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胸腺黏液表皮样癌。6例临床病理研究。

Mucoepidermoid carcinomas of the thymus. A clinicopathologic study of six cases.

作者信息

Moran C A, Suster S

机构信息

Department of Pulmonary & Mediastinal Pathology, Armed Forces Institute of Pathology, Washington, D.C. 20306-6000, USA.

出版信息

Am J Surg Pathol. 1995 Jul;19(7):826-34. doi: 10.1097/00000478-199507000-00011.

DOI:10.1097/00000478-199507000-00011
PMID:7793481
Abstract

Six cases of mucoepidermoid carcinoma of the thymus are presented. The patients were two men and four women aged 17 to 66 years (median age, 34.5). Clinically, three patients had symptoms of chest discomfort and three were asymptomatic. Grossly, the tumors in three patients were described as cystic structures varying in size from 7 to 8 cm in greatest dimension, with focal areas of induration within the walls of the cyst that averaged from 1.5 to 3 cm. The other three cases had grossly and radiographically well-circumscribed, homogeneous tumor masses. Histologically, the lesions showed a spectrum of features that ranged from those of well-differentiated, to moderately well-differentiated, to poorly differentiated mucoepidermoid carcinoma, with sheets and solid islands of squamoid cells admixed with mucin-secreting epithelium lining gland-like spaces. In four cases, the tumor was histologically seen in continuity with the epithelial lining of multilocular cystic structures; the nonneoplastic components of the cysts contained abundant inflammation and showed the features of otherwise conventional acquired multilocular thymic cysts. Clinical follow-up showed that the two patients with intermediate and high-grade tumors died within 2 and 7 months after initial diagnosis. One of these patients showed at autopsy residual tumor limited to the mediastinum, whereas the other patient died with metastases to pericardium and myocardium despite postoperative radiation therapy. Two patients with low-grade tumors were alive and well with no residual disease 2 and 3 years after surgery, and the other two were lost to follow-up. Mucoepidermoid carcinoma of the thymus should be included in the differential diagnosis of cystic neoplasms of the thymus. As with their counterparts at other sites, the biologic behavior of these tumors closely correlates with their degree of differentiation and amount of cytologic atypia.

摘要

本文报告6例胸腺黏液表皮样癌。患者包括2名男性和4名女性,年龄在17至66岁之间(中位年龄34.5岁)。临床上,3例患者有胸部不适症状,3例无症状。大体上,3例患者的肿瘤表现为囊性结构,最大直径为7至8厘米,囊壁有局灶性硬结区域,平均直径为1.5至3厘米。另外3例大体和影像学上表现为边界清晰、均匀的肿瘤肿块。组织学上,病变呈现出一系列特征,从高分化到中分化再到低分化黏液表皮样癌,有鳞状细胞片和实性岛,与分泌黏液的上皮混合,衬于腺样间隙。4例中,肿瘤在组织学上与多房囊性结构的上皮衬里连续;囊肿的非肿瘤成分有大量炎症,表现为其他方面典型的后天性多房胸腺囊肿特征。临床随访显示,2例中高级别肿瘤患者在初次诊断后2至7个月内死亡。其中1例尸检显示残留肿瘤局限于纵隔,而另1例患者尽管术后接受了放疗,但死于心包和心肌转移。2例低级别肿瘤患者术后2年和3年存活且情况良好,无残留疾病,另外2例失访。胸腺黏液表皮样癌应纳入胸腺囊性肿瘤的鉴别诊断。与其他部位的同类肿瘤一样,这些肿瘤的生物学行为与其分化程度和细胞异型性程度密切相关。

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