naa a22 4500 510773567 CHVBK 20180411083222.0 cr unu---uuuuu 180411e20131201xx s 000 0 eng 10.1007/s12022-013-9262-y doi (NATIONALLICENCE)springer-10.1007/s12022-013-9262-y Ectopic Duodenal Insulinoma: A Very Rare and Challenging Tumor Type. Description of a Case and Review of the Literature [Elektronische Daten] [Stefano La Rosa, Dario Pariani, Calogera Calandra, Alessandro Marando, Fausto Sessa, Ferdinando Cortese, Carlo Capella] Although most insulinomas are located in the pancreas, very rare ectopic cases have been described in the spleen, perisplenic tissue, duodenohepatic ligament, and adjacent to the ligament of Treitz. Moreover, three cases located in the duodenum have also been reported in the English literature. Ectopic insulinomas represent challenging neoplasms with clinical implications mainly due to the difficulties in their pre-operatory diagnosis and localization. In the present paper, we describe the fourth ectopic duodenal insulinoma so far reported. A 75-year-old woman presented at clinical observation due to neuroglycopenic symptoms that disappeared after glucose intake. Tumor was localized in the second portion of the duodenum in front of the papilla of Vater and was surgically enucleated. Microscopically, it was composed of monomorphic cells with eosinophilic cytoplasm arranged in trabecular and lobular patterns and diffusely positive for insulin, proinsulin, amylin, and PDX1. About 30% of tumor cells also showed immunoreactivity for somatostatin, while no positivity for glucagon, pancreatic polypeptide, gastrin, serotonin, and somatostatin receptor subtype 2A was found. The Ki67 proliferative index was 1%. We have also reviewed the literature on this topic to give the reader a comprehensive overview of this very rare tumor type. Springer Science+Business Media New York, 2013 Insulinoma nationallicence Ectopic tumor nationallicence Duodenum nationallicence Neuroendocrine tumor nationallicence La Rosa Stefano Department of Pathology, Ospedale di Circolo, Viale Borri 57, 21100, Varese, Italy aut Pariani Dario Unit of General and Thoracic Surgery, Ospedale di Circolo di Busto Arsizio, Presidio Ospedaliero di Saronno, Saronno, Italy aut Calandra Calogera Unit of Anatomic Pathology, Ospedale di Circolo di Busto Arsizio, Presidio Ospedaliero di Saronno, Saronno, Italy aut Marando Alessandro Department of Surgical and Morphological Sciences, University of Insubria, Varese, Italy aut Sessa Fausto Department of Surgical and Morphological Sciences, University of Insubria, Varese, Italy aut Cortese Ferdinando Unit of General and Thoracic Surgery, Ospedale di Circolo di Busto Arsizio, Presidio Ospedaliero di Saronno, Saronno, Italy aut Capella Carlo Department of Surgical and Morphological Sciences, University of Insubria, Varese, Italy aut Endocrine Pathology Springer US; http://www.springer-ny.com 24/4(2013-12-01), 213-219 1046-3976 24:4<213 2013 24 12022 https://doi.org/10.1007/s12022-013-9262-y text/html Onlinezugriff via DOI 1 research-article jats NATIONALLICENCE 856 40 https://doi.org/10.1007/s12022-013-9262-y text/html Onlinezugriff via DOI NATIONALLICENCE 700 1- La Rosa Stefano Department of Pathology, Ospedale di Circolo, Viale Borri 57, 21100, Varese, Italy aut NATIONALLICENCE 700 1- Pariani Dario Unit of General and Thoracic Surgery, Ospedale di Circolo di Busto Arsizio, Presidio Ospedaliero di Saronno, Saronno, Italy aut NATIONALLICENCE 700 1- Calandra Calogera Unit of Anatomic Pathology, Ospedale di Circolo di Busto Arsizio, Presidio Ospedaliero di Saronno, Saronno, Italy aut NATIONALLICENCE 700 1- Marando Alessandro Department of Surgical and Morphological Sciences, University of Insubria, Varese, Italy aut NATIONALLICENCE 700 1- Sessa Fausto Department of Surgical and Morphological Sciences, University of Insubria, Varese, Italy aut NATIONALLICENCE 700 1- Cortese Ferdinando Unit of General and Thoracic Surgery, Ospedale di Circolo di Busto Arsizio, Presidio Ospedaliero di Saronno, Saronno, Italy aut NATIONALLICENCE 700 1- Capella Carlo Department of Surgical and Morphological Sciences, University of Insubria, Varese, Italy aut NATIONALLICENCE 773 0- Endocrine Pathology Springer US; http://www.springer-ny.com 24/4(2013-12-01), 213-219 1046-3976 24:4<213 2013 24 12022 Metadata rights reserved Springer special CC-BY-NC licence nationallicence BK010053 XK010053 XK010000 NATIONALLICENCE NATIONALLICENCE NL-springer