Publication:
Pancreatoblastoma: cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics

dc.contributor.coauthorReid, Michelle D.
dc.contributor.coauthorBhattarai, Shristi
dc.contributor.coauthorGraham, Rondell P.
dc.contributor.coauthorPehlivanoglu, Burcin
dc.contributor.coauthorSigel, Carlie S.
dc.contributor.coauthorShi, Jiaqi
dc.contributor.coauthorSaqi, Anjali
dc.contributor.coauthorShirazi, Maryam
dc.contributor.coauthorXue, Yue
dc.contributor.coauthorBasturk, Olca
dc.contributor.departmentKUH (Koç University Hospital)
dc.contributor.departmentSchool of Medicine
dc.contributor.kuauthorAdsay, Nazmi Volkan
dc.contributor.schoolcollegeinstituteKUH (KOÇ UNIVERSITY HOSPITAL)
dc.contributor.schoolcollegeinstituteSCHOOL OF MEDICINE
dc.date.accessioned2024-11-09T23:45:34Z
dc.date.issued2019
dc.description.abstractBackground Pancreatoblastoma (PBL) is a rare malignant pancreatic tumor seen predominantly in childhood, and its cytologic diagnosis remains challenging. Methods Twelve fine-needle-aspirations from 11 adults were analyzed. Results In total, 6 men and 5 women (median age, 45 years; age range, 32-60 years) had tumors measuring a median 5.6 cm (range, 2.5-12 cm) located in the pancreatic head (n = 7) or tail (n = 4), including 3 with familial adenomatous polyposis (FAP)/FAP-related syndromes and 4 with metastasis at diagnosis. The median follow-up was 39.8 months (range, 0.8-348 months), and 5 patients died of disease. The original cytology diagnoses were: PBL (n = 2), neuroendocrine neoplasm (n = 2), poorly differentiated neuroendocrine carcinoma (n = 2), well differentiated neuroendocrine tumor (n = 1), poorly differentiated carcinoma (n = 2), "positive for malignancy" (n = 1), acinar cell carcinoma (n = 1), and epithelioid neoplasm with endocrine and acinar differentiation versus PBL (n = 1). Universal cytopathologic findings included hypercellularity; 3-dimensional clusters; and single, monotonous, blast-like cells that were from 1.5 to 2.0 times the size of red blood cells with high nuclear-to-cytoplasmic ratio, fine chromatin, small, distinct nucleoli, and a resemblance to well differentiated neuroendocrine tumor and poorly differentiated neuroendocrine carcinoma. Branching pseudopapillae (n = 7) and grooved nuclei (n = 3) raised the differential diagnosis of solid-pseudopapillary neoplasm, but with more atypia. Uncommon features included pleomorphism (n = 4) and numerous mitoses (n = 1). Squamoid morules were seen on smears (n = 5) or cell blocks (n = 6) in 70% of patients and were characterized by epithelioid cells with elongated, streaming nuclei, fine chromatin, absent nucleoli, and positive nuclear beta-catenin (n = 6 of 8). The median Ki-67 index was 21% (range, 2%-70%), and neuroendocrine marker expression was common (100%), but acinar markers were variable (63%). Conclusions A combination of cytologic findings in PBL, including a predominant population of primitive blast-like cells, subtle squamoid morules, frequent neuroendocrine and variable acinar phenotype, should facilitate accurate cytologic diagnosis and distinction from common mimics.
dc.description.indexedbyWOS
dc.description.indexedbyScopus
dc.description.indexedbyPubMed
dc.description.issue11
dc.description.openaccessYES
dc.description.publisherscopeInternational
dc.description.sponsoredbyTubitakEuN/A
dc.description.volume127
dc.identifier.doi10.1002/cncy.22187
dc.identifier.eissn1934-6638
dc.identifier.issn1934-662X
dc.identifier.quartileQ2
dc.identifier.scopus2-s2.0-85073963986
dc.identifier.urihttps://doi.org/10.1002/cncy.22187
dc.identifier.urihttps://hdl.handle.net/20.500.14288/13858
dc.identifier.wos489550300001
dc.keywordsCytology
dc.keywordsFine-needle aspiration (FNA)
dc.keywordsPancreas
dc.keywordsPancreatoblastoma
dc.language.isoeng
dc.publisherWiley
dc.relation.ispartofCancer Cytopathology
dc.subjectOncology
dc.subjectPathology
dc.titlePancreatoblastoma: cytologic and histologic analysis of 12 adult cases reveals helpful criteria in their diagnosis and distinction from common mimics
dc.typeJournal Article
dspace.entity.typePublication
local.contributor.kuauthorAdsay, Nazmi Volkan
local.publication.orgunit1SCHOOL OF MEDICINE
local.publication.orgunit1KUH (KOÇ UNIVERSITY HOSPITAL)
local.publication.orgunit2KUH (Koç University Hospital)
local.publication.orgunit2School of Medicine
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