Multiple small intestinal stromal tumours in a patient with previously unrecognised neurofibromatosis type 1: immunohistochemical and ultrastructural evaluation.
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.
Boldorini
R
R
Dipartimento di Scienze Mediche Facoltà di Medicina e Chirurgia, Università Amedeo Avogadro del Piemonte Orientale, Novara, Italy. anapatol@starnova.it
Tosoni
A
A
Leutner
M
M
Ribaldone
R
R
Surico
N
N
Comello
E
E
Min
K W
KW
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*
D018952:Antigens, CD34; D014408:Biomarkers, Tumor; D019009:Proto-Oncogene Proteins c-kit