Linked Life Data

16930335

Source:http://linkedlifedata.com/resource/pubmed/id/16930335

Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
9
pubmed:dateCreated
2006-8-25
pubmed:abstractText
A 7-year-old girl was hospitalized because of a tumorous mass in her left periorbital region. The tumor was removed by local excision. The soft-part tumor recurred in the parotid gland region 4 months later, and a second recurrence was noted on the left side of the neck 3 years and 3 months thereafter. The patient had not received chemotherapy or local irradiation. Histological and immunohistochemical examinations of the recurrent masses revealed morphological characteristics of small cell proliferation with desmoplastic stroma that were similar to those of the initial tumor. The cellular components showed immunoreactivity for desmin, cytokeratin, vimentin, and epithelial membrane antigen in part, but the cells were negative for myogenin, CD99, and neuron-specific enolase. These findings suggested a diagnosis of desmoplastic small cell tumor, despite its extra-abdominal location. The histological diagnosis was confirmed by reverse transcriptase polymerase chain reaction, which demonstrated an EWS-WT1 chimeric fusion gene. An in-frame fusion of EWS exon 9 and WT1 exon 8 was subsequently identified by cloning and sequencing. The chimeric fusion gene might be related to the tissue-specific phenotype of desmoplastic small cell tumors, although further investigation of this speculation is necessary.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Sep
pubmed:issn
1320-5463
pubmed:author
pubmed:issnType
Print
pubmed:volume
56
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
543-8
pubmed:dateRevised
2011-11-17
pubmed:meshHeading
pubmed-meshheading:16930335-Child, pubmed-meshheading:16930335-Desmin, pubmed-meshheading:16930335-Diagnosis, Differential, pubmed-meshheading:16930335-Female, pubmed-meshheading:16930335-Head and Neck Neoplasms, pubmed-meshheading:16930335-Humans, pubmed-meshheading:16930335-Immunohistochemistry, pubmed-meshheading:16930335-Lymphoma, Non-Hodgkin, pubmed-meshheading:16930335-Microscopy, Electron, Transmission, pubmed-meshheading:16930335-Mucin-1, pubmed-meshheading:16930335-Neoplasm Recurrence, Local, pubmed-meshheading:16930335-Oncogene Proteins, Fusion, pubmed-meshheading:16930335-Reverse Transcriptase Polymerase Chain Reaction, pubmed-meshheading:16930335-Rhabdomyosarcoma, pubmed-meshheading:16930335-Sarcoma, Ewing, pubmed-meshheading:16930335-Soft Tissue Neoplasms, pubmed-meshheading:16930335-Vimentin
pubmed:year
2006
pubmed:articleTitle
Desmoplastic small cell tumor of soft tissue: molecular variant of EWS-WT1 chimeric fusion.
pubmed:affiliation
Department of Pathology, Shizuoka Children's Hospital, Shizuoka, Japan. mhamasan@sch.pref.shizuoka.jp
pubmed:publicationType
Journal Article, Case Reports