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Desmoplastic Small Round Cell Tumor

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A rare, aggressive soft tissue sarcoma that primarily affects adolescents and young adults. It is most commonly found in the abdomen.

Also Known As:

Desmoplastic Small Cell Tumor; Desmoplastic Small Round-Cell Tumor; Desmoplastic Small-Cell Tumor; Desmoplastic Small-Cell Tumors; Small-Cell Tumor, Desmoplastic; Small-Cell Tumors, Desmoplastic; Tumor, Desmoplastic Small-Cell; Tumors, Desmoplastic Small-Cell

Networked: 93 relevant articles (2 outcomes, 3 trials/studies)

Relationship Network

Disease Context: Research Results

Neoplasms: 649836
- Neoplasms by Histologic Type: 1
  - Connective and Soft Tissue Neoplasms
    - Sarcoma: 14738
      - Desmoplastic Small Round Cell Tumor: 93

Related Diseases

1.	Rhabdomyosarcoma
2.	Neoplasms (Cancer)
3.	Ewing's Sarcoma (Sarcoma, Ewing)
4.	Neuroblastoma
5.	Primitive Neuroectodermal Tumors (PNET)

Experts

1.	Hayes-Jordan, Andrea: 3 articles (08/2015 - 01/2008)
2.	Haber, Daniel A: 3 articles (01/2008 - 12/2002)
3.	Wolden, Suzanne L: 2 articles (01/2015 - 09/2002)
4.	Modak, Shakeel: 2 articles (01/2015 - 12/2002)
5.	Kushner, Brian H: 2 articles (01/2015 - 09/2002)
6.	Petersson, Fredrik: 2 articles (01/2015 - 01/2011)
7.	La Quaglia, Michael P: 2 articles (02/2014 - 09/2002)
8.	Subbiah, Vivek: 2 articles (01/2013 - 04/2011)
9.	Anderson, Pete M: 2 articles (01/2013 - 04/2011)
10.	Ferrari, Andrea: 2 articles (09/2012 - 11/2007)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Desmoplastic Small Round Cell Tumor:

1.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 11/01/2007 - "Response to vinorelbine and low-dose cyclophosphamide chemotherapy in two patients with desmoplastic small round cell tumor." 11/01/2007 - "We report two cases of abdominal desmoplastic small round cell tumor (DSRCT) that showed a clinical response to the vinorelbine/low-dose cyclophosphamide combination that has been claimed to be effective for rhabdomyosarcoma. " 07/01/2000 - "A 5-year-old child with desmoplastic small round-cell tumor was treated with a protocol of very-high-dose, short-term chemotherapy, containing HD-CAV (cyclophosphamide, doxorubicin, vincristine, and mesna), ifosfamide, and etoposide. "
2.	vinorelbine (Navelbine)FDA LinkGeneric 11/01/2007 - "Response to vinorelbine and low-dose cyclophosphamide chemotherapy in two patients with desmoplastic small round cell tumor." 11/01/2007 - "We report two cases of abdominal desmoplastic small round cell tumor (DSRCT) that showed a clinical response to the vinorelbine/low-dose cyclophosphamide combination that has been claimed to be effective for rhabdomyosarcoma. "
3.	RNA-Directed DNA Polymerase (Reverse Transcriptase)IBA 10/01/2005 - "To study the clinicopathologic features and immunophenotype of desmoplastic small round cell tumor (DSRCT), and to assess the feasibility of reverse transcriptase-polymerase chain reaction (RT-PCR) as a diagnostic adjunct for DSRCT in routine practice. " 04/01/1996 - "Detection of the EWS/WT1 gene fusion by reverse transcriptase-polymerase chain reaction in the diagnosis of intra-abdominal desmoplastic small round cell tumor." 12/01/2009 - "We performed an endoscopic biopsy of the duodenal mass and diagnosed her disease as desmoplastic small round cell tumor using immunohistochemical staining, fluorescence in situ hybridization, and reverse transcriptase polymerase chain reaction. " 12/01/1995 - "Detection of chimeric transcripts by reverse transcriptase polymerase chain reaction is a very specific aid in differential diagnosis of developmental tumors and further establishes desmoplastic small round cell tumor as a distinct entity." 12/01/1995 - "Detection of chimeric transcripts in desmoplastic small round cell tumor and related developmental tumors by reverse transcriptase polymerase chain reaction. "
4.	Nicotinic Receptors (Nicotinic Acetylcholine Receptor)IBA 01/01/2008 - "Assessment of muscarinic and nicotinic acetylcholine receptor expression in primitive neuroectodermal tumor/ewing family of tumor and desmoplastic small round cell tumor: an immunohistochemical and Western blot study of tissue microarray and cell lines."
5.	17-(allylamino)-17-demethoxygeldanamycin (17AAG)IBA 03/15/2007 - "We conducted a phase I trial of 17-AAG in pediatric patients with recurrent or refractory neuroblastoma, Ewing's sarcoma, osteosarcoma, and desmoplastic small round cell tumor to determine the maximum tolerated dose, define toxicity and pharmacokinetic profiles, and generate data about molecular target modulation. "
6.	DesminIBA 03/01/1995 - "Desmoplastic small round cell tumor presenting in pleural fluid and accompanied by desmin-positive mesothelial cells." 01/01/2015 - "Beware of immunohistochemistry--report of a cytokeratin-, desmin- and INI-1-negative pelvic desmoplastic small round cell tumor in a 51 year old woman." 01/01/2015 - "We present a 51 year old female patient with a pelvic desmoplastic small round cell tumor with an unusual immunohistochemical profile, including absence of significant cytokeratin expression, complete negativity for desmin and widespread loss of nuclear INI-1 expression (>90% of tumor cells). " 03/01/1998 - "In the case of NB84-positive tumors other than neuroblastoma, their specific reactivity for other markers was useful (Ewing's sarcoma CD99 positive, desmoplastic small round cell tumor desmin and keratin positive). " 10/01/2004 - "Negative staining with CD99, desmin, NB84, alpha-inhibin, and TTF1 may aid in the cases in which primitive neuroectodermal tumor, rhabdomyosarcoma, intraabdominal desmoplastic small round cell tumor, neuroblastoma, a sex cord-stromal tumor, and metastatic pulmonary small cell carcinoma are in the differential. "
7.	Keratins (Keratin)IBA 01/01/2015 - "Beware of immunohistochemistry--report of a cytokeratin-, desmin- and INI-1-negative pelvic desmoplastic small round cell tumor in a 51 year old woman." 09/01/1999 - "Cytokeratin-negative desmoplastic small round cell tumor: a report of two cases emphasizing the utility of reverse transcriptase-polymerase chain reaction." 01/01/2015 - "We present a 51 year old female patient with a pelvic desmoplastic small round cell tumor with an unusual immunohistochemical profile, including absence of significant cytokeratin expression, complete negativity for desmin and widespread loss of nuclear INI-1 expression (>90% of tumor cells). " 03/01/1998 - "In the case of NB84-positive tumors other than neuroblastoma, their specific reactivity for other markers was useful (Ewing's sarcoma CD99 positive, desmoplastic small round cell tumor desmin and keratin positive). " 03/01/2010 - "The diagnosis of desmoplastic small round cell tumor was confirmed by the presence of a polyphenotypic immunoprofile (positive for cytokeratin, desmin, and neuron-specific enolase) and the characteristic EWS-WT1 gene fusion. "
8.	ParaffinIBA 12/01/2006 - "With use of fluorescence in situ hybridization, EWS break was detected in 10 of 11 paraffin blocks used and was negative in desmoplastic small round cell tumor. " 06/18/2005 - "[Detection of EWS-WT1 fusion transcripts in paraffin-embedded tissues for desmoplastic small round cell tumor]." 07/01/2000 - "Correspondence re: Kawano N, Inayama Y, Nagashima Y, Miyagi Y, Uemura H, Saitoh K, et al. Desmoplastic small round-cell tumor of the paratesticular region: report of an adult case with demonstration of EWS and WT1 gene fusion using paraffin-embedded tissue. " 07/01/1999 - "Desmoplastic small round-cell tumor of the paratesticular region: report of an adult case with demonstration of EWS and WT1 gene fusion using paraffin-embedded tissue." 03/01/2000 - "Formalin-fixed, paraffin-embedded tissue sections from 276 small round cell tumors, including 85 Ewing's sarcoma/primitive neuroectodermal tumors (ES/PNET), 52 rhabdomyosarcomas, 10 extraabdominal polyphenotypic small cell tumors, six desmoplastic small cell tumors, 11 neuroblastomas, 23 Wilms' tumors, 20 retinoblastomas, 13 esthesioneuroblastomas, and 56 small cell malignant lymphomas were stained with CD31 (JC/70A, 1:40), using a modified avidinbiotin-peroxidase complex technique, after citrate buffer microwave epitope retrieval. "
9.	Transcription Factors (Transcription Factor)IBA 12/01/2002 - "Desmoplastic small round cell tumor (DSRCT) is defined genetically by the chimeric fusion of the Ewing's sarcoma and Wilms' tumor genes, generating a novel transcription factor, EWS-WT1. " 09/01/2003 - "Desmoplastic small round cell tumor (DSRCT) is defined by a chimeric transcription factor, resulting from fusion of the N-terminal domain of the Ewing's sarcoma gene EWS to the three C-terminal zinc fingers of the Wilms' tumor suppressor WT1. " 03/01/2005 - "Other targets of the EWS-WT1 transcription factor other than PDGF-A may be directly responsible for the prominent tumor-associated desmoplasia seen in desmoplastic small round cell tumor." 09/01/1995 - "Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewing's sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1). "
10.	RNA (Ribonucleic Acid)IBA 05/01/2014 - "Total RNA from four ES cell lines plus 33 clinical samples representing ES, alveolar rhabdomyosarcoma, desmoplastic small round cell tumor, and congenital fibrosarcoma tumors was subjected to ChildSeq-RNA. " 05/01/2014 - "We developed a next-generation sequencing-based assay designated ChildSeq-RNA that uses the Ion Torrent platform to screen for EWSR1-FLI1 and EWSR1-ERG, PAX3-FOXO1 and PAX7-FOXO1, EWSR1-WT1, and ETV6-NTRK3 fusions of Ewing sarcoma (ES), alveolar rhabdomyosarcoma, desmoplastic small round cell tumor, and congenital fibrosarcoma, respectively. " 08/01/2002 - "RT-PCR was used to test tumor RNA for fusion transcripts characteristic of malignant round cell tumors (including Ewing sarcoma/primitive neuroectodermal tumor, desmoplastic small round cell tumor, and alveolar rhabdomyosarcoma), spindle cell tumors (including synovial sarcoma and congenital fibrosarcoma), and fatty tumors (myxoid liposarcoma). " 12/01/1995 - "One desmoplastic small round cell tumor was found to have a variant EWS-WT1 chimeric product that included exon 8 of EWS EWS-FLI-1 chimeric RNA was present in all Ewing's sarcoma/primitive neuroectodermal tumor and not identified in any other tumor types, including desmoplastic small round cell tumor. " 09/01/1995 - "Furthermore, six of the translocations, namely the t(11;22), t(21;22), and t(7;22) of Ewing's sarcoma, the t(12;22) of clear cell sarcoma, the t(12;16) of myxoid liposarcoma, and the t(11;22) of desmoplastic small round cell tumor, achieve this following a peculiar pattern, consisting in the fusion of a gene with an RNA-binding domain (EWS or TLS) with a transcription factor gene (FLI1, ERG, ETV1, ATF-1, CHOP, or WT1). "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 08/01/2015 - "Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy (HIPEC) is an approach for local control of desmoplastic small round cell tumor (DSRCT). " 03/01/2012 - "Metastatic desmoplastic small round cell tumor controlled by an anthracycline-based regimen: review of the role of chemotherapy." 05/01/2010 - "Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor." 04/01/2010 - "Optimal treatment with systemic chemotherapy, complete surgical excision and hyperthermic intraperitoneal chemotherapy for a desmoplastic small round cell tumor in an adult male patient." 01/01/2008 - "Outpatient and home chemotherapy with novel local control strategies in desmoplastic small round cell tumor."
2.	Radiotherapy 09/01/2002 - "Whole abdominopelvic radiotherapy for desmoplastic small round-cell tumor." 04/01/2015 - "Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option." 05/01/2005 - "The authors report the case of a 31-year-old black male diagnosed with a pelvic desmoplastic small round-cell tumor who was treated with a unique radiotherapy approach incorporating intensity-modulated radiotherapy and daily ultrasound localization to ensure accurate tumor targeting. "
3.	Combined Modality Therapy 04/01/2015 - "Multimodal treatment of abdominal and pelvic desmoplastic small round cell tumor with relative good prognosis." 01/01/2015 - "Results of multimodal treatment for desmoplastic small round cell tumor of the abdomen and pelvis." 04/01/2015 - "Abdominal desmoplastic small round cell tumor: multimodal treatment combining chemotherapy, surgery, and radiotherapy is the best option."
4.	Laparotomy 01/01/2011 - "He underwent an exploratory laparotomy and the resultant biopsy revealed desmoplastic small round cell tumor. " 12/01/2014 - "Incidental finding of desmoplastic small round cell tumor during trauma laparotomy."
5.	Immunotherapy 12/01/2002 - "Disialoganglioside GD2 and a novel tumor antigen: potential targets for immunotherapy of desmoplastic small round cell tumor." 01/01/1998 - "The focus of this review will be on the translocation events associated with Ewing's sarcomas/PNETs (ES), alveolar rhabdomyosarcoma (ARMS), malignant melanoma of soft parts (MMSP) (clear cell sarcoma), desmoplastic small round cell tumor (DSRCT), synovial sarcoma (SS), and liposarcoma (LS), and the potential for targeting the resulting chimeric proteins in novel immunotherapies."