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

Gallery

  • Micrograph of a desmoplastic small round cell tumor, showing the characteristic desmoplastic stroma and angulated nests of small round cells on H&E stain.[3]

    Micrograph of a desmoplastic small round cell tumor, showing the characteristic desmoplastic stroma and angulated nests of small round cells on H&E stain.[3]

  • Display of small round blue cells characteristic of desmoplastic small-round-cell tumor.[3]

    Display of small round blue cells characteristic of desmoplastic small-round-cell tumor.[3]

  • Cell exhibiting blue oval and round shapes of desmoplastic small round blue cell tumor.[3]

    Cell exhibiting blue oval and round shapes of desmoplastic small round blue cell tumor.[3]

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]

Gender

Males are more commonly affected with desmoplastic small round cell tumor than females.[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]

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 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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