Sclerosing epithelioid mesenchymal neoplasm of the pancreas - a proposed new entity.

Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc
Olca BasturkDavid S Klimstra

Abstract

We have encountered pancreatic tumors with unique histologic features, which do not conform to any of the known tumors of the pancreas or other anatomical sites. We aimed to define their clinicopathologic features and whether they are characterized by recurrent molecular signatures. Eight cases were identified; studied histologically and by immunohistochemistry. Selected cases were also subjected to whole-exome sequencing (WES; n = 4), RNA-sequencing (n = 6), Archer FusionPlex assay (n = 5), methylation profiling using the Illumina MethylationEPIC (850k) array platform (n = 6), and TERT promoter sequencing (n = 5). Six neoplasms occurred in females. The mean age was 43 years (range: 26-75). Five occurred in the head/neck of the pancreas. All patients were treated surgically; none received neoadjuvant/adjuvant therapy. All patients are free of disease after 53 months of median follow-up (range: 8-94). The tumors were well-circumscribed, and the median size was 1.8 cm (range: 1.3-5.8). Microscopically, the unencapsulated tumors had a geographic pattern of epithelioid cell nests alternating with spindle cell fascicles. Some areas showed dense fibrosis, in which enmeshed tumor cells imparted a slit-like pattern. The predominant epi...Continue Reading

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Citations

Jul 28, 2021·Modern Pathology : an Official Journal of the United States and Canadian Academy of Pathology, Inc·Christian KoelscheGunhild Mechtersheimer
Aug 6, 2021·Virchows Archiv : an International Journal of Pathology·Atsuko KasajimaGünter Klöppel
Oct 15, 2021·Virchows Archiv : an International Journal of Pathology·Björn KonukiewitzGünter Klöppel

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Methods Mentioned

BETA
methylation profiling
exome sequencing
Assay
PCR

Software Mentioned

GSEA
FusionCatcher
ABSOLUTE
INTEGRATE
VarScan2
FACETS
Lancet
Bowtie2
OncoFuse
Fusion

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