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Ectopic Duodenal Insulinoma: A Very Rare and Challenging Tumor Type. Description of a Case and Review of the Literature

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Abstract

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.

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Authors and Affiliations

  1. Department of Pathology, Ospedale di Circolo, Viale Borri 57, 21100, Varese, Italy

    Stefano La Rosa

  2. Unit of General and Thoracic Surgery, Ospedale di Circolo di Busto Arsizio, Presidio Ospedaliero di Saronno, Saronno, Italy

    Dario Pariani & Ferdinando Cortese

  3. Unit of Anatomic Pathology, Ospedale di Circolo di Busto Arsizio, Presidio Ospedaliero di Saronno, Saronno, Italy

    Calogera Calandra

  4. Department of Surgical and Morphological Sciences, University of Insubria, Varese, Italy

    Alessandro Marando, Fausto Sessa & Carlo Capella

Authors
  1. Stefano La Rosa
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  2. Dario Pariani
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  3. Calogera Calandra
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  4. Alessandro Marando
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  5. Fausto Sessa
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  6. Ferdinando Cortese
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  7. Carlo Capella
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Correspondence to Stefano La Rosa.

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La Rosa, S., Pariani, D., Calandra, C. et al. Ectopic Duodenal Insulinoma: A Very Rare and Challenging Tumor Type. Description of a Case and Review of the Literature. Endocr Pathol 24, 213–219 (2013). https://doi.org/10.1007/s12022-013-9262-y

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  • DOI: https://doi.org/10.1007/s12022-013-9262-y

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