Desmoplastic small round cell tumor of the lung.
An extra-abdominal desmoplastic small round cell tumor (DSRCT) of the lung with immunohistochemical, ultrastructural, and cytogenetic evidence of multidirectional differentiation is reported. We demonstrate that DSRCTs of the lung and pleura show morphologic, molecular, genetic, and ultrastructural features similar to DSRCTs arising in other sites. The tumor showed coexpression of cytokeratins (AE1/3, epithelial membrane antigen, CAM 5.2) and mesenchymal markers (vimentin, desmin, neuron-specific enolase), as well as WT1. Ultrastructurally, intracytoplasmic whorls of intermediate filaments, similar to previous descriptions of DSRCT in nonthoracic sites, were also demonstrated in this case. EWS-WT1 gene fusion characteristic of DSRCT with the t(11;22)(q13;q12) translocation was demonstrated in this tumor.
Syed
Salahuddin
S
Department of Pathology, University of Texas Medical Branch, Galveston 77555, USA.
Haque
Abida K
AK
Hawkins
Hal K
HK
Sorensen
Poul H B
PH
Cowan
Daniel F
DF
D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D018288:Carcinoma, Small Cell / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D002880:Chromosomes, Human, Pair 11; D002892:Chromosomes, Human, Pair 22; D004273:DNA, Neoplasm / Q000032:analysis; D003893:Desmin / Q000032:analysis; D006801:Humans; D007382:Intermediate Filaments / Q000648:ultrastructure; D008175:Lung Neoplasms / Q000737:chemistry / Q000235:genetics / Q000473:pathology*; D008297:Male; D009363:Neoplasm Proteins / Q000032:analysis; D015514:Oncogene Proteins, Fusion / Q000032:analysis / Q000235:genetics; D013902:Radiography, Thoracic; D020133:Reverse Transcriptase Polymerase Chain Reaction; D014057:Tomography, X-Ray Computed; D014178:Translocation, Genetic
D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D003893:Desmin; C106032:EWS1-WT1 fusion protein, human; D009363:Neoplasm Proteins; D015514:Oncogene Proteins, Fusion