Acinar cystic transformation of the pancreas: Histomorphology and molecular analysis to unravel its heterogeneous nature Journal Article
| Authors: | Luchini, C.; Mattiolo, P.; Basturk, O.; Mafficini, A.; Ozcan, K.; Lawlor, R. T.; Hong, S. M.; Brosens, L. A.; Marchegiani, G.; Pea, A.; Manfrin, E.; Sciacca, G.; Zampieri, F.; Polati, R.; De Robertis, R.; Milella, M.; D'Onofrio, M.; Malleo, G.; Salvia, R.; Adsay, V.; Scarpa, A. |
| Article Title: | Acinar cystic transformation of the pancreas: Histomorphology and molecular analysis to unravel its heterogeneous nature |
| Abstract: | Acinar cystic transformation (ACT) of the pancreas, previously called acinar cell cystadenoma, is a poorly understood and rare entity among pancreatic cystic lesions. This study aims to clarify its real nature. This research cohort included 25 patients with pancreatic ACT, representing the largest series in the literature. We describe their clinicopathological features and molecular profile using next-generation sequencing. ACT arose more often in women (F/M≃2:1), in the body-tail region, with a mean size of 4 cm. At the latest follow-up, all patients were alive and disease free. Histologically, a typical acinar epithelium lined all cysts, intermingled with ductal-like epithelium in 11/25 (44%) cases. All the cases lacked any evidence of malignancy. Three ACT showed peculiar features: 1 showed an extensive and diffuse microcystic pattern, and the other 2 harbored foci of low-grade pancreatic intraepithelial neoplasia (PanIN) in the ductal-like epithelium. Next-generation sequencing revealed the presence of 2 pathogenic/likely pathogenic mutations in 2 different cases, 1 with ductal-like epithelium and 1 with PanIN, and affecting KRAS (c.34G>C, p.G12R) and SMO (c.1685G>A, p.R562Q) genes, respectively. The other case with PanIN was not available for sequencing. Overall, our findings support that ACT is a benign entity, potentially arising from heterogeneous conditions/background, including: (1) acinar microcysts, (2) malformations, (3) obstructive/inflammatory setting, (4) genetic predisposition, (5) possible neoplastic origin. Although all indications are that ACT is benign, the potential occurrence of driver mutations suggests discussing a potential role of long-term surveillance for these patients. Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved. |
| Keywords: | immunohistochemistry; adult; clinical article; human tissue; aged; middle aged; somatic mutation; missense mutation; clinical feature; histopathology; disease free survival; pancreas cancer; pancreatic neoplasms; follow up; pancreaticoduodenectomy; pancreas; spleen; tumor differentiation; carcinoma, pancreatic ductal; cohort analysis; gene frequency; pathology; renal cell carcinoma; abdominal pain; pancreas carcinoma; lung embolism; microsatellite instability; family history; carcinoma in situ; diabetes mellitus; pancreas tumor; jaundice; bile duct carcinoma; prostate hypertrophy; epithelium; k ras protein; genetic predisposition; smoothened protein; genomic dna; acinar cell; pancreas islet cell tumor; trypsin; psammoma body; copy number variation; pancreatic cyst; acinar cell cystadenoma; adenomatoid tumor; endometrium sarcoma; papillary renal cell carcinoma; acute pancreatitis; abdominal tumor; squamous cell metaplasia; pancreas adenoma; act; high throughput sequencing; very elderly; dna sequencing; humans; human; male; female; article; pancreas calcification; acinar cells; b cell lymphoma/leukemia 10; microadenoma; pancreatic acinar cystic transformation; pancreatic cystadenoma; aca; acinar cystadenoma |
| Journal Title: | American Journal of Surgical Pathology |
| Volume: | 47 |
| Issue: | 3 |
| ISSN: | 0147-5185 |
| Publisher: | Lippincott Williams & Wilkins |
| Date Published: | 2023-03-01 |
| Start Page: | 379 |
| End Page: | 386 |
| Language: | English |
| DOI: | 10.1097/pas.0000000000002017 |
| PUBMED: | 36649476 |
| PROVIDER: | scopus |
| DOI/URL: | |
| Notes: | Article -- Source: Scopus |
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