“Pure” hepatoid tumors of the pancreas harboring CTNNB1 somatic mutations: a new entity among solid pseudopapillary neoplasms

Hepatoid tumors (HTs) represent a rare group of neoplasms that are histologically similar to hepatocellular carcinoma but arise outside the liver. The current World Health Organization classification recognizes the hepatoid morphology of pancreatic tumors only as a possible variant of pancreatic duc...

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Bibliographic Details
Published in:Virchows Archiv : an international journal of pathology 2022-07, Vol.481 (1), p.41-47
Main Authors: Mattiolo, Paola, Mafficini, Andrea, Lawlor, Rita T., Marchegiani, Giovanni, Malleo, Giuseppe, Pea, Antonio, Salvia, Roberto, Piccoli, Paola, Sciammarella, Concetta, Santonicco, Nicola, Parisi, Alice, Silvestris, Nicola, Milella, Michele, Adsay, Volkan, Scarpa, Aldo, Luchini, Claudio
Format: Article
Language:English
Subjects:
Adenocarcinoma
beta Catenin - genetics
Biomarkers, Tumor - genetics
Carcinoma, Pancreatic Ductal - genetics
Carcinoma, Pancreatic Ductal - pathology
Chromosomes
CTNNB1 gene
Globules
Hepatocellular carcinoma
Heterozygosity
Histology
Humans
Immunohistochemistry
Liver Neoplasms - genetics
Liver Neoplasms - pathology
Loss of heterozygosity
Medicine
Medicine & Public Health
Mutation
Mutation - genetics
Neoplasia
Neoplasms
Next-generation sequencing
Nodules
Original
Original Article
Pancreas
Pancreas - pathology
Pancreatic cancer
Pancreatic Neoplasms
Pancreatic Neoplasms - genetics
Pancreatic Neoplasms - pathology
Pathology
Taxonomy
Tumors
β-Catenin
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Description
Summary:Hepatoid tumors (HTs) represent a rare group of neoplasms that are histologically similar to hepatocellular carcinoma but arise outside the liver. The current World Health Organization classification recognizes the hepatoid morphology of pancreatic tumors only as a possible variant of pancreatic ductal adenocarcinoma (PDAC). Here, we describe two cases of “pure” HT of the pancreas showing common features and characterized by indolent biological behavior. These tumors were roundish nodules with pushing borders, hyaline globules, and pure hepatoid histology; they were diffusely positive for β-catenin and LEF1 on immunohistochemistry. At next-generation sequencing, both neoplasms harbored only one pathogenic somatic mutation that affected the CTNNB1 gene at exon 3 and showed a loss of heterozygosity on chromosomes 18 and 21. By integrating macroscopic and microscopic features, along with their molecular profiles, we advocate that such tumors represent a distinct entity from PDAC and should be considered a new variant of solid pseudopapillary neoplasms. The recognition of this new neoplastic category may have immediate implications not only for tumor taxonomy but also for clinical practice.
ISSN:0945-6317
1432-2307
DOI:10.1007/s00428-022-03317-4