A case of submandibular desmoplastic small round cell tumor: Diagnostic and management approaches to an atypical presentation of a rare tumor

Rubinstein, Jeremy D.; Gupta, Anita; Szabo, Sara; Pressey, Joseph G.

doi:10.1002/pbc.28178

Cited by 3 publications

(3 citation statements)

References 9 publications

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“…DSRCT, which was first described as a specific disease by Gerald and Rosai [ 16 , 17 ], is a rare and aggressive soft-tissue sarcoma. Generally, DSRCT develops on the serosal surface of the abdominal cavity [ 8 – 11 ], but it can also be found in the lung, eye and salivary gland [ 18 – 26 ]. DSRCT has no specific clinical symptoms.…”

Section: Discussionmentioning

confidence: 99%

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

Zhou

Luo

et al. 2022

Diagn Pathol

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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.

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Section: Discussionmentioning

confidence: 99%

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

Zhou

Luo

et al. 2022

Diagn Pathol

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“…Desmoplastic small round cell tumor (DSRCT), which was rst described as a speci c disease by Gerald and Rosai [8,9], is a rare and aggressive soft-tissue sarcoma. Generally, DSRCT originates from the serosal surface of the abdominal cavity [30][31][32][33], but it can also be found in the lung, sinus, bone, and mediastinum [3,[12][13][14][49][50][51][52][53]. Clinical symptoms are usually associated with the tumor sites and lack speci city.…”

Section: Discussionmentioning

confidence: 99%

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

Zhou

et al. 2021

Preprint

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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.

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“…Desmoplastic small round cell tumor (DSRCT), which was rst described as a speci c disease by Gerald and Rosai [8,9], is a rare and aggressive soft-tissue sarcoma. Generally, DSRCT originates from the serosal surface of the abdominal cavity [30][31][32][33], but it can also be found in the lung, eye and salivary gland [3,[43][44][45][49][50][51][52][53]. DSRCT Imaging examinations of DSRCT lack characteristic features.…”

Section: Discussionmentioning

confidence: 99%

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

Yuan²

2021

Preprint

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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.

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A case of submandibular desmoplastic small round cell tumor: Diagnostic and management approaches to an atypical presentation of a rare tumor

Cited by 3 publications

References 9 publications

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

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

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

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

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