Desmoplastic Small Round Cell Tumor of the Ovary: A Case Report with a New Modality of Treatment and Review of the Literature

Vujić, Goran; Mikuš, Mislav; Matak, Luka; Bonevski, Aleksandra; Babić, Ivan; Planinić, Pavao; Babić, Damir; Ćorušić, Ante

doi:10.1055/s-0040-1710350

Cited by 15 publications

(18 citation statements)

References 28 publications

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“… Previous case reports reported primary sites of DSRCT, including kidney ( 2 ), bladder ( 3 ), pancreas ( 4 ), transverse colon ( 5 ), testes ( 6 ), ovary ( 7 ), mediastinum ( 8 ), pleura ( 9 ), lung ( 10 ), parotid gland ( 11 ), salivary glands ( 12 ), middle ear ( 13 ), sinonasal ( 14 ), orbital ( 15 ), intracranial cavity ( 16 ), subcutaneous site ( 17 ), femur ( 18 ), and bone of the hand ( 19 ). …”

Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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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.

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Section: Introductionmentioning

confidence: 99%

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

et al. 2021

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“…But characteristic translocation of EWS involves EWSR1 and the ETS family of transcription factors, not WT1. Convincingly, documentation of EWSR1-WT1 fusion is the "gold standard" for the diagnosis of DSRCT [35,39,40]. It was not performed in our case due to limited conditions.…”

Section: Discussionmentioning

confidence: 94%

“…has no speci c clinical symptoms. Most patients have the initial symptoms of abdominal mass, constipation, ascites, and vomiting[18,32,33,[35][36][37][38]. It can be accompanied by the manifestations of cachexia, such as fatigue and emaciation.…”

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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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Section: Discussionmentioning

confidence: 98%

“…Clinical symptoms are usually associated with the tumor sites and lack speci city. Most patients have the initial symptoms of abdominal mass, constipation, ascites, and vomiting [18,32,33,[35][36][37][38]. It can be accompanied by the manifestations of cachexia, such as fatigue and emaciation.…”

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 of the Ovary: A Case Report with a New Modality of Treatment and Review of the Literature

Cited by 15 publications

References 28 publications

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

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

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

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