Desmoplastic small round cell tumor
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
Gallery
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![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]](/images/9/9b/Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg)
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]](/index.php/File:Desmoplastic_small_round_cell_tumour_-_intermed_mag.jpg)
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
- ↑ 1.0 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.
- ↑ Pathology of desmoplastic small round cell tumor. Wikipedia 2016. https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor. Accessed on March 29, 2016
- ↑ Desmoplastic small round cell tumor. Wikipedia 2016. Accessed on March 29, 2016
- ↑ Causes of desmoplastic small round cell tumor. Wikipedia 2016. https://en.wikipedia.org/wiki/Desmoplastic_small-round-cell_tumor. Accessed on March 29, 2016