A Primary Cardiac Sarcoma With Features of a Desmoplastic Small Round Cell Tumor in an Adolescent Male

2010 ◽  
Vol 32 (3) ◽  
pp. 236-239 ◽  
Author(s):  
Eleny C. Romanos-Sirakis ◽  
David B. Meyer ◽  
Anne Chun ◽  
Sharon Gardner
Keyword(s):  
Cell Tumor ◽  
Adolescent Male ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Primary Cardiac Sarcoma

scholarly journals A rare cause of bilateral pleural effusion – desmoplastic small round cell tumor

2021 ◽  
Author(s):  
Archana Baburao ◽  
Poonam Maurya ◽  
Amulya Chakenahalli ◽  
Huliraj Narayanswamy
Keyword(s):  
Pleural Effusion ◽  
Abdominal Mass ◽  
Cell Tumor ◽  
Young Adolescent ◽  
Bilateral Pleural Effusion ◽  
Adolescent Male ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Desmoplastic small round cell tumor (DSRCT) is a rare, extremely aggressive and malignant tumor predominantly affects young adolescent males and typically presents as a large intra-abdominal mass. However, tumor arising from other body sites are also reported in the literature. Histology and immunohistochemistry play an important role in the diagnosis and differentiating this rare tumor from other round cell tumors. A multidisciplinary approach consisting of a combination of surgery, chemotherapy and radiation therapy is the treatment of choice as there is no standard therapy.  We report a case of DSRCT of pleura presenting as bilateral pleural effusion in a young adolescent male who was treated with both surgery and chemotherapy. However patient succumbed to death after one year of diagnosis.

scholarly journals Extensive Abdominopelvic Desmoplastic Small Round Cell Tumor — Case Report and Review of the Literature

2016 ◽  
Vol 1 (2) ◽  
pp. 189-192
Author(s):  
Adrienne Horváth ◽  
Zsuzsanna Erzsébet Papp
Keyword(s):  
Clinical Status ◽  
Correct Diagnosis ◽  
Cell Tumor ◽  
Adolescent Male ◽  
Pelvic Cavity ◽  
Round Cell ◽  
Young Males ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

AbstractDesmoplastic small round cell tumor (DSRCT) is a rare malignant tumor, which affects mostly young males and has a poor prognosis. Since 1991, when it was first described as a distinct clinical entity by Gerald WL and Rosai J, some 200 cases were reported. DSRCT arises mainly from the abdominal and pelvic cavity, causes abdominal pain or discomfort, weight loss, urinary, bowel or bile obstruction due to compression. Metastases appear most frequently in the liver and lungs. Multimodal therapy is usually indicated with chemotherapy, surgery, radiotherapy and autologous stem cell transplantation. DSRCT should be differentiated from other small, blue round cell tumors, especially hematopoietic malignancies (leukemia, lymphoma), neuroblastoma, Ewing sarcoma, PNET, rhabdomyosarcoma, malignant mesothelioma, small cell carcinoma and Wilms tumors. We report the case of a patient with an extensive abdominopelvic desmoplastic small round cell tumor, with liver metastases, in an adolescent male patient, highlighting the alert deterioration of the clinical status of the patient after the biopsy, the need for a second review of the histopathological material in order to obtain a correct diagnosis, the chemoresistance of the tumor despite an apparently good clinical status, and the severe prognosis of this type of tumor.

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

Desmoplastic small round cell tumor 20 years after its discovery

2015 ◽  
Vol 11 (7) ◽  
pp. 1071-1081 ◽  
Author(s):  
Jaume Mora ◽  
Shakeel Modak ◽  
Nai-Kong Cheung ◽  
Paul Meyers ◽  
Enrique de Alava ◽  
...  
Keyword(s):  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell
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