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

Osborne, E.M.; Briere, Tina Marie; Hayes‐Jordan, Andrea; Levy, Lawrence B.; Huh, Winston W.; Mahajan, Anita; Anderson, Peter M.; McAleer, Mary Frances

doi:10.1016/j.radonc.2015.10.016

Cited by 43 publications

(52 citation statements)

References 16 publications

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“…The advent of intensity-modulated radiation therapy (IMRT) has resulted in a significant reduction in radiotherapy-related morbidity specifically gastrointestinal and hematologic toxicity 31 . However, groups at both MSKCC (IMRT after debulking surgery) and MD Anderson (IMRT after debulking surgery and HIPEC) have reported sub-optimal disease-free survival of <1 year 31, 32 . Most relapses were intraperitoneal, suggesting that intraperitoneal therapy in addition to conventional surgery, chemotherapy and radiotherapy is necessary to improve outcome.…”

Section: Radiotherapymentioning

confidence: 99%

Management of desmoplastic small round cell tumor

Hayes‐Jordan

LaQuaglia

Modak

2016

Seminars in Pediatric Surgery

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107

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Desmoplastic small round cell tumor (DSRCT) is a soft tissue sarcoma of mesenchymal cell origin that typically presents with multiple intra-abdominal tumors and exhibits a multi-phenotypic pattern of immunohistochemical staining. The specific organ or tissue type of origin has yet to be identified. DSRCT rarely arises as a singular tumor in the abdomen; in most cases, there are dozens to hundreds of abdominal peritoneal tumors that are detected on diagnosis. One very large dominant mass is usually present in the omentum, with an additional one or two large conglomerates of tumors in the pelvis and right peritoneum, respectively. Despite an often overwhelmingly large number of abdominal tumors, symptoms of bowel obstruction are rare. Ascites may be present. In late stages, pleural effusions, pleural implants, mediastinal adenopathy, supraclavicular adenopathy, or bone metastasis may be present. With this challenging disease, multidisciplinary therapy, including aggressive surgery, is warranted. This review will address DSRCT biology and treatment options and discuss outcomes.

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Section: Radiotherapymentioning

confidence: 99%

Management of desmoplastic small round cell tumor

Hayes‐Jordan

LaQuaglia

Modak

2016

Seminars in Pediatric Surgery

Self Cite

107

View full text Add to dashboard Cite

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“…Patients are managed with aggressive multimodal therapy, including neoadjuvant chemotherapy, maximal surgical debulking, intraperitoneal chemotherapy in some cases, adjuvant whole abdominopelvic radiation therapy, and stem cell or bone marrow transplant [1], [4], [5], [6], [7], [8]. Patients receiving trimodality therapy with chemotherapy, surgical resection, and radiation therapy have improved 3-year survival (55%) compared with patients who are not treated with all three modalities (27%) [1]; however, outcomes remain poor despite aggressive treatment.…”

Section: Discussionmentioning

confidence: 99%

“…A more recent study from M.D. Anderson does suggest potentially greater survival with improved salvage therapies, with a median overall survival of 60 months despite a median disease-free survival of 10 months [8].…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic small round cell tumor: postoperative retroperitoneal mass

Shen

Loeb

Terezakis

2016

Radiology Case Reports

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We describe the case of a 14-year-old boy who presented with a large, 17.6-cm retroperitoneal mass, along with multiple metastases, and was diagnosed with desmoplastic small round cell tumor. After initial chemotherapy, he underwent gross total resection with a positive margin. On postoperative radiation planning computed tomography, a 6.8-cm heterogeneous mass was noted in the surgical bed. Given the tumor's aggressive nature and positive surgical margins, there was real concern for recurrent disease. Further evaluation with magnetic resonance imaging elucidated that the mass consisted of simple fluid and fat, without contrast enhancement, suggesting a postoperative fluid collection. He was able to continue with adjuvant treatment as planned. This case example illustrates that even large postoperative heterogeneous masses may still be related to postoperative fluid collection in patients with aggressive tumor. However, it is important to rule out recurrent disease before starting adjuvant therapy given improved outcomes with gross total resection in desmoplastic small round cell tumor.

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“…Whole‐abdominal radiation (28‐30 Gy) has been applied to patients with DSRCT with peritoneal disease after complete cytoreductive surgery. Recent multivariate analysis has shown that whole‐abdominal radiation does not improve overall survival, but it appears to delay extrahepatic intra‐abdominal tumor recurrence and may improve quality of life for selected patients 15 . These endpoints must be balanced by the potential associated adverse effects.…”

Section: Principles Of Radiotherapymentioning

confidence: 99%

The Chicago Consensus on peritoneal surface malignancies: Management of desmoplastic small round cell tumor, breast, and gastrointestinal stromal tumors

Izquierdo¹,

Plana²,

Schuitevoerder³

et al. 2020

Cancer

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The Chicago Consensus Working Group provides multidisciplinary recommendations for the management of desmoplastic small round cell, breast, and gastrointestinal stromal tumors specifically related to peritoneal surface malignancy. These guidelines are developed with input from leading experts, including surgical oncologists, medical oncologists, pathologists, radiologists, palliative care physicians, and pharmacists. These guidelines recognize and address the emerging need for increased awareness in the appropriate management of peritoneal surface disease. They are not intended to replace the quest for higher levels of evidence.

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

Cited by 43 publications

References 16 publications

Management of desmoplastic small round cell tumor

Management of desmoplastic small round cell tumor

Desmoplastic small round cell tumor: postoperative retroperitoneal mass

The Chicago Consensus on peritoneal surface malignancies: Management of desmoplastic small round cell tumor, breast, and gastrointestinal stromal tumors

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