Rare primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation

Gianna N. DiGrazia*, Shuchi Zinzuwadia, Alessa P. Aragao, Gerald P. Camren, John F. Hibbeln, Marielia Gerena, Anugayathri Jawahar

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

Abstract

Angiosarcomas are aggressive, malignant endothelial cell tumors derived from the vascular or lymphatic epithelium. Angiosarcomas makes up less than 1% of all soft tissue sarcomas. Primary pulmonary angiosarcoma is a rare tumor that most commonly presents as metastatic disease and carries a grim prognosis. The disease rarity and low index of suspicion make the diagnosis problematic. In this report, we present a case of primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation. The diagnostic images, pathology and management strategy are discussed in this case with overall longer survival. The unique neuroendocrine pathologic subtype may contribute to the overall longer survival, however, there are only a few limited cases published. Further recognition and investigation of primary pulmonary angiosarcoma with aberrant neuroendocrine differentiation is warranted to further characterize diagnostic markers, determine more effective treatment strategies, and establish a possible link to improved survival.

Original languageEnglish (US)
Pages (from-to)88-93
Number of pages6
JournalClinical Imaging
Volume82
DOIs
StatePublished - Feb 2022

ASJC Scopus subject areas

  • Radiology Nuclear Medicine and imaging

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