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

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.

scholarly journals High-Dose Chemotherapy with Stem Cell Rescue in Desmoplastic Small Round Cell Tumor: A Single-Institution Experience and Review of the Literature

Sarcoma ◽  
2018 ◽  
Vol 2018 ◽  
pp. 1-10
Author(s):  
Kayleen Bailey ◽  
Michael Roth ◽  
Daniel Weiser ◽  
Jonathan Gill
Keyword(s):  
Cell Tumor ◽  
High Dose ◽  
Cochrane Central Register ◽  
Round Cell ◽  
Review Of The Literature ◽  
Histologic Diagnosis ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Significant Difference

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.

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

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.

scholarly journals Blue Round Small Cell tumor: A Surgical Update of DSRCT with review of literature ‘A Grim Affair’

2021 ◽  
pp. 31
Author(s):  
Keyword(s):  
Clinical Manifestations ◽  
Small Cell ◽  
Abdominal Discomfort ◽  
Primary Site ◽  
Cell Tumor ◽  
Round Cell ◽  
Review Of Literature ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Desmoplastic small round cell tumor (DSRCT) is a tumor derived from the Greek desmos referring to knot and plasis to formation an uncommon soft tissue malignant tumor, mesenchymal in origin and aggressive with a prelidiction for males and advanced at presentation. It was first described as a distinct clinical entity by Gerald WL and Rosai J (7). There are fewer than 200 reported to date. Depending on the primary site of location the Clinical manifestations vary. As most arise from the abdomen and pelvis they remain asymptomatic till they attain a huge size. Other reported sites are the skull, thorax, and paratesticular region (10,13). We report the case of a 19 yr old male who had non specific abdominal discomfort with asthenia for a period of six months and was referred to us for evaluation of left supraclavicular nodes. The prognosis of Desmoplastic small round cell tumor (DSRCT) is poor with few surviving less than two years.

scholarly journals Desmoplastic small round cell tumors of the pleura: a review of the clinical literature

2017 ◽  
Vol 12 ◽  
Author(s):  
Alessandro Giuseppe Fois ◽  
Pietro Pirina ◽  
Antonella Arcadu ◽  
Francesca Becciu ◽  
Sandra Manca ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Cell Tumor ◽  
Chest Roentgenogram ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
First Case ◽  
Rare Malignancy ◽  
Round Cell Tumors

Desmoplastic small round cell tumor of the pleura is a rare malignancy, with only a few cases reported in the scientific literature. The aim of the present review is to discuss the demographic, pathological, clinical, and therapeutic features of this rare tumor. English-language articles published since 1989, when the first case of desmoplastic small round cell tumor of the pleura was described, were retrieved, and fifteen cases included in fourteen articles were revised. The mean age of the patients was 25.5 years, out of them 60% were males. Chest pain, pleural effusion, and dyspnea were the most common clinical manifestations, while chest roentgenogram and computed tomography were the imaging techniques most commonly used. Surgical biopsy was employed in 80% of the cases for diagnosis. A multidisciplinary approach consisting in a combination of surgery with chemotherapy and radiation therapy was adopted in most cases. Only two patients (13.3%) were alive at 3 years from diagnosis, reflecting the aggressiveness of the disease, and the poor outcomes of the treatments currently available. Desmoplastic small round cell tumors of the pleura are extremely aggressive and challenging to diagnose, because of their rarity and unspecific demographic, clinical, and radiological features. An in-depth knowledge of such features is necessary for the optimal management of patients with this rare malignancy.

scholarly journals Multimodal treatment of an extremely rare desmoplastic small round cell tumor primary to the brachial plexus – A case report and review of literature

2019 ◽  
Vol 10 ◽  
pp. 140 ◽  
Author(s):  
José Fernando Guedes-Corrêa ◽  
Rogério Pires Amorim ◽  
Maristella Reis da Costa Pereira ◽  
Rodrigo Salvador Vivas Cardoso ◽  
Felipe D’Almeida Costa ◽  
...  
Keyword(s):  
Brachial Plexus ◽  
Malignant Neoplasm ◽  
Cell Tumor ◽  
Subtotal Resection ◽  
Rapid Progression ◽  
Adjuvant Radiation ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Background: Desmoplastic small round cell tumor (DSRCT) is a rare and aggressive malignant neoplasm typically located in the abdomen or pelvis. Other possible locations are the chest, pleura, scrotum, and central nervous system. DSRCT originally arising from the brachial plexus (BP) is extremely rare, to the best of our knowledge, only two cases have been previously described in the English scientific literature. Case Description: The authors present one new case of DSRCT arising from the left BP, the first in this location with rapid progression and in a female patient. We also highlight the importance of multimodal therapy, which included resection and both adjuvant radiation and chemotherapy. Macroscopic and microscopic characteristics of the lesion are detailed, as well as the patient’s status at 56-month follow-up. Conclusion: For primary BP DSRCT, aggressive subtotal resection followed by radiation and chemotherapy can be satisfactory for disease control and for maintaining or improving the neurological status.

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.

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