Schröder S, Stengel B, Radtke A, Kleemann D
HNO-Klinik des MediClin-Müritz-Klinikums, Weinbergstrasse 19, 17192, Waren/Müritz, Deutschland.
HNO. 2009 Dec;57(12):1311-6. doi: 10.1007/s00106-009-1975-3.
Myofibroblastic sarcomas or myofibrosarcoma, are extremely rare malignant neoplasms of myofibroblasts. They are characterized by the pattern of cells and special immunohistochemical markers such as vimentin, desmin and alpha-smooth-muscle actin.
The case of a patient with a history of frequently relapsing papillomas of the larynx is reported. Chronic laryngitis with focal low-grade dysplasia of the squamous epithelium was diagnosed approximately 1 year after the first treatment of the papillomas. After approximately 2 years the pathologist diagnosed the rare myofibroblastic sarcoma of the larynx. The patient underwent laryngectomy due to the spread of the tumor with a bilateral selective neck dissection. The patient is at present still free of recurrence and metastases.
There is a great danger of misjudging a myofibroblastic sarcoma as an inflammatory myofibroblastic tumor and consequently to delay the urgently needed treatment. Therefore, an overview of the present state of knowledge about diagnosis and treatment of myofibroblastic sarcomas will be given based on this case report.
肌成纤维细胞肉瘤或肌纤维肉瘤是极其罕见的肌成纤维细胞恶性肿瘤。它们以细胞形态模式及波形蛋白、结蛋白和α - 平滑肌肌动蛋白等特殊免疫组化标志物为特征。
报告1例有喉部乳头状瘤频繁复发病史的患者。在首次治疗乳头状瘤约1年后,诊断为慢性喉炎伴鳞状上皮局灶性低度发育异常。约2年后,病理学家诊断为罕见的喉部肌成纤维细胞肉瘤。由于肿瘤扩散,患者接受了喉切除术及双侧选择性颈部淋巴结清扫术。该患者目前仍无复发和转移。
将肌成纤维细胞肉瘤误诊为炎性肌纤维母细胞瘤并因此延误急需治疗的风险很大。因此,基于本病例报告,将对肌成纤维细胞肉瘤的诊断和治疗的现有知识概况进行阐述。