impairment of the hepatic function. Total-body CT scan detected multiple localizations at lungs, kidneys, pancreas, bones, muscles, lymph nodes and thyroid. The pathologic report after lung biopsy confirmed the diagnosis of metastasis from leiomyosarcoma and the patients was scheduled for first line chemo-radiotherapy.    Conclusions  Despite adequate primary treatment, distant and disseminated metastatic disease may be not excluded in leiomyosarcoma of the head and neck.

clinical sarcoma research

Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease