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

2021 ◽  
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

Abstract BackgroundDesmoplastic small round cell tumor (DSRCT) was a rare and aggressive malignant tumor mostly occurring in the abdominal and pelvic cavity of young patients. However, few cases had been reported concerning DSRCT located in the head and neck region. Case presentationA 25-year-old man was referred to our hospital due to the enlargement of the right submandibular mass. Magnetic resonance imaging revealed a 3 cm×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 marker (CD56, NSE, Syn and CgA), but negative for WT-1. Fluorescence in situ hybridization (FISH) revealed the presence of a break-apart involving Ewing sarcoma region 1 (EWSR1) gene. This tumor was finally diagnosed as DSRCT in the right submandibular gland. The patient received chemotherapy and radiotherapy, and showed recurrence after 17 months of follow-up.ConclusionsDSRCT of the submandibular gland is rare, the diagnosis of this tumor in uncommon location relies on the histomorphology, immunophenotype and EWSR1 gene detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.

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

2021 ◽  
Author(s):  
jiayu zhou ◽  
Qingling Li ◽  
baihua luo ◽  
xiaodan fu ◽  
chunlin ou ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Submandibular Gland ◽  
Tumor Therapy ◽  
Final Diagnosis ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
The Right

Abstract Background: Desmoplastic small round cell tumor (DSRCT) is a sporadic, highly malignant tumor with a poor prognosis. The abdomen and pelvis have been reported as the primary localization sites. However, to the best of our knowledge, there are few reports on primary DSRCT in the submandibular gland.Case presentation: We have reported a case of a 26-year-old Chinese man who presented with a mass in the right submandible. Imaging studies showed a hypoechoic mass in the right mandibular region. Intraoperative pathology revealed that the tumor tissue was composed of small round tumor cells and a dense desmoplastic stroma. On immunostaining, the tumor cells showed markers of epithelial, mesenchymal, myogenic, and neural differentiation. The EWSR1 gene rearrangement was detected by fluorescence in situ hybridization. Based on the overall morphological features and immunohistochemical findings, a final diagnosis of DSRCT was made. The patient was treated with comprehensive anti-tumor therapy mainly based on radiotherapy and chemotherapy.Conclusions: DSRCT is a very uncommon disease in which submandibular gland involvement is rare. Considering DSRCT in the differential diagnosis of small round blue cell tumors, even in extraperitoneal locations, is beneficial for a precise diagnosis. The purpose of this report was to describe a cases of DSRCT of submandibular gland and review the literature on the other published cases of DSRCT over the past five years. Full recognition of the clinicopathological features will help to better diagnose this disease.

scholarly journals Primary desmoplastic small round cell tumor of the submandibular gland: a case report and literature review

2022 ◽  
Vol 17 (1) ◽  
Author(s):  
Jiayu Zhou ◽  
Qingling Li ◽  
Baihua Luo ◽  
Xiaodan Fu ◽  
Chunlin Ou ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Submandibular Gland ◽  
Tumor Therapy ◽  
Cell Tumor ◽  
Round Cell ◽  
Submandibular Region ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
The Right

Abstract Background Desmoplastic small round cell tumor (DSRCT) is a sporadic, highly malignant tumor with a poor prognosis. The abdomen and pelvis have been reported as the primary localization sites. However, to the best of our knowledge, there are few reports on primary DSRCT in the submandibular gland. Case presentation We report a case of a 26-year-old Chinese man with a mass in the right submandibular gland. Imaging studies showed a hypoechoic mass in the right submandibular region. Intraoperative pathology revealed that the tumor tissue was composed of small round tumor cells and a dense desmoplastic stroma. On immunostaining, the tumor cells showed markers of epithelial, mesenchymal, myogenic, and neural differentiation. The EWSR1 gene rearrangement was detected by fluorescence in situ hybridization. Based on the overall morphological features and immunohistochemical findings, a final diagnosis of DSRCT was made. The patient was treated with comprehensive anti-tumor therapy mainly based on radiotherapy and chemotherapy. Conclusions DSRCT is an uncommon malignant neoplasm with rare submandibular gland involvement. In this report, we have described a case of DSRCT in the submandibular gland and reviewed the literature on DSRCT over the past 5 years. Considering the importance of differential diagnosis between DSRCT, especially with rare extra-peritoneal involvement, and small round blue cell tumors, a full recognition of the clinicopathological features will help to better diagnose this neoplasm.

scholarly journals Desmoplastic Small Round Cell Tumor of the Head and Neck: A Clinicopathological, Immunohistochemical and Molecular Analysis of Three Cases with Literature Review

2021 ◽  
Author(s):  
Meng Sun ◽  
Xue-bing Jiang ◽  
Meng-yuan Shao ◽  
Qi-feng Wang ◽  
Lu Zhao ◽  
...  
Keyword(s):  
Head And Neck ◽  
Rare Condition ◽  
Cell Tumor ◽  
Pelvic Cavity ◽  
Fish Analysis ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Eosinophilic Cytoplasm

Abstract Background: Desmoplastic small round cell tumor (DSRCT) is a rare aggressive malignancy typically originating from the abdominal or pelvic cavity. DSRCT presenting as a primary head and neck tumor has rarely been described in the literature. Case presentation: We present three cases of DSRCT arising in the head and neck to further characterize its clinicopathological features. All three patients were male and aged 36, 30 and 17 years. The involved sites included the orbit (1 case) and submandibular gland (2 cases). The tumors ranged in size from 2.4 to 3.5 cm (mean, 2.1 cm). Histologically, all tumors showed irregular-shaped, variable-sized nests of small round cells deposited in an abundant desmoplastic stroma. Tumor cells contained small hyperchromatic nuclei and scant amounts of eosinophilic cytoplasm with inconspicuous nucleoli. Immunohistochemically, the tumors were positive for AE1/AE3 (3/3), desmin (3/3), vimentin (2/2), NSE (1/1) and EMA (1/1). Fluorescence in situ hybridization (FISH) analysis demonstrated the presence of EWSR1 and WT1 rearrangements in all three cases. All patients received surgery and adjuvant chemotherapy and/or radiotherapy. There was no evidence of recurrence and metastasis in two patients, and the third suffered lung metastasis.Conclusions: DSRCT arising in the head and neck represents an extremely rare condition. It is easily mistaken as poorly differentiated carcinoma due to similar morphology and expression of epithelial markers. Immunohistochemistry assay in conjunction with molecular detection of EWSR1-WT1 fusion will be helpful for arriving at an accurate diagnosis to avoid misdiagnosis and inappropriate treatment.

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

2021 ◽  
Author(s):  
jiayu zhou ◽  
Qingling Li ◽  
Baihua Luo ◽  
Xiaodan Fu ◽  
Chunlin Ou ◽  
...  
Keyword(s):  
Tumor Cells ◽  
Tumor Therapy ◽  
Final Diagnosis ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Precise Diagnosis ◽  
The Right

Abstract Background: Desmoplastic small round cell tumor (DSRCT) is a sporadic, highly malignant tumor with a poor prognosis. The abdomen and pelvis have been reported as the primary localization sites. However, to the best of our knowledge, there are few reports on primary DSRCT in the mandible.Case presentation: We have reported a case of a 26-year-old Chinese man who presented with a mass in the right mandible. Imaging studies showed a hypoechoic mass in the right mandibular region. Intraoperative pathology revealed that the tumor tissue was composed of small round tumor cells and a dense desmoplastic stroma. On immunostaining, the tumor cells showed markers of epithelial, mesenchymal, myogenic, and neural differentiation. The EWSR1 gene rearrangement was detected by fluorescence in situ hybridization. Based on the overall morphological features and immunohistochemical findings, a final diagnosis of DSRCT was made. The patient was treated with comprehensive anti-tumor therapy mainly based on radiotherapy and chemotherapy.Conclusions: DSRCT is a very uncommon disease in which mandibular involvement is rare. Considering DSRCT in the differential diagnosis of small round blue cell tumors, even in extraperitoneal locations, is beneficial for a precise diagnosis. Full recognition of the clinicopathological features will help to better diagnose this disease.

Multiagent chemotherapy including IrIVA regimen and maintenance therapy in the treatment of desmoplastic small round cell tumor

2021 ◽  
pp. 030089162199525
Author(s):  
Andrea Ferrari ◽  
Luca Bergamaschi ◽  
Stefano Chiaravalli ◽  
Emilia Pecori ◽  
Barbara Diletto ◽  
...  
Keyword(s):  
Maintenance Therapy ◽  
Short Interval ◽  
Young Patients ◽  
Cell Tumor ◽  
Round Cell ◽  
Antiangiogenic Effect ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Small Series

This study reports the treatment feasibility and efficacy of a novel multiagent intensive treatment program for young patients with desmoplastic small round cell tumor. This small series includes three patients and should be seen as a first suggestion of integration of the dose density and the maintenance chemotherapy concept. The IrIVA regimen (irinotecan, ifosfamide, vincristine, and actinomycin-D) is added—used at a short interval between chemotherapy administrations—at more classic intensive ifosfamide–based regimens. The vinorelbine and low-dose oral cyclophosphamide maintenance therapy is added at the end of conventional chemotherapy to achieve an antiangiogenic effect.

Bone Marrow Metastasis of Desmoplastic Small round Cell Tumor

2007 ◽  
Vol 93 (5) ◽  
pp. 511-513 ◽  
Author(s):  
Jing Deng ◽  
Nong Xu ◽  
Peng Shen ◽  
Zhaoming Wang ◽  
Xiaochen Zhang ◽  
...  
Keyword(s):  
Bone Marrow ◽  
Lung Metastases ◽  
Abdominal Cavity ◽  
Cell Tumor ◽  
Pelvic Cavity ◽  
Round Cell ◽  
Bone Marrow Metastasis ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Desmoplastic small round cell tumor is an extremely rare and highly aggressive neoplasm. It usually arises as a single mass or multiple masses in the abdominal cavity, characterized by diffuse peritoneal implants, involvement of regional lymph nodes, and liver and lung metastases. However, bone marrow metastasis has rarely been reported in the literature. We present a case of clinically symptomatic bone marrow metastasis in a 25-year-old woman with a diagnosis of desmoplastic small round cell tumor originating from the pelvic cavity.

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
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