title: Multiple small intestinal stromal tumours in a patient with previously unrecognised neurofibromatosis type 1: immunohistochemical and ultrastructural evaluation.
abstract: 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.
authors.affiliation: Dipartimento di Scienze Mediche Facoltà di Medicina e Chirurgia, Università Amedeo Avogadro del Piemonte Orientale, Novara, Italy. anapatol@starnova.it
authors.forename: R
authors.initials: R
authors.lastname: Boldorini
authors.forename: A
authors.initials: A
authors.lastname: Tosoni
authors.forename: M
authors.initials: M
authors.lastname: Leutner
authors.forename: R
authors.initials: R
authors.lastname: Ribaldone
authors.forename: N
authors.initials: N
authors.lastname: Surico
authors.forename: E
authors.initials: E
authors.lastname: Comello
authors.forename: K W
authors.initials: KW
authors.lastname: Min
mesh_terms: D018952:Antigens, CD34 / Q000032:analysis; D014408:Biomarkers, Tumor / Q000032:analysis; D004064:Digestive System / Q000294:innervation / Q000473:pathology; D005260:Female; D006801:Humans; D016130:Immunophenotyping; D007414:Intestinal Neoplasms / Q000737:chemistry / Q000150:complications / Q000473:pathology*; D008854:Microscopy, Electron; D008875:Middle Aged; D009130:Muscle, Smooth / Q000294:innervation / Q000473:pathology; D009197:Myenteric Plexus / Q000473:pathology; D009456:Neurofibromatosis 1 / Q000150:complications / Q000378:metabolism / Q000473:pathology*; D019009:Proto-Oncogene Proteins c-kit / Q000032:analysis*
chemical_list: D018952:Antigens, CD34; D014408:Biomarkers, Tumor; D019009:Proto-Oncogene Proteins c-kit