Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report

Ambar, Natalia Brenneken Duarte; Alves, Maria Teresa de Seixas; Lederman, Henrique Manoel; Abib, Simone de Campos Vieira; Duarte, Adriana Maria; Caran, Eliana Maria Monteiro

doi:10.1186/s13256-019-1985-z

Cited by 9 publications

(10 citation statements)

References 16 publications

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“…DSCRT is characterized by the specific t(11;22)(p13;q12) translocation, with EWSR1-WT1 gene fusion in the majority of cases (18,19). By in situ hybridization, the identification of this characteristic fusion between exon 7 of EWSR1 and exon 8 of WT1 is therefore crucial in establishing diagnosis (19)(20)(21). EWSR1 is in fact the most frequent gene to partner with other genes to generate recurrent fusion genes in softtissue neoplasms associated with nonrandom chromosomal translocations (22).…”

Section: Discussionmentioning

confidence: 99%

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: Discussionmentioning

confidence: 99%

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

et al. 2021

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“…While response to vincristine, irinotecan, and temozolomide has been reported in DSRCT, 8,13‐18 use of all three agents has not been described for this disease in combination with interval‐compressed therapy in the upfront setting. We found that VIT has activity against DSRCT, and in our cohort, no patient experienced disease progression while on therapy.…”

Section: Discussionmentioning

confidence: 99%

“…3,4 Responses to vincristine and irinotecan in DSRCT have been reported in the published literature, and this combination is active in many sarcomas. [13][14][15][16][17][18] Temozolomide has also been of interest in DSRCT given its radiosensitizing effects. 8 The Children's Oncology Group (COG) protocol ARST08P1, which was initially developed for children with high-risk rhabdomyosarcoma, evaluated interval-compressed vincristine/doxorubicin/ cyclophosphamide (VDC) alternating with cycles of ifosfamide/ etoposide (IE), cycles of vincristine/dactinomycin/cyclophosphamide (VAC), and either cycles of vincristine/irinotecan/temozolomide (VIT, Arm P2) or vincristine/irinotecan/cixutumumab (Arm P1).…”

Section: Introductionmentioning

confidence: 99%

“…A wide range of chemotherapy regimens have been utilized for DSRCT, often based on treatment strategies for Ewing sarcoma 3,4 . Responses to vincristine and irinotecan in DSRCT have been reported in the published literature, and this combination is active in many sarcomas 13‐18 . Temozolomide has also been of interest in DSRCT given its radiosensitizing effects 8 …”

Section: Introductionmentioning

confidence: 99%

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The use of interval‐compressed chemotherapy with the addition of vincristine, irinotecan, and temozolomide for pediatric patients with newly diagnosed desmoplastic small round cell tumor

Liu

Collins

Greenzang

et al. 2020

Pediatric Blood & Cancer

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Background Desmoplastic small round cell tumor (DSRCT) is a rare aggressive sarcoma that affects children and young adults, and portends poor outcomes despite intensive multimodal treatment approaches. We report toxicity, response, and outcomes of patients with DSRCT treated with the addition of vincristine, irinotecan, and temozolomide (VIT) to interval‐compressed chemotherapy as per Children's Oncology Group ARST08P1. Methods All newly diagnosed pediatric patients with DSRCT treated at Dana‐Farber Cancer Institute and Boston Children's Hospital between 2014 and 2019 as per ARST08P1, Arm P2 with replacement of VAC cycles with VIT, were identified. Medical records were reviewed for clinical and disease characteristics, and treatment response and outcomes. Results Six patients were treated as per the above regimen. Median age at diagnosis was 15.1 years (range 3.2‐16.4) and five patients were male. Five patients had abdominal primary tumors, of which one had exclusively intraabdominal and four had extraabdominal metastases. Two initial cycles of VIT were well tolerated with nausea, vomiting, diarrhea, and constipation as the most common adverse events. Overall response rate defined as partial or complete response after two initial cycles of VIT was 50%. For local control, all patients had surgical resection followed by radiotherapy, and two patients received hyperthermic intraperitoneal chemotherapy at the time of surgery. Of the four patients who have completed therapy to date, three remain disease‐free with median follow‐up time of 46.7 months. Conclusions The addition of VIT to interval‐compressed chemotherapy is tolerable and active in DSRCT, with activity warranting additional investigation.

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“…Our patient was treated with Irinotecan. Some authors have shown that topoisomerase inhibitors, such as Irinotecan, can be effective in DSRCT [22][23][24] .…”

Section: Discussionmentioning

confidence: 99%

A testicular and paratesticular localization of Desmoplastic Small Round Cell Tumor: Is it a good prognostic factor?

Romagnol¹,

Alves²,

Oliveira³

et al. 2020

Preprint

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Irinotecan and vincristine for the treatment of refractory desmoplastic small round cell tumor in a developing country: a case report

Cited by 9 publications

References 16 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

The use of interval‐compressed chemotherapy with the addition of vincristine, irinotecan, and temozolomide for pediatric patients with newly diagnosed desmoplastic small round cell tumor

A testicular and paratesticular localization of Desmoplastic Small Round Cell Tumor: Is it a good prognostic factor?

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