Case report: pseudoendocrine sarcoma, a clinicopathologic report of a newly described soft tissue neoplasm

Virchows Arch. 2023 Jun;482(6):1057-1063. doi: 10.1007/s00428-022-03476-4. Epub 2022 Dec 23.

Abstract

Papke et al. recently reported a series of twenty-three soft tissue lesions chiefly arising in older adults featuring distinct morphological and genetic characteristics. Pseudoendocrine sarcoma (PS) is the somewhat descriptive and provisional term adopted for the newly reported mesenchymal neoplasm. Since the publication of the original paper published in January 2022, a single case of PS has been published. Pseudoendocrine sarcoma shows a predilection for the paravertebral deep soft tissues of the trunk, low-grade neuroendocrine-like histological features, and hallmark CTNNB1 activating mutations.Herein, we will discuss a case of a 72-year-old woman presenting with a 4-cm laterocervical mass. Hematoxylin and eosin slides showed a multilobular proliferation of monomorphic epithelioid cells with speckled chromatin arranged in nests and trabeculae. Immunohistochemical staining and molecular analysis were consistent with the newly proposed entity.

Keywords: Beta catenin; Beta-catenin; CTNNB1; Next-generation sequencing; Paraspinal; Pseudoendocrine sarcoma.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Biomarkers, Tumor / analysis
  • Biomarkers, Tumor / genetics
  • Female
  • Humans
  • Sarcoma* / pathology
  • Soft Tissue Neoplasms* / pathology

Substances

  • Biomarkers, Tumor