Desmoplastic small round cell tumor

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]Associate Editor(s)-in-Chief: Sujit Routray, M.D. [2]

Synonyms and keywords: Desmoplastic small round cell tumour; Desmoplastic small round blue cell tumor; Desmoplastic small round blue cell tumour; Intraabdominal desmoplastic small round blue cell tumor; Desmoplastic small cell tumor; Desmoplastic cancer; Desmoplastic sarcoma; DSRCT

Overview

Desmoplastic small round cell tumor is a rare but highly fatal malignancy.[1]

Historical Perspective

Desmoplastic small round cell tumor was first discovered by pathologists, William L. Gerald and Juan Rosai, in 1989.[2]

Classification

Pathophysiology

Causes

Differentiating Desmoplastic Small Round Cell Tumor from other Diseases

Epidemiology and Demographics

=Incidence

Age-adjusted incidence rate for African Americans and Caucasians is 0.05 and 0.02 per 100,000 individuals, respectively.[1]

Age

Peak age of incidence for desmoplastic small round cell tumor is between 20 and 24 years.[1]

Risk Factors

There are no established risk factors for desmoplastic small round cell tumor.[3]

Screening

Natural History, Complications, and Prognosis

Diagnosis

History and Symptoms

Physical Examination

Laboratory Findings

X Ray

CT

MRI

Ultrasound

Other Imaging Findings]

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

References

  1. 1.0 1.1 1.2 Lettieri, Christina K.; Garcia-Filion, Pamela; Hingorani, Pooja (2014). "Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database". Journal of Cancer Epidemiology. 2014: 1–5. doi:10.1155/2014/680126. ISSN 1687-8558.
  2. Pathology of desmoplastic small round cell tumor. Wikipedia 2016. https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor. Accessed on March 29, 2016
  3. Causes of desmoplastic small round cell tumor. Wikipedia 2016. https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor. Accessed on March 29, 2016

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