Pinotti Enrico, Sandini Marta, Famularo Simone, Jaconi Marta, Romano Fabrizio, Nespoli Luca, Gianotti Luca
Department of Surgery, School of Medicine and Surgery, San Gerardo Hospital (4° Piano A), University of Milano Bicocca, Via Pergolesi 33, 20900 Monza, Italy.
Unit of Pathology, School of Medicine and Surgery, San Gerardo Hospital, University of Milano Bicocca, Monza, Italy.
Clin Sarcoma Res. 2017 Jun 6;7:12. doi: 10.1186/s13569-017-0078-2. eCollection 2017.
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.
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.
Despite adequate primary treatment, distant and disseminated metastatic disease may be not excluded in leiomyosarcoma of the head and neck.
头颈部平滑肌肉瘤是一种罕见的癌症，具有高度局部侵袭性。根治性手术和辅助治疗提供了最佳的治愈机会，尽管如此，5年复发率仍然很高。尽管有国际软组织肉瘤指南，但由于头颈部平滑肌肉瘤病例罕见，长期预后数据稀缺，因此没有专门针对头颈部平滑肌肉瘤的推荐。
一名50岁女性，10年前因鼻窝平滑肌肉瘤接受手术，5年随访结果为阴性，因肝功能损害入住我们病房。全身CT扫描发现肺部、肾脏、胰腺、骨骼、肌肉、淋巴结和甲状腺有多处病变。肺活检后的病理报告证实为平滑肌肉瘤转移，该患者被安排进行一线放化疗。
尽管进行了充分的初始治疗，但头颈部平滑肌肉瘤仍可能出现远处和播散性转移性疾病。
