Desmoplastic Small Round Cell Tumor Achieved Complete Remission after High-Dose Chemotherapy with Aggressive Local Treatment Modalities

Purpose. Desmoplastic small round cell tumor (DSRCT) is a rare cancer that predominantly affects males averaging 21 years of age at the time of diagnosis. We describe four cases from our institution and place them within the context of a comprehensive review of the literature. Patients and Methods. Study population included any patient who received treatment at Children’s Hospital at Montefiore (CHAM) with histologic diagnosis of DSRCT. A search of the electronic databases PubMed, Cochrane Central Register of Controlled Trials, MEDLINE, and EMBASE for the terms “desmoplastic” AND “small” AND “round” AND “cell” AND “tumor” was performed. Results. One CHAM patient died of disease at 39 months, one patient has relapsed disease at 40 months, and two patients have no evidence of disease at 60 and 91 months. In the literature review, the 3-year OS was 36% and 5-year OS was 13%. There was a statistically significant difference in OS between no transplant and SCT in remission (p=0.004); however, there was no difference between no transplant and SCT not in remission (p=0.23). Conclusion. Given the poor prognosis in DSRCT, this study supports further prospective research into the possible benefit of consolidation of autologous SCT in patients with DSRCT who are in remission, with the alternative inference that these patients in remission may fare well without SCT. Our retrospective review of the literature does not support SCT for patients who are not in remission.

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Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

Frontiers in Oncology ◽

10.3389/fonc.2021.705760 ◽

2021 ◽

Vol 11 ◽

Author(s):

Guixia Wei ◽

Xinyao Shu ◽

Yuwen Zhou ◽

Xia Liu ◽

Xiaorong Chen ◽

...

Keyword(s):

Clinical Manifestations ◽

Cell Tumor ◽

High Dose ◽

Rapid Progression ◽

Round Cell ◽

Differentiation Markers ◽

Soft Tissue Neoplasm ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Small Round Cell

Intra-abdominal desmoplastic small round cell tumor (IDSRCT) is a rare and highly malignant soft tissue neoplasm, which is characterized by rapid progression and poor prognosis. The mechanism underlying the development of this neoplasm remains elusive, but all cases are characterized by the chromosomal translocation t (11;22) (p13; q12), which results in a formation of EWSR1-WT1 gene fusion. The diagnosis of IDSRCT is often made with core-needle tissue biopsy specimens or laparoscopy or laparotomy. Immunohistochemical analyses have shown the co-expression of epithelial, neuronal, myogenic, and mesenchymal differentiation markers. FISH or reverse transcription polymerase chain reaction detecting EWS-WT1 fusion can be performed to assist in molecular confirmation. There is no standard of care for patients with IDSRCT currently, and majority of newly diagnosed patients received the aggressive therapy, which includes >90% resection of surgical debulking, high-dose alkylator-based chemotherapy, and radiotherapy. More recently, targeted therapy has been increasingly administered to recurrent IDSRCT patients and has been associated with improved survival in clinical conditions. Immunotherapy as a possible therapeutic strategy is being explored in patients with IDSRCT. In this review, we summarize currently available knowledge regarding the epidemiology, potential mechanisms, clinical manifestations, diagnosis, treatment, and prognosis of IDSRCT to assist oncologists in comprehensively recognizing and accurately treating this malignancy.

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Treatment of Desmoplastic Small round Cell Tumor with Image-Guided Intensity Modulated Radiation Therapy as a Component of Multimodality Treatment

Tumori Journal ◽

10.1177/030089160509100308 ◽

2005 ◽

Vol 91 (3) ◽

pp. 253-255 ◽

Cited By ~ 3

Author(s):

Christopher D Jahraus ◽

Shawn D Glisson ◽

William H St Clair

Keyword(s):

Intensity Modulated Radiotherapy ◽

Cell Tumor ◽

Treatment Modalities ◽

Round Cell ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Ultrasound Localization ◽

Small Round Cell ◽

Image Guided ◽

Intensity Modulated

Aims and background The authors report the case of a 31-year-old black male diagnosed with a pelvic desmoplastic small round-cell tumor who was treated with a unique radiotherapy approach incorporating intensity-modulated radiotherapy and daily ultrasound localization to ensure accurate tumor targeting. Desmoplastic small round-cell tumor is a very rare tumor, most commonly presenting in the abdominopelvic regions of adolescents and young adults. It has generally been associated with a very poor prognosis. Methods The patient initially underwent biopsy of the mass and omentectomy for mesenteric implants followed by chemotherapy. Chemotherapy resulted in tumor shrinkage and was followed by a second-look laparotomy. Additional omental nodules were resected, but the primary tumor was adherent to the rectum and seminal vesicles. A radiation oncology consult was obtained, and a course of image-guided intensity-modulated radiotherapy ensued with daily ultrasound localization. This resulted in shrinkage of local disease, but a new lesion was identified. The patient was subsequently started on further chemotherapy, which has maintained the disease in a stable state for several months. Conclusions Image-guided intensity-modulated radiotherapy is a feasible option in the treatment of pelvic desmoplastic small round-cell tumor. Such therapy may permit escalation of conventional radiotherapy doses and could have a favorable impact on local control of disease. Confirmation of this belief will require additional data in the form of case reports like this. Pending such confirmation, we continue to be of the impression that desmoplastic small round-cell tumor has an overall unfavorable prognosis, regardless of treatment modalities employed.

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Intraabdominal and ganglionic desmoplastic small round cell tumor: a case series

Journal of Medical Case Reports ◽

10.1186/s13256-021-03094-9 ◽

2021 ◽

Vol 15 (1) ◽

Author(s):

S. Slim ◽

I. Zemni ◽

A. Bouida ◽

M. Bouhani ◽

N. Boujelbene ◽

...

Keyword(s):

Abdominal Mass ◽

Case Series ◽

Cell Tumor ◽

High Dose ◽

Round Cell ◽

North African ◽

Round Cell Tumor ◽

Small Round Cell Tumor ◽

Presenting Symptoms ◽

Small Round Cell

Abstract Introduction Desmoplastic small round cell tumor is a rare malignancy with poor prognosis, affecting young male patients. It frequently presents as a large abdominal mass with widespread peritoneal involvement at diagnosis. In late stages, metastases may be present. Aim We retrospectively reviewed patient characteristics, presenting symptoms, tumor pathology, treatment, and outcome of four patients with desmoplastic small round cell tumor at our institution. Cases presentation The first three cases reported are 32-, 17-, and 30-year-old North African males with intraabdominal desmoplastic small round cell tumor treated by surgery, chemotherapy, and radiation therapy with different follow-ups. The final case is a 16-year-old North African male with ganglionic desmoplastic small round cell tumor but no evidence of a tissue mass. He underwent two lines of chemotherapy with no response. The patient was lost after 2 years of follow-up. In all cases, desmoplastic small round cell tumor was confirmed by presence of t(11,22) (p13,q12) translocation. Conclusion Treatment of desmoplastic small round cell tumor is based on multidisciplinary therapy. Despite high-dose chemotherapy, extensive surgical resection, and radiotherapy, desmoplastic small round cell tumor remains lethal.

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

Medical Molecular Morphology ◽

10.1007/s00795-019-00242-5 ◽

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

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

Pediatric Radiology ◽

10.1007/s00247-015-3315-y ◽

2015 ◽

Vol 45 (9) ◽

pp. 1308-1315 ◽

Cited By ~ 15

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

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

ORL ◽

10.1159/000517563 ◽

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 Ewing sarcoma (EWS) 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 EWS gene translocation detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.

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

Clinical Medicine Insights Oncology ◽

10.1177/1179554920987107 ◽

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.

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