直肠碰撞瘤临床病理观察  

Clinicopathologic features of gastrointestinal schwannomas coexisting extranodal marginal zone lympoma of mucosa associated lymphoid tissue lymphoma of rectum

作　　者：崔华娟[1] 王卓才[1] 赖日权[1] 彭大云[1] 陈敬文[1] 

机构地区：[1]广州军区广州总医院病理科,广州510010

出　　处：《诊断病理学杂志》2013年第12期767-770,共4页Chinese Journal of Diagnostic Pathology

摘　　要：目的提高对直肠碰撞瘤的认识。方法对1例直肠胃肠神经鞘瘤与黏膜相关淋巴组织结外边缘区B细胞性淋巴瘤(MALT)形成的碰撞瘤,进行组织形态学观察及免疫组化检测,并复习相关文献。结果患者女性,65岁。体检发现下腹部肿块。CT提示直肠右侧壁可见2.8 cm×1.9 cm大小软组织影,邻近直肠管腔受压变窄。在直肠肿瘤切除标本中,同一肿物内可见胃肠神经鞘瘤和MALT淋巴瘤2种成分。2种肿瘤成分界限清楚,形成碰撞瘤,其中淋巴瘤成分包裹神经鞘瘤。神经鞘瘤S-100、PDG9.5、nestin和GFAP(-);淋巴瘤LCA、CD20和CD79α(+),CD3和CD45RO(-)。基因重排显示B细胞单克隆性增生。IgH/MALT1 t(14;18)(q32;q21)及API2/MALT1 t(11;18)(q21;q21)染色体异位(-)。c-kit及PDGFRA基因均为野生型。结论直肠胃肠神经鞘瘤与淋巴瘤形成的碰撞瘤罕见。应与胃肠间质瘤及淋巴组织反应性增生鉴别。Objective To improve the knowledge of rectal collision tumor. Methods A case of gastrointestinal schwannomas coexisting with extranodal marginal zone lympoma of mucosa associated lymphoid tissue lymphoma（ MALT）of rectum was observed by using HE stain and immunohistochemistry with review of the related literature. Results A 65- year-old woman was found a hypogastric lump in the physical check-up. CT showed a 28 mm × 19 mm shadow of rectal dextral wall,and adjacent rectum cavity was narrowed and pressed. There were two components in the tumor of the colorectal resection specimen including gastrointestinal schwannomas and MALT. Though coexistent,the two tumor phenotypes respected their boundaries with no diffuse intermingling or transition between them,and lympoma element enwrapped schwannomas. The schwannomas strongly expressed the nerve markers such as S-100 protein,PGP9. 5,nestin and GFAP,the lymphoma revealed positivity with antibodies to B cell such as LCA,CD20,CD79α whereas CD3 and CD45RO were negative. Gene rearrangement revealed B cell single clonality proliferation. FISH detected IgH / MALT1 t（ 14; 18）（ q32; q21） and API2/MALT1 t（ 11; 18）（ q21; q21） negative. Gene sequencing showed C-Kit gene and PDGFRA gene all were wild type. Conclusions Collision tumors of the intestinal tract are rare. To the best of our knowledge,there is no report of a rectal collision tumor composed of gastrointestinal schwannomas and lymphoma which must be distinguished from gastrointestinal stromal tumor and lymph node reactive hyperplasia. Our report highlights the need for pathologists,surgeons,and oncologists to be aware of the rare possibility of collisions tumors. Clinical awareness and recognition of such tumors are important as they will dictate appropriate treatment strategies.

关 键 词：直肠肿瘤 碰撞瘤 胃肠神经鞘瘤 淋巴瘤 

分 类 号：R733.4[医药卫生—肿瘤]