Metastatic Desmoplastic Small Round Cell Tumor Controlled by an Anthracycline-Based Regimen: Review of the Role of Chemotherapy

Mrabti, Hind; Kaikani, Wafaa; Ahbeddou, N.; Abahssain, Halima; Khannoussi, Basma El; Amrani, M.; Errihani, Hassan

doi:10.1007/s12029-011-9260-6

Cited by 12 publications

(10 citation statements)

References 40 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…When such aggressive strategies are not possible, several case reports describing modest activity with anthracyclin-based regimen, trabectedin, or temsirolimus are found in the literature [12–14]. …”

Section: Management Of Patients With Dsrctmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings

Dufresne

Cassier

Couraud

et al. 2012

Sarcoma

128

View full text Add to dashboard Cite

Desmoplastic small round cell tumor (DSRCT) is a rare and highly aggressive mesenchymal tumor that develops in the abdominal cavity of young men adults. Patients typically present with symptoms of abdominal sarcomatosis. Diagnosis is based on histological analysis of biopsies which typically show small round blue cells in nests separated by an abundant desmoplastic stroma. DSRCT is associated with a unique chromosomal translocation t(11:22) (p 13; q 12) that involves the EWSR1 and WT1 genes. The prognosis is particularly poor; median survival ranges from 17 to 25 months, largely due to the presentation of the majority of patients with metastatic disease. Management of DSRCT remains challenging and current schemes lack a significant cure rate despite the use of aggressive treatments such as polychemotherapy, debulking surgery and whole abdominal radiation. Several methods are being evaluated to improve survival: addition of chemotherapy and targeted therapies to standard neoadjuvant protocol, completion of surgical resection with HIPEC, postoperative IMRT, treatment of hepatic metastases with [90Y]Yttrium microsphere liver embolization.

show abstract

Section: Management Of Patients With Dsrctmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings

Dufresne

Cassier

Couraud

et al. 2012

Sarcoma

128

View full text Add to dashboard Cite

show abstract

“…However, more than half of these patients had no distant metastasis. Currently, there is no standard therapy for patients with DSRCT, particularly for inoperable/metastatic DSRCT cases, and there are few reports of metastatic DSRCT treatment (13). To the best of our knowledge, this is the first case of metastatic DSRCT in a patient who lived for more than three years without surgical resection (14).…”

Section: Discussionmentioning

confidence: 96%

A case of long-term survival of metastatic desmoplastic small round cell tumor treated with multimodal therapy

et al. 2011

View full text Add to dashboard Cite

Abstract. Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive and malignant tumor that predominantly affects young males. No standard therapy is currently available for patients with DSRCT and the prognosis remains extremely poor. In this study, we report a thought-provoking DSRCT case. A 24-year-old male was admitted to our hospital with a chief complaint of hematemesis. Computed tomography revealed a retrovesical mass with a splenic hilar tumor, multiple lung and liver tumors and marked lymph node swellings. The source of hematemesis was gastric varices caused by the compression of the splenic vein by a splenic hilar tumor. The patient was provided with a histological diagnosis of DSRCT based on needle biopsy from the liver tumors and the pelvic mass was thought to be the primary lesion. This is a long-term survival case of metastatic DSRCT treated with multimodal therapy including 15 courses of multiagent chemotherapy, radiation therapy for the hepatic portal region using 42.5 Gy, and four instances of therapeutic endoscopy. The prolonged progression-free survival period (15 months) obtained following chemotherapy suggests the chemosensitive feature of the disease. We used a modified P6 regimen (cyclophosphamide, pirarubicin, vincristine, ifosfamide and etoposide) and a modified PAVEP regimen (cyclophosphamide, pirarubicin, etoposide and cisplatin) to decrease severe adverse events and to improve the completion rate of chemotherapy. DSRCT is an aggressive but chemo-sensitive disease, and continuous chemotherapy using an appropriate regimen with possible supportive care is essential for long-term survival. This case report may represent a treatment option for this rare disease.

show abstract

“…In the majority of cases, DSRCT appears as a large mass in the abdominal cavity with serosal and omental spreading, and rapidly metastasizes to the liver, lungs, lymph nodes and the peritoneum (7,8). The most common symptoms of DSRCT are non-specific.…”

Section: Discussionmentioning

confidence: 99%

Removal of an intra-abdominal desmoplastic small round cell tumor by repetitive debulking surgery: A case report and literature review

et al. 2014

View full text Add to dashboard Cite

In the current study, a case of recurrent desmoplastic small round cell tumor (DSRCT) is presented, which was successfully treated by repetitive debulking surgery. In May 2010, a 39-year-old male, with a history of surgical resection of intra-abdominal DSRCT, visited the Ibaraki Medical Center, Tokyo Medical University Hospital (Ami, Japan) with severe lower abdominal discomfort. Abdominal computed tomography revealed a large tumor in the pouch of Douglas with a small number of nodules in the abdominal cavity. The recurrent DSRCT was diagnosed and removed via lower anterior resection; however, complete resection was impossible due to multiple peritoneal metastases. One year later, the patient developed pain in the right groin due to the growth of metastasized tumor cells in the groin lymph nodes. The affected lymph nodes were removed utilizing an extra-peritoneal approach. At the time of writing, the patient continues to survive without any symptoms 60 months since the initial surgery. In conclusion, surgical debulking is a significant procedure for relieving patient symptoms as well as improving the survival time of patients with metastatic and recurrent DSRCT.

show abstract

Metastatic Desmoplastic Small Round Cell Tumor Controlled by an Anthracycline-Based Regimen: Review of the Role of Chemotherapy

Cited by 12 publications

References 40 publications

Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings

Desmoplastic Small Round Cell Tumor: Current Management and Recent Findings

A case of long-term survival of metastatic desmoplastic small round cell tumor treated with multimodal therapy

Removal of an intra-abdominal desmoplastic small round cell tumor by repetitive debulking surgery: A case report and literature review

Contact Info

Product

Resources

About