Diffuse Intraabdominal Desmoplastic Small Round Cell Tumor: A Ten-Year Experience

Livaditi, E.; Mavridis, G.; Soutis, Michael; Papandreou, Evangelos; Moschovi, Maria; Papadakis, V.; Stefanaki, Kalliopi; Christopoulos-Geroulanos, George

doi:10.1055/s-2006-924736

Cited by 30 publications

(20 citation statements)

References 19 publications

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“…A chromosomal translocation, t (11,22)(p13;q12), involving the EWS and the WT1 genes is highly specific for this tumor. 1 Despite aggressive multimodal treatment, few long-term survivors are reported in most reports.…”

Section: Introductionmentioning

confidence: 99%

Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor

Bisogno

Ferrari

Rosolen

et al. 2009

Bone Marrow Transplant

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The RMS4.99 study was designed to explore the role of early sequential intensified chemotherapy (SICT) with PBSC rescue in patients with soft tissue sarcoma with a poor prognosis. Fourteen patients with desmoplastic small round-cell tumor (DSRCT) were included in this study. Initial chemotherapy was followed by a course of CY and etoposide with subsequent PBSC harvest, then three consecutive intensified chemotherapy combinations followed by PBSC rescue and G-CSF administration: first cycle thiotepa (150 mg/m(2) x 2 on day 1) and melphalan (60 mg/m(2) on day 2), second cycle CY (2 g/m(2) on days 1 and 2) and thiotepa (150 mg/m(2) x 2 on day 3), third cycle melphalan (80 mg/m(2) on day 1). The interval between cycles had to be kept as short as possible. Then patients underwent surgery or radiotherapy or both, after which six courses of vincristine, actinomycin D, CY were administered. Ten patients received SICT, which was well tolerated. With a median follow-up of 27 months only three patients are alive without evidence of disease. The 3-year event-free and overall survival rates were 15.5 and 38.9%, respectively. The prognosis for pediatric patients with DSRCT did not improve after administering intensified chemotherapy early in their treatment, so different strategies are needed.

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Section: Introductionmentioning

confidence: 99%

Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor

Bisogno

Ferrari

Rosolen

et al. 2009

Bone Marrow Transplant

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“…Similarly, Lal in its cohort has reported a significant impact of surgery on overall survival with 3 year survival of 58% as compared to 0% in no resectable disease [15], the benefit is greater after complete resection but frequently this act is not feasible due to the extensive involvement as the case of our patient. In contrast, other authors have concluded that surgery has no significant benefit on survival [16,17].…”

Section: Discussionmentioning

confidence: 86%

Desmoplastic small round cell tumor of the abdomen: A case report and literature review of therapeutic options

Benhammane¹,

Chbani²,

Ousadden³

et al. 2012

Health

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Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive variety of sarcoma arising typically from abdominal or pelvic peritoneum. Diagnosis and treatment approaches of this entity are complex and require a skilled, experienced, multidisciplinary team. Authors report their experience with a case of an intra-abdominal DSRCT arising in a 37-year-old young man in order to discuss the clinico-pathological and radiological behavior of this tumor and underline diagnostic and therapeutic difficulties.

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“…Hassan et al [4] reviewed 12 patients with a DSRCT that were treated in their institution from 1999 to 2001; the median age was 26 years; patients who received complete resection had a median survival of 34 months, compared with 14 months for patients who did not receive surgery. However, in a study by Livaditi et al [5], they analyzed five patients with DSRCT with a median age of 9.6 years and every patient who received surgery relapsed within 2-6 months. We believe that surgery should always be attempted, even when only palliative surgery is feasible, because debulking plays a very important role in the control of local symptoms.…”

Section: Discussionmentioning

confidence: 99%

Activity of trabectidin in desmoplastic small round cell tumor

et al. 2010

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Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive cancer that typically arises in teenage boys. As a result of its low prevalence, there have been few studies of its treatment and impact on survival. Here, we report the case of a patient who was refractory to multiple drugs but remains stable with trabectedin. To our knowledge, this is the first published report that supports antitumoral activity of trabectedin in DSRCT.

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Diffuse Intraabdominal Desmoplastic Small Round Cell Tumor: A Ten-Year Experience

Cited by 30 publications

References 19 publications

Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor

Sequential intensified chemotherapy with stem cell rescue for children and adolescents with desmoplastic small round-cell tumor

Desmoplastic small round cell tumor of the abdomen: A case report and literature review of therapeutic options

Activity of trabectidin in desmoplastic small round cell tumor

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