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 described by pathologists, William L. Gerald and Juan Rosai, in 1989.[2]

Classification

Pathophysiology

Pathogenesis

Desmoplastic small round cell tumor is a highly aggressive, rare tumor of mesenchymal origin whose oncogenic effect is presumed to originate from the unique chromosomal translocation t(11;22)(p13:q12), leading to the fusion of the N-terminal domain of Ewing’s sarcoma gene EWS, to the C-terminal domain of Wilms’ tumor suppressor gene, WT1, which is found in most but not all desmoplastic small round cell tumors.[1]

Gross Pathology

Desmoplastic small round cell tumor tends to grow along a serosal lining, most commonly the peritoneal surface, but other primary sites have been described.[1]

Gallery

Causes

Differentiating Desmoplastic Small Round Cell Tumor from other Diseases

Epidemiology and Demographics

Incidence

  • Age-adjusted incidence rate of desmoplastic small round cell tumor for African Americans and Caucasians is 0.05 and 0.02 per 100,000 individuals, respectively.[1]
  • The overall age-adjusted incidence rate of desmoplastic small round cell tumor is 0.03 per 100,000 individuals, with a peak incidence of 0.074 per 100,000 individuals in the 20–24 years of age group.[1]

Age

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

Gender

Males are more commonly affected with desmoplastic small round cell tumor than females. The male to female ratio is approximately 3.57 to 1.[1]

Race

Desmoplatic small round cell tumor usually affects individuals of the African American and Caucasian race. Latin American and Asian individuals are less likely to develop desmoplatic small round cell tumor.[1]

Gallery

(a) Overall age-adjusted incidence of desmoplastic small round cell tumor, (b) Sex-based age-adjusted incidence of desmoplastic small round cell tumor. Males are more likely than females to get desmoplastic small round cell tumor, (c) Race-based, age-adjusted incidence of DSRCT. African Americans are more likely than Caucasians to get desmoplastic small round cell tumor.[1]

Demographics


Adapted from Christina K. Lettieri et al.[1]

Risk Factors

There are no established risk factors for desmoplastic small round cell tumor.[4]

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 1.3 1.4 1.5 1.6 1.7 1.8 1.9 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. 3.0 3.1 3.2 Desmoplastic small round cell tumor. Wikipedia 2016. Accessed on March 29, 2016
  4. 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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