naa a22 4500 510773664 CHVBK 20180411083223.0 cr unu---uuuuu 180411e20130601xx s 000 0 eng 10.1007/s12022-013-9239-x doi (NATIONALLICENCE)springer-10.1007/s12022-013-9239-x Neurofibromatosis 1 Presenting with Multiple Duodenal Gists Associated with a Somatostatin-Producing D Cell Neoplasm [Elektronische Daten] [Giovanni Serio, Clementina Zampatti, Andrea Ballabio, Riccardo Ricci, Maurizio Martini, Francesco Zurleni] The co-existence of a duodenal somatostatin-producing D cell neoplasm and multiple duodenal gastrointestinal stromal tumours (GISTs) in a 61-year-old woman with neurofibromatosis type 1 is reported. Histologically, the D cell neoplasm showed a glandular pattern with psammoma bodies and was metastatic to regional lymph nodes and liver at the time of surgery. Tumour cells were monomorph and showed intense and diffuse immunoreactivity for somatostatin, focal positivity for calcitonin, while were negative for other gastroenteropancreatic hormones including insulin, glucagon, pancreatic polypeptide, serotonin and gastrin. Four submucosal and subserosal GISTs, ranging from 5 to 15mm in diameter, were composed of uniform spindle-shaped cells lacking mitoses and contained numerous skeinoid fibres. The tumours were positive for CD117, DOG1, vimentin and CD34 and did not have KIT or PDGFRA mutations. The clinical and pathological importance of this unusual association is discussed. Springer Science+Business Media New York, 2013 Somatostatinoma nationallicence GIST nationallicence Neurofibromatosis 1 nationallicence Serio Giovanni Operative Unit of Anatomic Pathology, Azienda Ospedaliera "Ospedale di Circolo” di Busto Arsizio, Via A. da Brescia 1, 21052, Busto Arsizio, Italy aut Zampatti Clementina Operative Unit of Anatomic Pathology, Azienda Ospedaliera "Ospedale di Circolo” di Busto Arsizio, Via A. da Brescia 1, 21052, Busto Arsizio, Italy aut Ballabio Andrea Operative Unit of Surgery, Azienda Ospedaliera "Ospedale di Circolo” di Busto Arsizio, Busto Arsizio, Italy aut Ricci Riccardo Institute of Anatomic Pathology, Università Cattolica del Sacro Cuore, Policlinico Gemelli, Roma, Italy aut Martini Maurizio Institute of Anatomic Pathology, Università Cattolica del Sacro Cuore, Policlinico Gemelli, Roma, Italy aut Zurleni Francesco Operative Unit of Surgery, Azienda Ospedaliera "Ospedale di Circolo” di Busto Arsizio, Busto Arsizio, Italy aut Endocrine Pathology Springer US; http://www.springer-ny.com 24/2(2013-06-01), 100-105 1046-3976 24:2<100 2013 24 12022 https://doi.org/10.1007/s12022-013-9239-x text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s12022-013-9239-x text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- Serio Giovanni Operative Unit of Anatomic Pathology, Azienda Ospedaliera "Ospedale di Circolo” di Busto Arsizio, Via A. da Brescia 1, 21052, Busto Arsizio, Italy aut NATIONALLICENCE 700 1- Zampatti Clementina Operative Unit of Anatomic Pathology, Azienda Ospedaliera "Ospedale di Circolo” di Busto Arsizio, Via A. da Brescia 1, 21052, Busto Arsizio, Italy aut NATIONALLICENCE 700 1- Ballabio Andrea Operative Unit of Surgery, Azienda Ospedaliera "Ospedale di Circolo” di Busto Arsizio, Busto Arsizio, Italy aut NATIONALLICENCE 700 1- Ricci Riccardo Institute of Anatomic Pathology, Università Cattolica del Sacro Cuore, Policlinico Gemelli, Roma, Italy aut NATIONALLICENCE 700 1- Martini Maurizio Institute of Anatomic Pathology, Università Cattolica del Sacro Cuore, Policlinico Gemelli, Roma, Italy aut NATIONALLICENCE 700 1- Zurleni Francesco Operative Unit of Surgery, Azienda Ospedaliera "Ospedale di Circolo” di Busto Arsizio, Busto Arsizio, Italy aut NATIONALLICENCE 773 0- Endocrine Pathology Springer US; http://www.springer-ny.com 24/2(2013-06-01), 100-105 1046-3976 24:2<100 2013 24 12022 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer