Malignant mesothelioma with heterologous elements: clinicopathological correlation of 27 cases and literature review
Malignant mesothelioma with heterologous elements: clinicopathological correlation of 27 cases and literature review
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DOI：
10.1038/modpathol.2008.125
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发表时间：
2008-09-01
期刊：
影响因子：
7.5
通讯作者：
Roggli, Victor L.
中科院分区：
文献类型：
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作者：
Klebe, Sonja;Mahar, Annabelle;Roggli, Victor L.
只有少数恶性间皮瘤与异源成分已被描述。目前还没有诊断标准，关于预后的数据也很少。我们建议术语异种性间皮瘤应保留为具有恶性异质性成分的肿瘤，特别是骨肉瘤、软骨肉瘤或横纹肌肉母细胞成分，但具有间皮瘤的免疫组织化学和临床特征。我们确定了27个这样的病例，并描述了这些肿瘤的临床和病理特征。在我们的系列中，89%起源于胸膜，11%起源于腹膜。确诊时的中位年龄为68岁，从27岁到85岁不等。在这些病例中，93%发生在男性，7%发生在女性。27例间皮瘤中，肉瘤样16例(59%)，双相10例(37%)，上皮样1例；40%(11例)仅显示骨肉瘤成分，19%(19%)仅显示横纹肌肉瘤区域，19%(19%)仅显示软骨肉瘤区域，22%(22%)显示骨软骨瘤成分。免疫组织化学标记的细胞角蛋白存在于大多数病例中。在所有17例有暴露史的病例中，均已确定暴露于石棉(63%)。中位生存期为确诊后6个月，与肉瘤样间皮瘤的生存期相似。鉴别诊断包括原发性和继发性胸膜肉瘤，包括骨肉瘤和软骨肉瘤。免疫组织化学标记细胞角蛋白有助于鉴别，但在梭形细胞/肉瘤样瘤中缺乏细胞角蛋白标记并不排除间皮瘤的诊断，无论是否存在异源成分。我们建议，如果解剖分布符合间皮瘤，则无论细胞角蛋白阳性与否，异种性间皮瘤的诊断应优先于原发性胸膜骨肉瘤或软骨肉瘤，就像传统的非异质性肉瘤样间皮瘤一样。
Only a small number of malignant mesotheliomas with heterologous elements have been described. There are currently no criteria for diagnosis and little data regarding prognosis. We suggest that the term heterologous mesothelioma should be reserved for tumours that show malignant heterologous elements, notably osteosarcomatous, chondrosarcomatous, or rhabdomyoblastic elements but have immunohistochemical and clinical characteristics of mesothelioma. We identified 27 such cases and characterized the clinical and pathological characteristics of these tumours. In our series, 89% originated in the pleura, and 11% from the peritoneal cavity. The median age at diagnosis was 68 years, ranging from 27 to 85 years. Of these cases, 93% occurred in males and 7% in women. Of the 27 mesothelioma cases 16 (59%) were sarcomatoid, 10 (37%) were biphasic, and one was reported as epithelioid; 40% ( 11 cases) showed osteosarcomatous elements only, 19% showed areas of rhabdomyosarcoma only, 19% contained areas of chondrosarcoma only, and 22% exhibited osteochondromatous elements. Immunohistochemical labelling for cytokeratins was present in the majority of cases. Exposure to asbestos was identified in all the 17 cases for which an exposure history was available (63%). Median survival was 6 months after diagnosis, similar to the survival seen in sarcomatoid mesotheliomas. The differential diagnosis includes primary and secondary pleural sarcomas, including osteosarcomas and chondrosarcomas. Immunohistochemical labelling for cytokeratins is helpful in the distinction, but lack of labelling for cytokeratins in a spindle cell/sarcomatoid tumour does not exclude the diagnosis of mesothelioma, irrespective of the presence of heterologous elements. We suggest that if the anatomical distribution conforms to that of mesothelioma, a diagnosis of heterologous mesothelioma should be made in preference to a diagnosis of primary pleural osteosarcoma or chondrosarcoma, regardless of cytokeratin positivity, as for conventional non-heterologous sarcomatoid mesothelioma.