Reduced Toxicity With Intensity Modulated Radiation Therapy (IMRT) for Desmoplastic Small Round Cell Tumor (DSRCT)

2012 ◽  
Vol 84 (3) ◽  
pp. S169
Author(s):  
N.B. Desai ◽  
K.M. Alektiar ◽  
N.F. Stein ◽  
M.P. LaQuaglia ◽  
B.H. Kushner ◽  
...  
Keyword(s):  
Radiation Therapy ◽  
Intensity Modulated Radiation Therapy ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Intensity Modulated ◽  
Reduced Toxicity

scholarly journals Incidence and Outcomes of Desmoplastic Small Round Cell Tumor: Results from the Surveillance, Epidemiology, and End Results Database

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Christina K. Lettieri ◽  
Pamela Garcia-Filion ◽  
Pooja Hingorani
Keyword(s):  
Radiation Therapy ◽  
Cohort Analysis ◽  
Large Population ◽  
Incidence Rates ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Significant Difference

Desmoplastic small round cell tumor (DSRCT) is a rare but highly fatal malignancy. Due to the rarity of this neoplasm, no large population based studies exist.Procedure. This is a retrospective cohort analysis. Incidence rates were calculated based on sex and ethnicity and compared statistically. Gender-, ethnicity-, and treatment- based survival were calculated using the Kaplan-Meier method.Results. A total of 192 cases of DSRCT were identified. Peak incidence age was between 20 and 24 years. Age-adjusted incidence rate for blacks was 0.5 cases/million and for whites was 0.2 cases/million (P=0.037). There was no statistically significant difference in survival based on gender or ethnicity. When adjusted for age, there was no statistically significant difference in survival amongst patients who received radiation therapy compared to those who did not (HRadj = 0.73; 95% CI 0.49, 1.11). There was a statistically significant survival advantage for patients who received radiation after surgery compared to those who did not (HR 0.49; 95% CI 0.30, 0.79).Conclusion. DSRCT is more common in males and in people of African-American descent. Although overall survival remains poor, radiation therapy following surgery seems to improve outcome in these patients.

Treatment of Desmoplastic Small round Cell Tumor with Image-Guided Intensity Modulated Radiation Therapy as a Component of Multimodality Treatment

2005 ◽  
Vol 91 (3) ◽  
pp. 253-255 ◽  
Author(s):  
Christopher D Jahraus ◽  
Shawn D Glisson ◽  
William H St Clair
Keyword(s):  
Intensity Modulated Radiotherapy ◽  
Cell Tumor ◽  
Treatment Modalities ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Ultrasound Localization ◽  
Small Round Cell ◽  
Image Guided ◽  
Intensity Modulated

Aims and background 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. Desmoplastic small round-cell tumor is a very rare tumor, most commonly presenting in the abdominopelvic regions of adolescents and young adults. It has generally been associated with a very poor prognosis. Methods The patient initially underwent biopsy of the mass and omentectomy for mesenteric implants followed by chemotherapy. Chemotherapy resulted in tumor shrinkage and was followed by a second-look laparotomy. Additional omental nodules were resected, but the primary tumor was adherent to the rectum and seminal vesicles. A radiation oncology consult was obtained, and a course of image-guided intensity-modulated radiotherapy ensued with daily ultrasound localization. This resulted in shrinkage of local disease, but a new lesion was identified. The patient was subsequently started on further chemotherapy, which has maintained the disease in a stable state for several months. Conclusions Image-guided intensity-modulated radiotherapy is a feasible option in the treatment of pelvic desmoplastic small round-cell tumor. Such therapy may permit escalation of conventional radiotherapy doses and could have a favorable impact on local control of disease. Confirmation of this belief will require additional data in the form of case reports like this. Pending such confirmation, we continue to be of the impression that desmoplastic small round-cell tumor has an overall unfavorable prognosis, regardless of treatment modalities employed.

Desmoplastic small round cell tumor showing solid proliferation with limited desmoplasia and confusing immunohistochemical findings: an autopsy report

2020 ◽  
Vol 53 (3) ◽  
pp. 177-182
Author(s):  
Atsushi Kihara ◽  
Kazuya Takahashi ◽  
Ayataka Ishikawa ◽  
Yusuke Amano ◽  
Daisuke Matsubara ◽  
...  
Keyword(s):  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Autopsy Report

scholarly journals FDG PET/CT imaging of desmoplastic small round cell tumor: findings at staging, during treatment and at follow-up

2015 ◽  
Vol 45 (9) ◽  
pp. 1308-1315 ◽  
Author(s):  
Austin Ostermeier ◽  
M. Beth McCarville ◽  
Fariba Navid ◽  
Scott E. Snyder ◽  
Barry L. Shulkin
Keyword(s):  
Fdg Pet ◽  
Ct Imaging ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Pet Ct ◽  
Fdg Pet Ct

scholarly journals Desmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review

ORL ◽  
2021 ◽  
pp. 1-6
Author(s):  
Qingjiao Li ◽  
Xiaolu Yuan
Keyword(s):  
Submandibular Gland ◽  
Young Patients ◽  
Neck Region ◽  
Cell Tumor ◽  
Pelvic Cavity ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
The Right

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressively malignant tumor mostly occurring in the abdominal and pelvic cavity of young patients. However, few cases had been reported concerning DSRCT occurring in the head and neck region. We presented a rare case of DSRCT of the right submandibular in a 25-year-old man. MRI revealed a 3 × 2-cm solid nodule located in the right submandibular, and physical examination showed no other occupying lesion elsewhere. Histologically, the tumor was composed of various-sized small round cell nests, embedded in an abundant desmoplastic stroma. Immunohistochemically, the tumor cells were typically positive for epithelial (CK and EMA), mesenchymal (vimentin and desmin), and neuroendocrine (CD56, NSE, Syn, and CgA) markers, but negative for WT1. Fluorescence in situ hybridization revealed the presence of a break apart involving the <i>Ewing sarcoma</i> (<i>EWS</i>) gene. The patient received chemotherapy and radiotherapy and relapsed after 19 months of follow-up. DSRCT of the submandibular gland is rare, and the diagnosis of this tumor in an uncommon location relies on the histomorphology, immunophenotype, and <i>EWS</i> gene translocation detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.

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