Desmoplastic small round-cell tumor: a case report on the large cell variant with immunohistochemical, ultrastructural, and molecular genetic analysis.
examination. The patient died 10 months after surgery. Histologically, the tumor was composed of predominantly large epithelioid cells with foci of anaplasia mimicking metastatic carcinoma. Immunohistochemically, the tumor cells stained with anti-cytokeratin, EMA, desmin, and NSE antisera. Electron microscopy showed secretory lumina, desmosomes, cell processes with microtubules and electron-dense granules, and focal whorls of intermediate filaments. Reverse transcriptase-polymerase chain reaction performed on paraffin block-retrieved tissue demonstrated the EWS/WT-1 fusion transcript characteristic of the t(11;22)(p13;q12). This case illustrates a less common histological pattern of DSRCT, i.e., diffuse large cells, thus supporting the view that this tumor presents a wider morphological spectrum than that previously recognized.
Pasquinelli
G
G
Department of Clinical Radiology and Anatomic Pathology, University of Bologna, Italy. pasqui@med.unibo.it
Montanaro
L
L
Martinelli
G N
GN
D000008:Abdominal Neoplasms / Q000737:chemistry / Q000235:genetics* / Q000648:ultrastructure*; D000328:Adult; D014408:Biomarkers, Tumor / Q000032:analysis; D018288:Carcinoma, Small Cell / Q000737:chemistry / Q000235:genetics* / Q000648:ultrastructure*; D004273:DNA, Neoplasm / Q000032:analysis; D004268:DNA-Binding Proteins / Q000032:analysis; D017809:Fatal Outcome; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D006801:Humans; D007124:Immunoenzyme Techniques; D008297:Male; D008967:Molecular Biology; D009363:Neoplasm Proteins / Q000032:analysis; D034802:RNA-Binding Protein EWS; D020133:Reverse Transcriptase Polymerase Chain Reaction; D012261:Ribonucleoproteins / Q000032:analysis; D014157:Transcription Factors / Q000032:analysis; D025721:WT1 Proteins
D014408:Biomarkers, Tumor; D004273:DNA, Neoplasm; D004268:DNA-Binding Proteins; D034441:Heterogeneous-Nuclear Ribonucleoproteins; D009363:Neoplasm Proteins; D034802:RNA-Binding Protein EWS; D012261:Ribonucleoproteins; D014157:Transcription Factors; D025721:WT1 Proteins