scholarly journals Clinical Characteristics, Management, and Outcomes of 19 Nonpediatric Patients with Desmoplastic Small Round Cell Tumor: A Cohort of Brazilian Patients

Sarcoma ◽  
2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Fernando Campos ◽  
Daniel L. Coutinho ◽  
Maria Letícia G. Silva ◽  
Ademar Lopes ◽  
Antônio Nascimento ◽  
...  
Keyword(s):  
Multimodality Treatment ◽  
Cell Tumor ◽  
Rank Test ◽  
Cancer Center ◽  
Round Cell ◽  
Imaging Data ◽  
Young Males ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive mesenchymal malignancy, usually affecting young males. There is no consensus on the best therapeutic approach. We seek to characterize a cohort of nonpediatric patients with DSRCT treated at a large Brazilian cancer center. We performed a retrospective analysis of patients with histologically confirmed DSRCT referred to our institution (2007–2020). Clinical and imaging data were extracted and summarized with descriptive statistics. Survival analyses were conducted by the Kaplan–Meier method and compared with the log-rank test. We included 19 patients with DSRCT, the median age at diagnosis was 26 years (range: 15–41 years), and 68% were male. Ninety percent presented with abdominopelvic masses, and 32% had extra-abdominal metastasis at diagnosis. Eleven patients (58%) underwent surgery, four patients (21%) received whole abdominal adjuvant radiotherapy, and five patients (26%) had hyperthermic intraperitoneal chemotherapy. Median OS was 27 months (interquartile range: 18–51 m). The five-year OS rate was 12%. Our data confirm the aggressiveness of DSRCT despite intense multimodality treatment. Outcomes of patients treated in a reference cancer center in a developing country are similar to cancer centers in developed nations. Multicenter cooperation is urgent to the development of clinical trials and to improve diagnosis and treatment efficacy.

Survival and toxicity following sequential multimodality treatment including whole abdominopelvic radiotherapy for patients with desmoplastic small round cell tumor

2016 ◽  
Vol 119 (1) ◽  
pp. 40-44 ◽  
Author(s):  
Eleanor Marshall Osborne ◽  
Tina Marie Briere ◽  
Andrea Hayes-Jordan ◽  
Lawrence B. Levy ◽  
Winston W. Huh ◽  
...  
Keyword(s):  
Multimodality Treatment ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Peritoneal metastatic desmoplastic small round cell tumor: A single institution experience.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. e22541-e22541
Author(s):  
Juliana Souza ◽  
Gelcio L. Q. Mendes
Keyword(s):  
Median Number ◽  
Cell Tumor ◽  
High Morbidity ◽  
Round Cell ◽  
Aggressive Approach ◽  
Female Ratio ◽  
Young Males ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

e22541 Background: Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive tumor that usually occurs in young males. The treatment remains a challenge and despite multiple strategies the tumor leads to death in most cases. Methods: Patients with peritoneal and metastatic DSRCT treated at or referred to National Cancer Institute of Brazil with histological documented diagnosis between July 1997 and September 2014 were identified and their medical records were analyzed. Results: There were 17 patients, the median age of diagnosis was 14.8 years (range: 4-49), with male-female ratio of 3.25. The most common sites of metastasis were liver (n: 7; 53.8%), lung (n: 6; 35%) and both (n: 2; 11.7%). Eight patients (47%) received radiotherapy and 15 patients (88.2%) received chemotherapy, 13 received vincristine, cyclophosphamide, doxorubicin and regimen with or without ifosfamide and etoposide. The median number of cycles received was 12.9 (range:1-27). Three patients (17.6%) who underwent to surgery, received the three treatments. Two patients received only palliative care. After median follow-up of 74 months, 13 patients (76.4%) died, nine patients during the first 2 years. The median overall survival was 16 months. One 6-year old patient was alive after 74 months, after chemotherapy, radiotherapy and surgery. Conclusions: The DSCRT is a rare disease with poor prognosis and high morbidity, that demand an aggressive approach. However, most patients are diagnosed with advanced disease, receiving noncurative approach.

scholarly journals Multimodality Treatment of Desmoplastic Small Round Cell Tumor: Chemotherapy and Complete Cytoreductive Surgery Improve Patient Survival

2018 ◽  
Vol 24 (19) ◽  
pp. 4865-4873 ◽  
Author(s):  
Vivek Subbiah ◽  
Salah-Eddine Lamhamedi-Cherradi ◽  
Branko Cuglievan ◽  
Brian A. Menegaz ◽  
Pamela Camacho ◽  
...  
Keyword(s):  
Cytoreductive Surgery ◽  
Patient Survival ◽  
Multimodality Treatment ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Improve Patient Survival ◽  
Small Round Cell ◽  
Improve Patient

Optimizing the therapy of desmoplastic small round cell tumor: Combined experience from the two major cancer centers.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10021-10021
Author(s):  
Vivek Subbiah ◽  
Aaron D Viny ◽  
Peter Meade Anderson ◽  
Andrea Anita Hayes-Jordan ◽  
Wei Qiao ◽  
...  
Keyword(s):  
Overall Survival ◽  
Univariate Analysis ◽  
Cell Tumor ◽  
Cancer Center ◽  
Cell Transplant ◽  
Round Cell ◽  
Cancer Centers ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

10021 Background: Desmoplastic small round cell tumor (DSRCT) is a rare translocation-positive (EWS-WT1)sarcoma subtype that often presents as diffuse peritoneal sarcomatosis in male adolescents and young adults. The objective of this study was to assess the survival of patients with DSRCT from the two major cancer centers. Methods: A multi-center retrospective review was performed of patients diagnosed with DSRCT treated at The University of Texas MD Anderson Cancer Center (MDACC) and Memorial Sloan-Kettering Cancer Center (MSKCC). Results: Of the 197 pts evaluated at both cancer centers (109 from MSKCC and 88 from MDACC) 87 % were male. The mean age was 25 ± 11 years; 139 (70.6%) underwent surgery; 38 ( 19.6%) had debulking surgery, 30 received radiation therapy, and 27 underwent hyperthermic chemotherapy after debulking (CHPP), 11 had a stem cell transplant. 112 patients were seen after 2003 in both the centers vs 85 before 2003. Neoadjuvant chemotherapy included Ewing’s-like chemotherapy with MSKCC’s P6 regimen (<18 yrs of age) and VAI in adults. We further analyzed the MDACC cohort. In univariate analysis radiotherapy, surgery, CHPP, removal of primary mass + metastases, age < 30 and patients treated after 2003 were associated with significantly improved overall survival. Multi-variate analysis showed that patients treated after 2003 had 59% lesser hazard (HR=0.41) than those treated earlier (p=0.04). Comparing 1990-2003 vs. 2004-2010 survival >3 yr with DSRCT was <25 % and is now >50 %, a significant improvement. Conclusions: We present long-term outcomes for the largest series of patients diagnosed with DSRCT. A multidisciplinary approach to DSRCT treatment is required to prolong 3- and 5-yr overall survival. Our recent improved outcomes required not only aggressive chemotherapy but also regional local control measures. Best adjuvant therapy remains to be determined. A treatment algorithm will be proposed.

Primary Desmoplastic Small round Cell Tumor of the Kidney: A Case Report in a 14-Year-Old Girl with Molecular Confirmation

2007 ◽  
Vol 10 (4) ◽  
pp. 320-324 ◽  
Author(s):  
Sophie Collardeau-Frachon ◽  
Dominique Ranchère-Vince ◽  
Olivier Delattre ◽  
Stelly Hoarau ◽  
Philippe Thiesse ◽  
...  
Keyword(s):  
Fusion Transcript ◽  
Cell Tumor ◽  
Round Cell ◽  
Young Males ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Molecular Features ◽  
The Subject ◽  
Round Cell Tumors

We report a case of desmoplastic small round cell tumor (DSRCT) arising in the kidney of a 14-year-old female. The subject presented with gross hematuria. Medical imaging uncovered a left renal mass without regional or metastatic extension. The tumor showed morphological, immunohistochemical, and molecular features of DSRCT. Immunostaining revealed polyphenotypic differentiation. Molecular analysis detected the fusion transcript resulting from the t(11;22)(p13;q12) reciprocal translocation, which characterized this neoplasm. Desmoplastic small round cell tumor is a rare, aggressive neoplasm that mainly affects young males and that usually presents with widespread abdominal serosal involvement. This unusual localization should lead one to consider this tumor in the differential diagnosis of small blue round cell tumors of the kidney.

scholarly journals Extensive Abdominopelvic Desmoplastic Small Round Cell Tumor — Case Report and Review of the Literature

2016 ◽  
Vol 1 (2) ◽  
pp. 189-192
Author(s):  
Adrienne Horváth ◽  
Zsuzsanna Erzsébet Papp
Keyword(s):  
Clinical Status ◽  
Correct Diagnosis ◽  
Cell Tumor ◽  
Adolescent Male ◽  
Pelvic Cavity ◽  
Round Cell ◽  
Young Males ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

AbstractDesmoplastic small round cell tumor (DSRCT) is a rare malignant tumor, which affects mostly young males and has a poor prognosis. Since 1991, when it was first described as a distinct clinical entity by Gerald WL and Rosai J, some 200 cases were reported. DSRCT arises mainly from the abdominal and pelvic cavity, causes abdominal pain or discomfort, weight loss, urinary, bowel or bile obstruction due to compression. Metastases appear most frequently in the liver and lungs. Multimodal therapy is usually indicated with chemotherapy, surgery, radiotherapy and autologous stem cell transplantation. DSRCT should be differentiated from other small, blue round cell tumors, especially hematopoietic malignancies (leukemia, lymphoma), neuroblastoma, Ewing sarcoma, PNET, rhabdomyosarcoma, malignant mesothelioma, small cell carcinoma and Wilms tumors. We report the case of a patient with an extensive abdominopelvic desmoplastic small round cell tumor, with liver metastases, in an adolescent male patient, highlighting the alert deterioration of the clinical status of the patient after the biopsy, the need for a second review of the histopathological material in order to obtain a correct diagnosis, the chemoresistance of the tumor despite an apparently good clinical status, and the severe prognosis of this type of tumor.

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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