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Intraductal papillary squamous neoplasm of the pancreas: Cyto-histologic correlation of a novel entity. Ann Diagn Pathol 2020 Oct;48:151583

Date

08/28/2020

Pubmed ID

32847795

Pubmed Central ID

PMC8388256

DOI

10.1016/j.anndiagpath.2020.151583

Scopus ID

2-s2.0-85089745040 (requires institutional sign-in at Scopus site)   1 Citation

Abstract

We correlate the cytologic and histologic features of a squamous-lined pancreatic cystic lesion with a complex papillary architecture and an associated KRAS mutation, which to our knowledge has not been previously described. A 69 year-old woman presented with intermittent left upper quadrant pain. CT imaging revealed a 1 cm solid lesion in the pancreatic tail with peripheral calcification. Endoscopic ultrasound-guided fine needle biopsy showed a proliferation of epithelial cells with fibrovascular cores. An immunohistochemical stain for p40 was positive in the lesional cells. A distal pancreatectomy revealed a unilocular, cystic, well-circumscribed, soft and friable mass measuring 1.0 × 1.0 × 0.8 cm. Histologically, the cyst was lined by nonkeratinizing stratified squamous epithelium with a complex papillary architecture, filling the cyst lumen. Molecular sequencing revealed a KRAS G12V missense mutation. While the lesion shared some histologic features with the previously described "squamoid cyst of the pancreatic ducts", the complex papillary architecture and presence of a KRAS mutation are unique to the entity we describe herein and we propose the name "intraductal papillary squamous neoplasm of the pancreas." Reporting the cytomorphologic features of this novel entity may help in identification of similar lesions and understanding of the clinicopathologic significance.

Author List

Hagen CE, Christians K, Hopp A, Giorgadze T, Hartley CP, Hunt B, Basturk O, Klimstra D

Authors

Kathleen K. Christians MD Professor in the Surgery department at Medical College of Wisconsin
Bryan C. Hunt MD Associate Professor in the Pathology department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Aged
Carcinoma, Pancreatic Ductal
Carcinoma, Squamous Cell
Diagnosis, Differential
Endoscopic Ultrasound-Guided Fine Needle Aspiration
Female
Humans
Mutation, Missense
Pain
Pancreas
Pancreatectomy
Pancreatic Neoplasms
Proto-Oncogene Proteins p21(ras)
Splenectomy