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Gastric Amphicrine Carcinoma Showing Neuroendocrine and Pancreatic Acinar Cell Differentiation. Lesson from a Challenging Case Opening New Perspectives in the Diagnostic Work-Up of Gastric Neuroendocrine Neoplasms

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Abstract

Amphicrine carcinomas are epithelial neoplasms composed of cells with co-existing exocrine-neuroendocrine phenotype and are challenging lesions from both diagnostic and therapeutic perspectives.

Here, we report the case of a 63-year-old male patient with a gastric nodule that was endoscopically biopsied, revealing histological features of a type 3 well-differentiated gastric neuroendocrine tumor (NET). At imaging, the lesion was single and limited to the stomach, but did not present In−111Octreotide uptake, despite SSTR2A immunohistochemical expression. The patient underwent a wedge resection of the gastric wall, with a final pathological diagnosis of amphicrine carcinoma with pancreatic acinar cell and neuroendocrine features (pT1b). Predictive immunohistochemistry showed microsatellite stability and negative HER2 status. Hotspot targeted deep sequencing of 57 genes showed no somatic mutation, in agreement with the low mutational burden reported for gastric amphicrine carcinomas. Due to a low stage of the tumor and the poor performance status of the patient, no additional oncological treatment was administered. The patient was disease-free after 18 months.

This unusual case highlights the importance of considering amphicrine carcinoma in the diagnostic work-up of gastric type 3 NET. This can be done by including in the immunohistochemical panel non-neuroendocrine markers, such as the pancreatic acinar cell and glandular ones. Correct pathological diagnosis is pivotal to determine the appropriate staging (NET vs exocrine one) for surgical and oncological management.

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Authors and Affiliations

  1. Histopathology, Central Institute, Valais Hospital, Avenue du Grand-Champsec 86, CH-1951, Sion, Switzerland

    Amedeo Sciarra

  2. Unit of Pathology, Department of Medicine and Technological Innovation, University of Insubria, Varese, Italy

    Amedeo Sciarra & Stefano La Rosa

  3. Department of Biomedical Sciences, Humanitas University, Pieve Emanule, Milan, Italy

    Silvia Uccella

  4. Pathology Service, IRCCS, Humanitas Research Hospital, Rozzano, Milano, Italy

    Silvia Uccella

  5. Gastroenterology, Valais Hospital, Sion, Switzerland

    Philippe Hiroz

  6. General Surgery, Valais Hospital, Sion, Switzerland

    Ian Fournier

  7. Nuclear Medicine, Valais Hospital, Sion, Switzerland

    Vincent Soubeyran

  8. Unit of Pathology, Department of Oncology, ASST Sette Laghi, Varese, Italy

    Giovanna Finzi & Stefano La Rosa

  9. Hereditary Cancer Research Center, Department of Medicine and Technological Innovation, University of Insubria, Varese, Italy

    Stefano La Rosa

Authors
  1. Amedeo Sciarra
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  2. Silvia Uccella
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  4. Ian Fournier
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  5. Vincent Soubeyran
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  7. Stefano La Rosa
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Contributions

AS, SLR study design, data collection, drafting. SU, PH, IF, VS, GF data collection, critical review of the manuscript. All authors approved the final version of the paper.

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Correspondence to Amedeo Sciarra.

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Sciarra, A., Uccella, S., Hiroz, P. et al. Gastric Amphicrine Carcinoma Showing Neuroendocrine and Pancreatic Acinar Cell Differentiation. Lesson from a Challenging Case Opening New Perspectives in the Diagnostic Work-Up of Gastric Neuroendocrine Neoplasms. Endocr Pathol 34, 349–357 (2023). https://doi.org/10.1007/s12022-023-09773-1

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