Mixed acinar-neuroendocrine carcinoma of the pancreas

a case report and a review

Mark Jakobsen, Günter Klöppel, Sönke Detlefsen

Research output: Contribution to journalJournal articleResearchpeer-review

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Abstract

A 62-year-old woman presented with abdominal discomfort. Imaging studies showed a tumor in the pancreatic tail. At contrast-enhanced CT and macroscopy, the tumor showed cystic, solid and hemorrhagic areas. Histologically, the tumor was well-circumscribed and entirely encapsulated. Some of the tumor cells in the cystic areas were reminiscent of acinar cells, and the majority was arranged in a solid growth pattern. Immunohistochemistry revealed >30% positivity for chymotrypsin, chromogranin A, synaptophysin, and CD56. The diagnosis of a mixed acinar-neuroendocrine carcinoma (MAEC) was made. Review of the English-language literature revealed 44 previously published cases of resected MAECs. We found that, compared to pure acinar cell carcinoma, patients with MAEC have a slightly higher age and are less frequently males, as the male / female ratio was almost equal. The histogenesis of MAEC is still controversial. Due to the small number of cases it is at present not possible to define an evidence-based optimal treatment strategy for these patients.

Original languageEnglish
JournalHistology and Histopathology
Volume31
Issue number12
Pages (from-to)1381-1388
ISSN0213-3911
DOIs
Publication statusPublished - 2016

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Acinar Cell Carcinoma
Pancreas
Neoplasms
Synaptophysin
Acinar Cells
Chymotrypsin
Language
Growth

Keywords

  • Journal Article

Cite this

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title = "Mixed acinar-neuroendocrine carcinoma of the pancreas: a case report and a review",
abstract = "A 62-year-old woman presented with abdominal discomfort. Imaging studies showed a tumor in the pancreatic tail. At contrast-enhanced CT and macroscopy, the tumor showed cystic, solid and hemorrhagic areas. Histologically, the tumor was well-circumscribed and entirely encapsulated. Some of the tumor cells in the cystic areas were reminiscent of acinar cells, and the majority was arranged in a solid growth pattern. Immunohistochemistry revealed >30{\%} positivity for chymotrypsin, chromogranin A, synaptophysin, and CD56. The diagnosis of a mixed acinar-neuroendocrine carcinoma (MAEC) was made. Review of the English-language literature revealed 44 previously published cases of resected MAECs. We found that, compared to pure acinar cell carcinoma, patients with MAEC have a slightly higher age and are less frequently males, as the male / female ratio was almost equal. The histogenesis of MAEC is still controversial. Due to the small number of cases it is at present not possible to define an evidence-based optimal treatment strategy for these patients.",
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Mixed acinar-neuroendocrine carcinoma of the pancreas : a case report and a review. / Jakobsen, Mark; Klöppel, Günter; Detlefsen, Sönke.

In: Histology and Histopathology, Vol. 31, No. 12, 2016, p. 1381-1388.

Research output: Contribution to journalJournal articleResearchpeer-review

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