Montgomery E, Goldblum J R, Fisher C
Department of Pathology, The Johns Hopkins Hospital, Baltimore, MD, USA.
Histopathology. 2002 Jun;40(6):518-25. doi: 10.1046/j.1365-2559.2002.01412.x.
The behaviour of leiomyosarcoma is site-related and there are few data on such tumours located in the head and neck. We studied the clinicopathological features of these lesions.
Cases diagnosed as leiomyosarcoma of the head and neck were retrieved from the archives of three institutions. Immunohistochemistry was performed and follow-up information was obtained. There were seven men and six women, aged 21-73 years, and lesions involved the neck (n=3), maxilla (n=4), buccal area (n=3), and maxillary sinus, nose, and pharynx (n=1 each). Tumours ranged from 10 to 80 mm. All tumours showed at least focally typical histological features of leiomyosarcoma with perpendicularly arranged fascicles of smooth muscle cells with blunt-ended nuclei, eosinophilic cytoplasm and paranuclear vacuoles. They expressed muscle-specific actin (8/9), smooth muscle actin (7/9), and desmin (10/12). Follow-up information was available on nine patients. All had surgery, with radiation and/or chemotherapy in seven. Three (27%) recurred at 6-24 months; in one case twice. Five (56%) had metastases, including the three with prior recurrences at 1-128 months. Five (including two who received adjuvant therapy) were disease-free at a median of 47 months, one was alive with metastatic disease at 24 months), and three were dead of disease (median 13 months).
Head and neck leiomyosarcomas are rare and aggressive neoplasms which metastasize. Adjuvant therapy has limited effect.
平滑肌肉瘤的行为与部位相关，而关于头颈部此类肿瘤的数据较少。我们研究了这些病变的临床病理特征。
从三个机构的档案中检索出诊断为头颈部平滑肌肉瘤的病例。进行了免疫组织化学检测并获取了随访信息。患者共13例，男性7例，女性6例，年龄21 - 73岁，病变累及颈部（3例）、上颌骨（4例）、颊部（3例）以及上颌窦、鼻腔和咽部（各1例）。肿瘤大小为10至80毫米。所有肿瘤至少局灶性地表现出平滑肌肉瘤典型的组织学特征，即平滑肌细胞束垂直排列，核钝圆，胞质嗜酸性，核旁有空泡。它们表达肌特异性肌动蛋白（8/9）、平滑肌肌动蛋白（7/9）和结蛋白（10/12）。9例患者有随访信息。所有患者均接受了手术，7例接受了放疗和/或化疗。3例（27%）在6至24个月复发，其中1例复发两次。5例（56%）发生转移，包括3例先前在1至128个月复发的患者。5例（包括2例接受辅助治疗的患者）在中位时间47个月时无疾病，1例在24个月时带转移灶存活，3例死于疾病（中位时间13个月）。
头颈部平滑肌肉瘤是罕见且具有侵袭性的转移性肿瘤。辅助治疗效果有限。
