scholarly journals Myeloablative Chemotherapy with Autologous Stem Cell Transplant for Desmoplastic Small Round Cell Tumor

Sarcoma ◽  
2015 ◽  
Vol 2015 ◽  
pp. 1-9 ◽  
Author(s):  
Christopher J. Forlenza ◽  
Brian H. Kushner ◽  
Nancy Kernan ◽  
Farid Boulad ◽  
Heather Magnan ◽  
...  
Keyword(s):  
Stem Cell ◽  
Stem Cell Transplant ◽  
Autologous Stem Cell Transplant ◽  
Cell Tumor ◽  
Cell Transplant ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Myeloablative Chemotherapy

Desmoplastic small round cell tumor (DSRCT), a rare, aggressive neoplasm, has a poor prognosis. In this prospective study, we evaluated the role of myeloablative chemotherapy, followed by autologous stem cell transplant in improving survival in DSRCT. After high-dose induction chemotherapy and surgery, 19 patients with chemoresponsive DSRCT underwent autologous stem cell transplant. Myeloablative chemotherapy consisted of carboplatin (400–700 mg/m2/day for 3 days) + thiotepa (300 mg/m2/day for 3 days) ± topotecan (2 mg/m2/day for 5 days). All patients were engrafted and there was no treatment-related mortality. Seventeen patients received radiotherapy to sites of prior or residual disease at a median of 12 weeks after transplant. Five-year event-free and overall survival were 11 ± 7% and 16 ± 8%, respectively. Two patients survive disease-free 16 and 19 years after transplant (both in complete remission before transplant). 14 patients had progression and died of disease at a median of 18 months following autologous transplant. These data do not justify the use of myeloablative chemotherapy with carboplatin plus thiotepa in patients with DSRCT. Alternative therapies should be considered for this aggressive neoplasm.

Desmoplastic small round-cell tumor: prolonged progression-free survival with aggressive multimodality therapy.

1996 ◽  
Vol 14 (5) ◽  
pp. 1526-1531 ◽  
Author(s):  
B H Kushner ◽  
M P LaQuaglia ◽  
N Wollner ◽  
P A Meyers ◽  
K L Lindsley ◽  
...  
Keyword(s):  
Stem Cell ◽  
Cell Tumor ◽  
Round Cell ◽  
Female Ratio ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Stem Cell Rescue ◽  
Previously Treated Patients ◽  
Previously Treated

PURPOSE To test intensive alkylator-based therapy in desmoplastic small round-cell tumor (DSRCT). PATIENTS AND METHODS Patients received the P6 protocol, which has seven courses of chemotherapy. Courses 1, 2, 3, and 6 included cyclophosphamide 4,200 mg/m2, doxorubicin 75 mg/m2, and vincristine (HD-CAV). Courses 4, 5, and 7 consisted of ifosfamide 9 g/m2 and etoposide 500 mg/m2 for previously untreated patients, or ifosfamide 12 g/m2 and etoposide 1,000 mg/m2 for previously treated patients. Courses started after neutrophil counts reached 500/microL and platelet counts reached 100,000/microL. Tumor resection was attempted. Post-P6 treatment options included radiotherapy and a myeloablative regimen of thiotepa (900 mg/m2) plus carboplatin (1,500 mg/m2), with stem-cell rescue. RESULTS Ten previously untreated and two previously treated patients have completed therapy. The male-to-female ratio was 11:1. Ages were 7 to 22 years (median, 14). The largest masses were infradiaphragmatic (n = 11) or intrathoracic (n = 1). Other findings included serosal implants (n = 11), regional lymph node invasion (n = 8), ascites or pleural effusion (n = 7), and metastases to liver (n = 5), lungs (n = 4), distant lymph nodes (n = 3), spleen (n = 2), and skeleton (n = 2). Tumors uniformly responded to HD-CAV, but there were no complete pathologic responses. One patient died at 1 month from tumor-related Budd-Chiari syndrome. Of seven patients who achieved a complete remission (CR), five remain in CR 9, 12, 13, 33, and 38 months from the start of P6, one patient died of infection at 12 months (autopsy-confirmed CR), and one patient relapsed 4 months off therapy. Of four patients who achieved a partial remission (PR), one remains progression-free at 34 months and three developed progressive disease. Five patients received local radiotherapy: three were not assessable for response, but in two patients, antitumor effect was evident. Four patients received thiotepa/carboplatin: two were in CR and remain so, and two patients had measurable disease that did not respond. CONCLUSION For control of DSRCT, our experience supports intensive use of HD-CAV, aggressive surgery to resect visible disease, radiotherapy to high-risk sites, and myeloablative chemotherapy with stem-cell rescue in selected cases.

Is There a Role for ASCT in Patients with Desmoplastic Small Round Cell Tumor of the Peritoneum?

Blood ◽  
2011 ◽  
Vol 118 (21) ◽  
pp. 3092-3092
Author(s):  
Rachel J Cook ◽  
Zhiwei Wang ◽  
Mukta Arora ◽  
Hillard M. Lazarus ◽  
Kimberly A. Kasow ◽  
...  
Keyword(s):  
Stem Cell ◽  
Median Survival ◽  
Stem Cell Transplant ◽  
Cell Tumor ◽  
Cell Transplant ◽  
Round Cell ◽  
Entire Cohort ◽  
Small Round Cell ◽  
Treatment Related Mortality ◽  
Related Mortality

Abstract Abstract 3092 Desmoplastic small round cell tumor of the peritoneum (DSRCTP) is a rare, frequently fatal solid tumor that occurs most commonly in adolescent males. Tumor characteristics were first described in 1989 and the largest review of 101 patients in 1996 demonstrated a median survival of 17 months (range 3–72) in patients with a median age of 21 years (range 6–38), none of whom underwent transplant. Survivorship has improved with the use of intensive alkylator regimens and incorporation of autologous stem cell transplant (ASCT). This study represents the largest reported cohort of patients with DSRCTP who have undergone ASCT. Increasingly aggressive chemotherapy regimens and ASCT have been used to try to prolong response and increase cure. The purpose of this study was to examine the outcomes in the largest reported cohort of patients with DSRCTP who have undergone ASCT. We identified 36 patients with DSRCTP reported to the Center for International Blood and Marrow Transplant Research between 1999 and 2007 who received an autologous hematopoietic stem cell transplant. The median age was 19 years (range 8–46) and 29 (81%) were male. Pre-transplant disease status was known in 31 patients, 13 of whom were in complete remission (CR). Twenty different conditioning regimens were identified for the 30 patients on which data was available with the most common agents being thiotepa (19 patients), etoposide (12 patients), mephalan (12 patients), cyclophosphamide (9 patients) and carboplatin (8 patients). The median follow up was 44 months. A subset of 18 patients had supplemental information obtained for confirmation of diagnosis (verification of the presence of translocation: t(11;22)(p13;q12)) and expanded clinical detail. We performed descriptive statistics to characterize the patients and outcomes. The probability of disease free survival (DFS) at one year for those patients in CR and not in CR was 75% (95% CI: 48–94%) and 35% (15–59%), respectively. For the entire cohort, 1 year DFS was 53% (39–69%). DFS at 3 years was 40% (15–69%) and 9% (0–29%) in the two groups, respectively. The probability of overall survival at three years was 57% (29–83%) for patients in CR and 28% (9–51%) for patients not in CR. Median survival for the entire cohort was 31 months (36 months for those in CR, 21 months for those not in CR). Treatment related mortality was low with only 1 death in the first 100 days. Engraftment at 42 days was 97% (88–100%). In summary, ASCT appears to be a tolerable approach in patients with DSCRTP with the greatest benefit seen in those patients who obtain CR. High intensity chemotherapy and ASCT leads to the longest DFS and OS in those patients who obtain a CR pre-transplant as might be expected. For those not in CR, the median OS in this series is greater (21 months) than previously reported (17 months) in patients not treated with ASCT suggesting a potential role for ASCT to prolong DFS and OS even in patients with residual or persistent disease pre-transplant. Overall, with low treatment related mortality and prolonged survival compared to historical controls, ASCT is a reasonable option for patients with DSRCTP though its role should be confirmed in larger studies. Disclosures: No relevant conflicts of interest to declare.

Optimizing the therapy of desmoplastic small round cell tumor: Combined experience from the two major cancer centers.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10021-10021
Author(s):  
Vivek Subbiah ◽  
Aaron D Viny ◽  
Peter Meade Anderson ◽  
Andrea Anita Hayes-Jordan ◽  
Wei Qiao ◽  
...  
Keyword(s):  
Overall Survival ◽  
Univariate Analysis ◽  
Cell Tumor ◽  
Cancer Center ◽  
Cell Transplant ◽  
Round Cell ◽  
Cancer Centers ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

10021 Background: Desmoplastic small round cell tumor (DSRCT) is a rare translocation-positive (EWS-WT1)sarcoma subtype that often presents as diffuse peritoneal sarcomatosis in male adolescents and young adults. The objective of this study was to assess the survival of patients with DSRCT from the two major cancer centers. Methods: A multi-center retrospective review was performed of patients diagnosed with DSRCT treated at The University of Texas MD Anderson Cancer Center (MDACC) and Memorial Sloan-Kettering Cancer Center (MSKCC). Results: Of the 197 pts evaluated at both cancer centers (109 from MSKCC and 88 from MDACC) 87 % were male. The mean age was 25 ± 11 years; 139 (70.6%) underwent surgery; 38 ( 19.6%) had debulking surgery, 30 received radiation therapy, and 27 underwent hyperthermic chemotherapy after debulking (CHPP), 11 had a stem cell transplant. 112 patients were seen after 2003 in both the centers vs 85 before 2003. Neoadjuvant chemotherapy included Ewing’s-like chemotherapy with MSKCC’s P6 regimen (<18 yrs of age) and VAI in adults. We further analyzed the MDACC cohort. In univariate analysis radiotherapy, surgery, CHPP, removal of primary mass + metastases, age < 30 and patients treated after 2003 were associated with significantly improved overall survival. Multi-variate analysis showed that patients treated after 2003 had 59% lesser hazard (HR=0.41) than those treated earlier (p=0.04). Comparing 1990-2003 vs. 2004-2010 survival >3 yr with DSRCT was <25 % and is now >50 %, a significant improvement. Conclusions: We present long-term outcomes for the largest series of patients diagnosed with DSRCT. A multidisciplinary approach to DSRCT treatment is required to prolong 3- and 5-yr overall survival. Our recent improved outcomes required not only aggressive chemotherapy but also regional local control measures. Best adjuvant therapy remains to be determined. A treatment algorithm will be proposed.

Desmoplastic small round cell tumor showing solid proliferation with limited desmoplasia and confusing immunohistochemical findings: an autopsy report

2020 ◽  
Vol 53 (3) ◽  
pp. 177-182
Author(s):  
Atsushi Kihara ◽  
Kazuya Takahashi ◽  
Ayataka Ishikawa ◽  
Yusuke Amano ◽  
Daisuke Matsubara ◽  
...  
Keyword(s):  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Autopsy Report

scholarly journals FDG PET/CT imaging of desmoplastic small round cell tumor: findings at staging, during treatment and at follow-up

2015 ◽  
Vol 45 (9) ◽  
pp. 1308-1315 ◽  
Author(s):  
Austin Ostermeier ◽  
M. Beth McCarville ◽  
Fariba Navid ◽  
Scott E. Snyder ◽  
Barry L. Shulkin
Keyword(s):  
Fdg Pet ◽  
Ct Imaging ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Pet Ct ◽  
Fdg Pet Ct

scholarly journals Desmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review

ORL ◽  
2021 ◽  
pp. 1-6
Author(s):  
Qingjiao Li ◽  
Xiaolu Yuan
Keyword(s):  
Submandibular Gland ◽  
Young Patients ◽  
Neck Region ◽  
Cell Tumor ◽  
Pelvic Cavity ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
The Right

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressively malignant tumor mostly occurring in the abdominal and pelvic cavity of young patients. However, few cases had been reported concerning DSRCT occurring in the head and neck region. We presented a rare case of DSRCT of the right submandibular in a 25-year-old man. MRI revealed a 3 × 2-cm solid nodule located in the right submandibular, and physical examination showed no other occupying lesion elsewhere. Histologically, the tumor was composed of various-sized small round cell nests, embedded in an abundant desmoplastic stroma. Immunohistochemically, the tumor cells were typically positive for epithelial (CK and EMA), mesenchymal (vimentin and desmin), and neuroendocrine (CD56, NSE, Syn, and CgA) markers, but negative for WT1. Fluorescence in situ hybridization revealed the presence of a break apart involving the <i>Ewing sarcoma</i> (<i>EWS</i>) gene. The patient received chemotherapy and radiotherapy and relapsed after 19 months of follow-up. DSRCT of the submandibular gland is rare, and the diagnosis of this tumor in an uncommon location relies on the histomorphology, immunophenotype, and <i>EWS</i> gene translocation detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.

scholarly journals The Role and Clinical Effectiveness of Multiline Chemotherapy in Advanced Desmoplastic Small Round Cell Tumor

2021 ◽  
Vol 15 ◽  
pp. 117955492098710
Author(s):  
Hyehyun Jeong ◽  
Yong Sang Hong ◽  
Young-Hoon Kim ◽  
Chan Wook Kim ◽  
Si Yeol Song ◽  
...  
Keyword(s):  
Medical Center ◽  
Complete Resection ◽  
Cell Tumor ◽  
Line Treatment ◽  
Round Cell ◽  
First Line ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
First Line Treatment

Background: A multimodal approach is the standard treatment for desmoplastic small round cell tumor (DSRCT); however, many patients are diagnosed with inoperable disease, which leaves chemotherapy as the only treatment option. There are limited data on the effectiveness of palliative chemotherapy, especially when used after first-line treatment. Here, we evaluated the clinical outcomes of patients with DSRCT treated with multiple lines of chemotherapy. Methods: We reviewed medical records of 14 patients with pathologically confirmed DSRCT at Asan Medical Center between 2004 and 2018. Results: The median age at diagnosis was 25, with males comprising 92.9% of patients. All patients had inoperable disease at presentation and received chemotherapy as the initial treatment. Four patients (28.6%) were treated with surgery, and complete resection was achieved in 1 patient. Median overall survival (OS) was 23.9 months, and 1-, 2-, and 3-year survival rates were 92.9%, 48.6%, and 19.5%, respectively. In patients receiving first- (N = 14), second- (N = 10), and third-line (N = 8) chemotherapy, median time-to-progression was 9.9, 3.5, and 2.5 months, respectively, and the disease control rates were 100%, 88.9%, and 75.0%, respectively. Factors associated with longer OS in the univariable analysis were ⩽2 metastatic sites at presentation (27.0 vs 14.7 months; P = .024) and surgery with intended complete resection (43.5 vs 20.1 months; P = .027). Conclusions: Although advanced DSRCT may initially respond to chemotherapy after first-line treatment, the response becomes less durable as the disease progresses. Individualized treatment decisions focused on palliation should be made.

scholarly journals Desmoplastic small round cell tumor: A case report of a rare differential diagnosis of solid tumors of the pleura

2015 ◽  
Vol 10 (5) ◽  
pp. 2991-2995 ◽  
Author(s):  
YUAN CAO ◽  
YING CHEN ◽  
LI YANG ◽  
ZI-HUA QIAN ◽  
SHU-GAO HAN ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Case Report ◽  
Solid Tumors ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Rare Differential Diagnosis
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