Kazmierczak B, Rosigkeit J, Wanschura S, Meyer-Bolte K, Van de Ven W J, Kayser K, Krieghoff B, Kastendiek H, Bartnitzke S, Bullerdiek J
Center for Human Genetics and Genetic Counselling, University of Bremen, Germany.
Oncogene. 1996 Feb 1;12(3):515-21.
Pulmonary chondroid hamartomas (PCH) are benign tumors of the lung characterized by a more or less high degree of mesenchymal metaplasia. In our series we investigated 30 PCH by a combination of cytogenetic and molecular methods. 18 tumors (60%) had cytogenetically detectable aberrations involving either 12q14-15 or 6p21 with a clear predominance of chromosomal abnormalities involving 12q14-15 (15 tumors). As in subgroups of pleomorphic adenomas of the salivary glands, leiomyomas of the uterus, and lipomas with 12q14-15 abnormalities the HMGI-C gene is frequently rearranged we tested PCH with either 12q14-15 abnormalities or normal karyotype by FISH and 3' RACE experiments for rearrangements of HMGI-C. Rearrangements were found in all cases with chromosomal 12q14-15 abnormalities and further six cases with an apparently normal karyotype. By the combination of cytogenetics with molecular techniques the percentage of cases with intragenic rearrangements of HMGI-C or rearrangements of its immediate surrounding was thus increased to 70% (21/30 cases). Considering all types of aberrations within this series 80% (24/30) of all PCH were aberrant. This is the first report on a combined molecular and cytogenetic analysis of a large series of pulmonary chondroid hamartomas indicating that rearrangements of HMGI-C, a member of the high mobility group protein gene family, are the leading molecular events in the genesis of PCH.
肺软骨样错构瘤(PCH)是肺部的良性肿瘤,其特征为或多或少存在一定程度的间充质化生。在我们的研究系列中,我们通过细胞遗传学和分子方法相结合的方式对30例PCH进行了研究。18例肿瘤(60%)在细胞遗传学上可检测到涉及12q14 - 15或6p21的畸变,其中涉及12q14 - 15的染色体异常明显占优势(15例肿瘤)。正如在唾液腺多形性腺瘤、子宫平滑肌瘤和具有12q14 - 15异常的脂肪瘤亚组中一样,HMGI - C基因经常发生重排,我们通过荧光原位杂交(FISH)和3' 端快速扩增cDNA末端(3' RACE)实验对具有12q14 - 15异常或核型正常的PCH进行了HMGI - C重排检测。在所有染色体12q14 - 15异常的病例以及另外6例核型明显正常的病例中均发现了重排。通过细胞遗传学与分子技术相结合,HMGI - C基因内重排或其紧邻区域重排的病例百分比因此增加到了70%(21/30例)。考虑到该系列中的所有类型畸变,所有PCH中有80%(24/30)存在异常。这是关于大量肺软骨样错构瘤的分子和细胞遗传学联合分析的首篇报道,表明高迁移率族蛋白基因家族成员HMGI - C的重排是PCH发生过程中的主要分子事件。