Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Pivot Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Target Concepts:
Gene/Protein
Disease
Symptom
Drug
Enzyme
Compound
Query: UMLS:C0268596 (
EMA
)
2,520
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Clear cell sarcoma
(
CCS
) is a high grade soft tissue sarcoma with a distinct molecular profile and with morphological features resembling those of melanoma.
CCS
has been rarely described in other locations other than the soft tissues, including the gastrointestinal tract. In this study, we report a case of
CCS
arising in the ileum of a 31-year-old woman. Histologically, the tumor involved the entire thickness of the intestinal wall. Tumor cells were polygonal or fusiform, with clear or eosinophilic cytoplasm, arranged in a uniform nested to fascicular growth pattern. Immunohistochemical studies revealed strong positivity for vimentin and S-100 protein. HMB-45, Melan-A, tyrosinase, cytokeratins,
EMA
, smooth muscle actin, CD34, CD31, CD117, CD99, synaptophysin, chromogranin A, CD56, and NSE were negative. Fluorescence in situ hybridization analysis demonstrated the presence of a t(12;22)(q13;q12) translocation, the diagnostic hallmark of
CCS
of soft parts. The present case, together with a detailed review of the literature on this topic, demonstrates that the gastrointestinal tract is a possible site of
CCS
of soft tissues and that making a reliable diagnosis of this tumor requires cytogenetic or molecular diagnostic investigations.
...
PMID:Clear cell sarcoma of the ileum: report of a case and review of literature. 1763 26
