Boldorini R, Tosoni A, Leutner M, Ribaldone R, Surico N, Comello E, Min K W
Dipartimento di Scienze Mediche Facoltà di Medicina e Chirurgia, Università Amedeo Avogadro del Piemonte Orientale, Novara, Italy.
Pathology. 2001 Aug;33(3):390-5.
Neurofibromatosis type 1 could be associated with multiple gastrointestinal stromal tumours, although their presence is not considered among the major diagnostic criteria. We present here a case of a 50-year-old female complaining of abdominal pain, with about 100 small intestinal stromal tumours. This finding prompted us to suspect a neurofibromatosis which was clinically confirmed afterwards. Light microscopy examination revealed a low-grade stromal tumour with skeinoid fibres. Mixed neural-interstitial cells of Cajal origin or, alternatively, neural differentiation of interstitial cells of Cajal are discussed on the basis of immunophenotype (CD117+, CD34+) and ultrastructure. A 2-year follow-up did not indicate an aggressive course in the case of this neoplasm.
1型神经纤维瘤病可能与多发胃肠道间质瘤相关，尽管其存在并不被视为主要诊断标准之一。我们在此报告一例50岁女性，主诉腹痛，发现约有100个小肠间质瘤。这一发现促使我们怀疑患有神经纤维瘤病，随后经临床确诊。光镜检查显示为伴有束状纤维的低度间质瘤。基于免疫表型（CD117 +、CD34 +）和超微结构，对起源于Cajal的混合神经 - 间质细胞或Cajal间质细胞的神经分化进行了讨论。两年的随访表明该肿瘤并无侵袭性病程。
