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Curr Oncol. 2026;33(6):curroncol33060367.

Morphologic Features and Clinical Outcomes of Acinar Cell Carcinoma of the Pancreas: A Multicenter Retrospective Study of 37 Patients in South Korea.

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제1저자 이윤석, 백우현 · 교신저자 황진혁, 우상명

소속 (8)
  1. Department of Internal Medicine, Ilsan Paik Hospital, Inje University College of Medicine, Goyang 10380, Republic of Korea.
  2. Department of Internal Medicine, Seoul Metropolitan Government-Seoul National University Boramae Medical Center, Seoul 07061, Republic of Korea.
  3. Department of Internal Medicine and Liver Research Institute, Seoul National University Hospital, Seoul 03080, Republic of Korea.
  4. Department of Internal Medicine, Kyungpook National University Hospital, Daegu 41944, Republic of Korea.
  5. Research Institute, Center for Liver and Pancreatobiliary Cancer, National Cancer Center, 323 Ilsan-ro, Ilsandong-gu, Goyang 10408, Republic of Korea.
  6. Department of Medicine, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul 06351, Republic of Korea.
  7. Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul 06591, Republic of Korea.
  8. Department of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, 82, Gumi-ro 173 Beon-gil, Bundang-gu, Seongnam 13620, Republic of Korea.

Abstract

Background: The clinical characteristics of pancreatic acinar cell carcinoma (ACC) remain poorly defined due to its rarity. This study aimed to evaluate the morphological features and clinical outcomes of pancreatic ACC. Method: This multicenter retrospective study analyzed clinical data from seven referral hospitals. Electronic medical records were comprehensively reviewed to extract patient data. Survival outcomes were calculated from the date of pathologic confirmation of ACC. Results: Of the 37 patients, 28 (75.7%) were male. The age distribution at diagnosis ranged widely from 12 to 86 years, with a median of 62.0 years; seven patients (18.9%) were aged under 50 years. Morphologically, 24 patients (64.9%) presented with solid masses, whereas four had cystic masses and four exhibited mixed solid and cystic components. Regarding tumor resectability, 19 patients (51.4%) had resectable disease, 7 (18.9%) were locally advanced, and 11 (29.7%) were metastatic. In terms of treatment, 22 patients (59.4%) underwent surgical resection, 12 (32.4%) received palliative chemotherapy, and the remainder received best supportive care. In the surgical resection group, the median OS was not reached, demonstrating significantly prolonged survival (mean OS, 7.6 years; 5-year OS rate, 51%). In contrast, the median OS was 0.9 years in the palliative chemotherapy group and 0.1 years in the best supportive care group (p = 0.040). Conclusions: Pancreatic ACC showed a broad age distribution, with approximately 20% of patients aged <50 years, and pleomorphic morphological features, including solid, cystic, and mixed patterns. Patients who underwent surgical resection demonstrated favorable long-term survival outcomes compared to historical data for pancreatic ductal adenocarcinoma.

Keywords

Multicenter Studies as TopicRepublic of Koreaacinar cell carcinomapancreatic neoplasms

Figures

Figure 1
Figure 1 Representative histologic and immunohistochemical features of pancreatic acinar cell carcinoma. (A) Hematoxylin and eosin staining (×400) shows solid/acinar architecture with tumor cells containing granular eosinophilic cytoplasm. (B) Immunohistochemistry for BCL10 (×400) shows diffuse cytoplasmic positivity for the acinar marker BCL10. (C) Immunohistochemistry for chymotrypsin C (×400) shows strong cytoplasmic positivity. (D) Immunohistochemistry for Ki-67 (×200) shows a significantly increased proliferative index in tumor cells, approximately 50%.
Figure 2
Figure 2 Computed tomography (CT) findings of pancreatic acinar cell carcinoma. (A,B) CT images in the arterial phases reveal a relatively well-defined, hypo-enhancing heterogenous solid mass in the pancreatic head/body without associated pancreatic duct dilation. (C) A large, heterogeneous mass is observed in the hepatic parenchyma, with internal low-density areas suggestive of central necrosis or cystic degeneration, accompanied by a huge mass in the pancreas head showing mixed enhancement features. (D) A mixed solid and cystic tumor replaces the right hepatic parenchyma, and a poorly defined tumor with necrotic components replaces a significant portion of the pancreatic body and tail.
Figure 3
Figure 3 Radiologic assessment of a large pancreatic acinar cell carcinoma. (A) Axial arterial phase CT demonstrates a large, exophytic mass in the pancreatic head with prominent internal low-density areas, suggesting central necrosis. (B,C) Axial T1-weighted MR images obtained before and after Gadovist enhancement. The tumor shows a characteristic hypo-enhancing and heterogenous pattern. (D) Diffusion-weighted imaging reveals marked high signal intensity within the tumor.
Figure 4
Figure 4 Distribution of clinical stages at the time of diagnosis. More than half of the patients presented with resectable disease (n = 19, 51.4%), followed by metastatic disease (n = 11, 29.7%) and locally advanced disease (n = 7, 18.9%).
Figure 5
Figure 5 Kaplan–Meier survival curves for overall survival (OS). (A) OS stratified by initial clinical stage at diagnosis. Patients with resectable disease showed significantly superior survival outcomes compared to those with locally advanced or metastatic disease (Log-rank p = 0.005). (B) OS according to the primary treatment modality. Surgical resection was associated with a markedly better prognosis compared to palliative chemotherapy or best supportive care (Log-rank p = 0.040).

그림 출처: PubMed Central 공개 논문 (PMC13298538)

Conflict of interest statement

The authors declare no conflicts of interests.

그 밖의 정보
출판 형태
Journal Article, Multicenter Study, Research Support, Non-U.S. Gov't
저널
Current oncology (Toronto, Ont.) · eISSN 1718-7729
전자 출판
2026-06-18
MeSH
Humans · Carcinoma, Acinar Cell* / pathology / therapy / mortality · Male · Retrospective Studies · Pancreatic Neoplasms* / pathology / therapy / mortality · Female · Middle Aged · Republic of Korea · Aged, 80 and over · Aged · Adult · Adolescent · Child · Treatment Outcome · Young Adult
연구비
National Cancer Center NCC-2610521
참고문헌
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