Scattone A, Pennella A, Gentile M, Musti M, Nazzaro P, Buonadonna A L, Marzullo A, Cavone D, Pollice L, Serio G
Department of Pathology, Medical School, University of Bari, Italy.
J Clin Pathol. 2006 Jul;59(7):764-9. doi: 10.1136/jcp.2005.026435. Epub 2006 Mar 28.
Malignant deciduoid mesothelioma is a rare variant of epithelioid mesothelioma. This tumour generally has poor prognosis, and can be asbestos related.
To identify peculiar genetic changes responsible for critical phases in pathogenesis of malignant deciduoid mesothelioma and their prognostic relevance.
Comparative genomic hybridisation was carried out in six cases of malignant pleural deciduoid mesothelioma, four sporadic and two familial. All cases were found to be asbestos related. Four patients died during follow-up and the mean survival was 29.5 (SD 14.2, range 12-43) months.
Genetic abnormalities were found in all the tumour tissues, the most frequent being chromosomal gains at 1p, 12q, 17, 8q, 19 and 20 and losses at 13q, 6q and 9p. Survival was found to be longer in those patients who presented a smaller number of losses (< or =2) in the tumorous chromosomes.
Although numerous genetic changes are presented by deciduoid mesotheliomas, certain chromosomal regions are preferentially affected. The clinical outcome for this mesothelioma subtype is predicted by the number of losses.
恶性蜕膜样间皮瘤是上皮样间皮瘤的一种罕见变异型。该肿瘤通常预后较差，且可能与石棉有关。
确定导致恶性蜕膜样间皮瘤发病关键阶段的特殊基因变化及其预后相关性。
对6例恶性胸膜蜕膜样间皮瘤进行比较基因组杂交分析，其中4例为散发性，2例为家族性。所有病例均被发现与石棉有关。4例患者在随访期间死亡，平均生存期为29.5（标准差14.2，范围12 - 43）个月。
在所有肿瘤组织中均发现基因异常，最常见的是1p、12q、17、8q、19和20号染色体的增加以及13q、6q和9p号染色体的缺失。发现肿瘤染色体中缺失数量较少（≤2个）的患者生存期较长。
尽管蜕膜样间皮瘤存在众多基因变化，但某些染色体区域更易受到影响。该间皮瘤亚型的临床结局可通过缺失数量来预测。
