High-Dose Chemotherapy with Stem Cell Rescue in Desmoplastic Small Round Cell Tumor: A Single-Institution Experience and Review of the Literature

Bailey, Kayleen; Roth, Michael; Weiser, Daniel A.; Gill, Jonathan

doi:10.1155/2018/1948093

Cited by 6 publications

(13 citation statements)

References 25 publications

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“…However, HIPEC has not been shown to statistically improve survival (36). Similar findings were observed in other countries such as Canada and USA (6,30,(37)(38)(39). In our study, two out of four patients had disease amenable to CRS-HIPEC and had the longest survival compared to other patients.…”

Section: Novel Systemic Therapysupporting

confidence: 81%

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Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

et al. 2021

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Background: Desmoplastic small round-cell tumor (DSCRT) in adults is an extremely rare (age-adjusted incidence 0.3 per million) and aggressive sarcoma with limited data for optimal management. Patients and Methods: Retrospective analysis of patients with DSCRT diagnosis (2010DSCRT diagnosis ( -2020 was performed following Institutional Review Board approval. The follow-up period was from pathological diagnosis to the last patient contact. Endpoints were type of response and duration of response. Results: In the current analysis, first-line treatment in all cases was vincristine, anthracycline, and cyclophosphamide alternating with ifosfamide and etoposide (VAC-IE) with 100% response for a mean duration of 9.8 (range=5-12) months. Patients received 1-4 subsequent lines of therapy. All patients received temozolomide with irinotecan (50% partial response, duration 8-9 months). Two patients that underwent consolidative cytoreductive surgery with hyperthermic intraperitoneal chemotherapy had a longer survival (30.6 vs. 11.2 months). Patients suffered 100% mortality with a median survival was 17.8 (range=11.2-30.6) months. Conclusion: While aggressive multimodality treatment is always warranted for DSCRT, the options are limited by the multicentric presentation, short-lived initial response and lack of established subsequent therapy portending a poor prognosis. Consolidative cytoreductive surgery following first-line therapy may improve survival.Desmoplastic small round-cell tumor (DSCRT) in adults is a rare sarcoma predominantly affecting young adults, with an 3859 This article is freely accessible online.

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Section: Novel Systemic Therapysupporting

confidence: 81%

“…Unlike Ewing sarcoma, CD133+ stem cells have not yet been identified in DSCRT (27). Aggressive chemotherapy followed by stem cell therapy has been proposed but is not supported by evidence to date (30,31).…”

Section: Novel Systemic Therapymentioning

confidence: 99%

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

et al. 2021

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“…In a case report, a complete response (CR) was seen in IDSRCT patients after resection, chemotherapy (VAC+VAP), and ASCT (146). Another study also found that ASCT could significantly improve OS in DSRCT patients who were in remission (147). Moreover, a retrospective study reported improved disease-free survival and OS in ASCT-treated DSRCT patients with residual tumors (148).…”

Section: Autologous Stem Cell Transplantmentioning

confidence: 95%

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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“…It occurs mainly in adolescents, with a male to female ratio being approximately 4:1. [ 6 – 8 ] Currently, most cases of DSRCT have been reported to be found in the peritoneum, ovary, liver, kidney, pancreas, bone, skull, head, and neck, but there are also a few cases of pulmonary and pleural DSRCT reported. [ 9 – 13 ] The disease does not present specific clinical signs and symptoms.…”

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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High-Dose Chemotherapy with Stem Cell Rescue in Desmoplastic Small Round Cell Tumor: A Single-Institution Experience and Review of the Literature

Cited by 6 publications

References 25 publications

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review

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

Mediastinal desmoplastic small round cell tumor

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