# A case of acinar cell carcinoma originating from the accessory papilla of the duodenum

**Authors:** Kiyoshi Narita, Masataka Okuno, Seiji Natsume, Tomonari Asano, Hisafumi Saito, Masashi Negita, Seiji Ito, Koji Komori, Tetsuya Abe, Kazuo Hara, Nozomi Okuno, Waki Hosoda, Yasuhiro Shimizu

PMC · DOI: 10.1186/s40792-024-01872-3 · 2024-04-16

## TL;DR

A rare case of acinar cell carcinoma was found in the accessory papilla of the duodenum in a 61-year-old woman.

## Contribution

This is the first reported case of acinar cell carcinoma originating from the accessory papilla of the duodenum.

## Key findings

- The tumor was localized in the submucosa of the duodenum near the accessory papilla.
- Histopathological and immunohistochemical analyses confirmed acinar cell differentiation.
- The tumor was separated from pancreatic tissue by the duodenal muscle layer.

## Abstract

A 61-year-old female was referred to our hospital with a neoplastic lesion in the duodenum. Computed tomography with contrast enhancement revealed a 10-mm tumor in the duodenum. Upper gastrointestinal endoscopy revealed a submucosal tumor-like lesion in the descending part of the duodenum. Endoscopic ultrasound revealed a well-defined hypoechoic tumor. Biopsy and immunohistochemical findings including negative Synaptophysin and Chromogranin A staining and positive Trypsin and BCL10 staining suggested a carcinoma with acinar cell differentiation. Pancreatoduodenectomy was performed, and the resected specimen had a 15-mm solid nodule in the submucosal layer of the duodenum. Pancreatogram of the resected specimen revealed a tumor localized in the accessory papilla region. In histopathological examination, the tumor was found in the submucosa of the duodenum with pancreatic tissue present nearby, and these were separated from the pancreatic parenchyma by the duodenal muscle layer. These findings led to a diagnosis of acinar cell carcinoma originating from the accessory papilla of the duodenum.

Acinar cell carcinoma originating from the accessory papilla of the duodenum is exceptionally rare, with no reported cases to date. The origin was considered to be pancreatic tissue located in the accessory papilla region.

## Linked entities

- **Proteins:** prss1.L (serine protease 1 L homeolog), BCL10 (BCL10 immune signaling adaptor)
- **Diseases:** acinar cell carcinoma (MONDO:0004965)

## Full-text entities

- **Genes:** BCL10 (BCL10 immune signaling adaptor) [NCBI Gene 8915] {aka CARMEN, CIPER, CLAP, IMD37, c-E10, mE10}, SYP (synaptophysin) [NCBI Gene 6855] {aka MRX96, MRXSYP, XLID96}, CHGA (chromogranin A) [NCBI Gene 1113] {aka CGA, PHE5, PHES}
- **Diseases:** Acinar cell carcinoma (MESH:D018267), neoplastic lesion (MESH:D009062), carcinoma (MESH:D009369)

## Figures

5 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11019189/full.md

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Source: https://tomesphere.com/paper/PMC11019189