Desmoplastic Small Round Cell Tumor: Report of 2 Cases Treated with Chemotherapy Alone or in Combination with Bevacizumab

Araújo, Rogério Agenor de; Araújo, Breno Jeha

doi:10.1159/000359997

Cited by 11 publications

(10 citation statements)

References 14 publications

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“…Until now, targeted therapy has shown its clinical benefit in IDSRCT patients who had tumor relapse or progression despite first-line or second-line treatment ( 38 ). Based on previous studies, tyrosine kinase inhibitors (TKI), including pazopanib ( 110 ), sunitinib ( 53 ), sorafenib ( 23 ), anlotinib ( 104 ), apatinib ( 76 ), imatinib ( 111 ), anti-VEGFR monoclonal antibody (e.g., bevacizumab) ( 68 ), IGF-1-R inhibitors ( 112 , 113 ), mTOR inhibitors ( 114 ), and PARP inhibitors ( 100 ) were used for the therapies and were found to be effective in select IDSRCT cases. Targeted therapy-treated patients who were reviewed from case reports/series and our hospital are listed in Table 5 .…”

Section: Treatmentsmentioning

confidence: 99%

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

et al. 2021

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Intra-abdominal desmoplastic small round cell tumor (IDSRCT) is a rare and highly malignant soft tissue neoplasm, which is characterized by rapid progression and poor prognosis. The mechanism underlying the development of this neoplasm remains elusive, but all cases are characterized by the chromosomal translocation t (11;22) (p13; q12), which results in a formation of EWSR1-WT1 gene fusion. The diagnosis of IDSRCT is often made with core-needle tissue biopsy specimens or laparoscopy or laparotomy. Immunohistochemical analyses have shown the co-expression of epithelial, neuronal, myogenic, and mesenchymal differentiation markers. FISH or reverse transcription polymerase chain reaction detecting EWS-WT1 fusion can be performed to assist in molecular confirmation. There is no standard of care for patients with IDSRCT currently, and majority of newly diagnosed patients received the aggressive therapy, which includes >90% resection of surgical debulking, high-dose alkylator-based chemotherapy, and radiotherapy. More recently, targeted therapy has been increasingly administered to recurrent IDSRCT patients and has been associated with improved survival in clinical conditions. Immunotherapy as a possible therapeutic strategy is being explored in patients with IDSRCT. In this review, we summarize currently available knowledge regarding the epidemiology, potential mechanisms, clinical manifestations, diagnosis, treatment, and prognosis of IDSRCT to assist oncologists in comprehensively recognizing and accurately treating this malignancy.

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Section: Treatmentsmentioning

confidence: 99%

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

et al. 2021

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“…Patients may suffer from abdominal distension, abdominal pain, abdominal mass, ascites, urinary tract irritation, hepatomegaly, constipation, and intestinal obstruction. [ 14 – 16 ] DSRCT primarily occurred in the chest is extremely rare. Patients may have symptoms of coughing and difficulty in breathing.…”

Section: Discussionmentioning

confidence: 99%

Mediastinal desmoplastic small round cell tumor

et al. 2020

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Rationale: Desmoplastic small round cell tumor (DSRCT) is a rare distinct tumor with a high-grade malignancy. Patient concerns: A 51-year-old male visited a local hospital in April 2016 complaining of shortness of breath, chest tightness and pain, and exhibited significant swelling in both sides of the chest. Diagnoses: CT demonstrated thoracic symmetry and no abnormalities were observed in the soft tissues of the ribs and the chest wall. A general observation of CT-guided puncture biopsy revealed 2 stripes of gray and grayish-white puncture tissues of 0.5 and 1 cm in length, respectively, and 0.1 cm in diameter. These results preliminarily suggested a (mediastinum) malignant small round cell tumor. Intervention: Given the progression of the disease, the chemotherapy regimen, consisting of ifosfamide and etoposide, was altered during the course and radiotherapy (total of 70 Gy of mediastinal Y field radiation) was conducted. Outcomes: The patient and his family declined further treatment. Through follow-up, the total survival period was determined as 17 months. Lessons: DSRCT is a rare interstitial malignant tumor. Effective cytoreduction combined with comprehensive therapies could achieve partial remission or prolong the survival of patients.

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“…In our study, a patient with liver metastasis received pazopanib in the second line, presenting progressive disease after 3 months of use. Less evidence exists for the use of sunitinib and bevacizumab, mostly based on small case series and case reports [37,38]. One ongoing trial was designed to assess the impact of the addition of irinotecan, temozolomide, and bevacizumab into conventional chemotherapy for the treatment of newly diagnosed patients [39].…”

Section: Discussionmentioning

confidence: 99%

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

Campos

Coutinho

Silva

et al. 2020

Sarcoma

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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.

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Desmoplastic Small Round Cell Tumor: Report of 2 Cases Treated with Chemotherapy Alone or in Combination with Bevacizumab

Cited by 11 publications

References 14 publications

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

Mediastinal desmoplastic small round cell tumor

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

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