Desmoplastic Small Round Cell Tumor: A Rare Location in the Parotid Gland

Ninchritz-Becerra, Elisabeth; González-García, José Ángel; García-Iza, Leire; Estomba, Carlos Miguel Chiesa

doi:10.7759/cureus.10068

Cited by 4 publications

(3 citation statements)

References 6 publications

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“…Complete surgical resection can be exceedingly difficult, especially in the head and neck, due to its infiltrative nature and proximity to vital structures. The patients were followed up for a period ranging from one to 48 months; four patients died of the disease or its complications [10,[13][14][15], six patients had lymph nodes metastases [9][10][11][14][15][16], three patients lived with recurrence [8,16,17], and one lived with distant metastases [9]. Due to the rarity of this tumor in the head and neck and its unique immunohistochemical profile, four cases were misdiagnosed as Ewing sarcoma/peripheral primitive neuroectodermal tumor, olfactory neuroblastoma, and poorly differentiated carcinoma [8,9,18].…”

Section: Resultsmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor of the Head and Neck: A Potential Diagnostic Pitfall

Alharbi¹

2022

Cureus

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Primary desmoplastic small round cell tumor (DSRCT) in the head and neck region is extremely rare. There is limited information about its clinicopathological characteristics, prognosis, and treatment modalities. The purpose of this study is to provide a comprehensive review of DSRCT occurring primarily in the head and neck, to demonstrate its peculiar morphology and immunohistochemical expression, and to address the differential diagnoses. A total of 25 cases were collected after a thorough review of the relevant literature. DSRCT was most frequently reported in the major salivary glands, followed by the eyes. Furthermore, some cases were misinterpreted as poorly differentiated carcinoma, Ewing sarcoma, and olfactory neuroblastoma. Diagnosing DSRCTs in the head and neck can be very challenging due to their rarity in this location, overlapping morphology, and immunohistochemistry. In these cases, following a systemic approach helps to solve diagnostic problems.

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Section: Resultsmentioning

confidence: 99%

Desmoplastic Small Round Cell Tumor of the Head and Neck: A Potential Diagnostic Pitfall

Alharbi¹

2022

Cureus

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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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Desmoplastic Small Round Cell Tumor: A Rare Location in the Parotid Gland

Abstract: Ninchritz-Becerra et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0., which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

Cited by 4 publications

References 6 publications

Desmoplastic Small Round Cell Tumor of the Head and Neck: A Potential Diagnostic Pitfall

Desmoplastic Small Round Cell Tumor of the Head and Neck: A Potential Diagnostic Pitfall

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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