Abdominal Desmoplastic Small Round Cell Tumor: Multimodal Treatment Combining Chemotherapy, Surgery, and Radiotherapy is the Best Option

Honoré, Charles; Amroun, Kocéila; Vilcot, Laurence; Mir, Olivier; Dômont, Julien; Terrier, Philippe; Cesne, Axel Le; Péchoux, C. Le; Bonvalot, Sylvie

doi:10.1245/s10434-014-4123-6

Cited by 87 publications

(158 citation statements)

References 39 publications

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“…This tumor is somewhat alkylator-sensitive, and response seems dose-responsive [31]. Unfortunately, durable responses are limited, and the prognosis for patients remains poor [32]. Median survival ranges from 14 to 25 years [14] with some reports with patients as young as 5 months [33].…”

Section: Discussionmentioning

confidence: 99%

“…Median survival ranges from 14 to 25 years [14] with some reports with patients as young as 5 months [33]. 3-year overall survival is dismal at around 44% and the 5-year survival rate a dismal 15% [8] with a slightly higher survival reported among those undergoing debulking including 1-2 mm implants [34] of at least 90% of tumor absence of extraperitoneal metastasis [32]. Radiotherapy to high-risk sites, and myeloablative chemotherapy with stem-cell rescue is applied in selected cases especially those with residual disease [31].…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Feasibility of Administering A Modified Outpatient Regimen of Vincristine, Doxorubicin, Cyclophosphamide Alternating with Ifosfamide, and Etoposide (VAC/IE) For High-Risk Desmoplastic Small Round Blue Cell Tumor and the Role of Tumor Profiling in Selecting Treatments

Bulbul¹

2017

JCPCR

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Feasibility of Administering A Modified Outpatient Regimen of Vincristine, Doxorubicin, Cyclophosphamide Alternating with Ifosfamide, and Etoposide (VAC/IE) For High-Risk Desmoplastic Small Round Blue Cell Tumor and the Role of Tumor Profiling in Selecting Treatments

Bulbul¹

2017

JCPCR

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“…To date, the optimal management of DSRCT remains challenging and current schemes lack a significant cure rate despite the use of aggressive treatments (15). Chemoradiotherapy is significant in the treatment of advanced abdominal DSRCT.…”

Section: Case Reportmentioning

confidence: 99%

Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy: A case report

Zhang

et al. 2015

Oncology Letters

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Abstract. Desmoplastic small round cell tumor (DSRCT) is a rare tumor that mainly affects adolescents, and typically involves the abdominal and pelvic peritoneum. The present study reports one case of giant DSRCT, treated with concurrent chemoradiotherapy, and reviews the available medical literature. A 38-year-old man presented with a 3-month history of pain in the left lower abdomen and nausea, associated with decreased appetite and weight loss. Computed tomography (CT) showed a 12.3x7.9 cm confluent solid mass in the lower abdomen and pelvic cavity. The patient underwent exploratory laparotomy and the final pathological diagnosis was DSRCT. Following laparotomy, the patient was treated with external beam radiotherapy to the whole abdomen and pelvis to a dose of 40 Gy plus a 20 Gy boost to the residual disease. The results indicated that synchronous chemotherapy with cyclophosphamide, adriamycin and cisplatin combined with radiotherapy significantly improved locoregional control of DSRCT and a complete response, as measured by CT assessment 2 months subsequent to radiotherapy. In conclusion, DSRCT is a rare malignancy requiring multidisciplinary treatment, including surgery, chemotherapy and radiotherapy. The results of the present study confirm that radiotherapy has a significant role in the treatment of advanced abdominal DSRCT and may contribute to durable remission. IntroductionDesmoplastic small round cell tumor (DSRCT), a rare, high-grade malignant neoplasm, which mainly affects young men and adolescents, was first described as a mesenchymal entity with distinct clinicopathological features by Gerald and Rosai in 1989 (1). It has been reported that 95% of DSRCTs are located in the abdominal cavity and pelvis, while <5% of cases may affect other organs (2). Diagnosis is typically based on the histological and immunohistochemical features of the tumor. A specific chromosomal translocation, t(11;22) (p13;q12), has been identified in DSRCT and is increasingly used to confirm diagnosis (3). The prognosis for patients with DSRCT remains very poor, particularly for those with metastatic diseases (3-year survival rate, 29%; 5-year survival rate, 18%) (4). The standard treatment protocol for DSRCT has not been well established.The present study reports a case in which this uncommon tumor was too large to be completely resected, and therefore concurrent radiotherapy and chemotherapy [consisting of cyclophosphamide, doxorubicin and cisplatin (CAP)] were utilized. This case is reported with the goal of contributing valuable information about this unusual tumor to the current literature. Case reportIn May 2014, a 38-year-old Chinese man was admitted to Shandong Cancer Hospital and Institute, with a 3-month history of pain in the lower left abdomen, and nausea associated with decreased appetite and weight loss. There was no history of fever or jaundice, and the patient had no relevant personal or family history of malignancy.On physical examination, abdominal distension with shifting dullness was identified. Ultrasono...

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“…It has a predilection for the serosal surface, most often the abdominal and pelvic cavity. At the time of presentation, up to 50% of the patients may have extra-peritoneal metastasis and even higher rates of local metastasis (2)(3)(4)(5). The most common presenting symptom is abdominal pain, followed by abdominal distention, abdominal mass, and hepatomegaly (4,5).…”

Section: Introductionmentioning

confidence: 99%

“…There is also no reported data on the management of liver metastasis from this disease. Multimodal management including cytoreductive surgery (CRS), chemotherapy, and radiotherapy has been shown to be effective in the treatment of DSRCT (2,3). Also, recent studies have reported the benefit from hyperthermic intraperitoneal chemotherapy (HIPEC) in this disease for intraperitoneal metastatic disease (6).…”

Section: Introductionmentioning

confidence: 99%

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy combined with two-stage hepatectomy for multiple and bilobar desmoplastic small round cell tumor liver metastases

Cracco

Roy

Simpfendorfer

2017

J. Gastrointest. Oncol.

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Desmoplastic small round cell tumor (DSRCT) is a rare mesenchymal tumor usually affecting young patients. Local dissemination is common, and liver is the most common site for extraperitoneal metastases. Multimodal management has been shown to be the most effective treatment. Some authors consider liver metastases especially bi-lobar disease as a contraindication for surgical resection. We present a case of a DSRCT with bi-lobar metastases in an adult patient who underwent multi-modal management along with hepatectomy. A 51-year-old man was found to have a large intraperitoneal mass with bi-lobar liver metastases during work up for new onset reflux and abdominal pain. Biopsy confirmed it as DSRCT.The patient was treated with multi-modal therapy including cytoreductive surgery (CRS), hyperthermic intraperitoneal chemotherapy (HIPEC), two-stage hepatectomy (TSHP) combined with ( 90 Y) Yittrium-90 radioembolization of the right hepatic lobe and systemic chemotherapy. The patient had a right-sided pleural empyema one month after the surgery for which he required right-sided video-assisted thoracoscopy and decortication. He remains disease free at 2 years follow-up. DSRCT with bi-lobar liver metastases are best managed with multimodal therapy. TSHP seems to be a feasible and safe option in selected cases, with a potentially good outcome. The liver is the most common site of extra-peritoneal metastasis. To our knowledge, there is no published data regarding surgical management of metastatic bi-lobar hepatic lesions from DSRCT. In this report, we present a case of an abdominal DSRCT in an adult patient with peritoneal and hepatic metastatic disease. The patient received a multimodal treatment, including CRS, HIPEC and two-stage hepatectomy (TSHP). Case presentationA 51-year-old male presented with a history of new onset reflux and intermitted mild abdominal pain of nondescript origin in October 2014. His liver function tests and tumor markers including alpha-fetoprotein and carcinoembryonic antigen were within normal limit. A computer tomography (CT) scan showed a large tumor in the left upper abdomen with multiple bi-lobar liver metastases (Figure 1). A biopsy of the abdominal mass was consistent with a DSRCT of the peritoneal cavity. Given these findings, the patient was treated with multi-modal therapy including CRS, HIPEC, TSHP combined with ( 90 Y) Yittrium-90 radioembolization of the right hepatic lobe and systemic chemotherapy. After informed consent, the patient was taken to the operating room in November 2014. A large mass was appreciated in the left upper quadrant over the splenic flexure of the colon extending towards the tail of the pancreas and multiple lesions along the colon extending towards the pelvis. There were multiple lesions in the right lobe of the liver with two lesions noted in the left lateral segment corresponding to the CT images. CRS with tumor debulking and excision of peritoneal metastases and mesenteric metastases was performed. This included an en bloc resection of the tumor to...

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Abdominal Desmoplastic Small Round Cell Tumor: Multimodal Treatment Combining Chemotherapy, Surgery, and Radiotherapy is the Best Option

Cited by 87 publications

References 39 publications

Feasibility of Administering A Modified Outpatient Regimen of Vincristine, Doxorubicin, Cyclophosphamide Alternating with Ifosfamide, and Etoposide (VAC/IE) For High-Risk Desmoplastic Small Round Blue Cell Tumor and the Role of Tumor Profiling in Selecting Treatments

Feasibility of Administering A Modified Outpatient Regimen of Vincristine, Doxorubicin, Cyclophosphamide Alternating with Ifosfamide, and Etoposide (VAC/IE) For High-Risk Desmoplastic Small Round Blue Cell Tumor and the Role of Tumor Profiling in Selecting Treatments

Complete response of giant desmoplastic small round cell tumor treated with chemoradiotherapy: A case report

Cytoreductive surgery and hyperthermic intraperitoneal chemotherapy combined with two-stage hepatectomy for multiple and bilobar desmoplastic small round cell tumor liver metastases

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