Clear cell sarcoma of the ileum: report of a case and review of the literature.
BACKGROUND
Clear cell sarcoma of the gastrointestinal tract (CCS-GI) is an extremely rare and aggressive tumor, which often mimics other neoplastic processes. Because of this feature, its real incidence may have been underestimated, especially in the past when genetic tests were less available than nowadays. To date, less then 30 cases have been described in the literature on the GI tract.


METHODS
We report the case of a 69-year-old woman who presented with active rectal bleeding. After a negative colonoscopy, the patient underwent a video-capsule endoscopy. The latter detected an ileal mass that was surgically resected. The microscopic appearance was consistent with a malignant mesenchymal neoplasm; immunohistochemistry was positive for S100 protein, CD56, and INI1. Fluorescence in situ hybridization showed a translocation involving the EWSR1 (Ewing sarcoma 1) gene region. All these findings were consistent with a CCS-GI.


CONCLUSIONS
Herein we present a case of CCS-GI, discuss its clinical and pathological features, and review the literature on the subject.
II Department of Surgery (IV unit), Regional Hospital Ca' Foncello, Treviso, Italy. fr.damico@gmail.com
Francesco E
FE
D'Amico
Cesare
C
Ruffolo
Salvatore
S
Romeo
Marco
M
Massani
Angelo P
AP
Dei Tos
Nicolò
N
Bassi
D000368:Aged; D000903:Antibiotics, Antineoplastic / Q000627:therapeutic use; D014408:Biomarkers, Tumor / Q000235:genetics / Q000378:metabolism; D019002:CD56 Antigen / Q000378:metabolism; D002148:Calmodulin-Binding Proteins / Q000235:genetics; D002868:Chromosomal Proteins, Non-Histone / Q000378:metabolism; D003131:Combined Modality Therapy; D004268:DNA-Binding Proteins / Q000378:metabolism; D015251:Epirubicin / Q000627:therapeutic use; D005260:Female; D006801:Humans; D007078:Ileal Neoplasms / Q000235:genetics / Q000378:metabolism / Q000473:pathology* / Q000628:therapy; D007082:Ileum / Q000473:pathology* / Q000601:surgery; D017404:In Situ Hybridization, Fluorescence; D008198:Lymph Nodes / Q000473:pathology; D008207:Lymphatic Metastasis; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins / Q000235:genetics; D009418:S100 Proteins / Q000378:metabolism; D000071796:SMARCB1 Protein; D018227:Sarcoma, Clear Cell / Q000235:genetics / Q000378:metabolism / Q000556:secondary* / Q000628:therapy; D014157:Transcription Factors / Q000378:metabolism; D014178:Translocation, Genetic; D016896:Treatment Outcome
D000903:Antibiotics, Antineoplastic; D014408:Biomarkers, Tumor; D019002:CD56 Antigen; D002148:Calmodulin-Binding Proteins; D002868:Chromosomal Proteins, Non-Histone; D004268:DNA-Binding Proteins; C500986:EWSR1 protein, human; D034802:RNA-Binding Protein EWS; D016601:RNA-Binding Proteins; D009418:S100 Proteins; D000071796:SMARCB1 Protein; C513266:SMARCB1 protein, human; D014157:Transcription Factors; D015251:Epirubicin