Subcutaneous desmoplastic small round‐cell tumor: An unusual primary location expanding the differential of superficial round‐cell tumors

Asadbeigi, Sepideh; Zhang, Lisheng; Linos, Konstantinos

doi:10.1111/cup.13703

Cited by 9 publications

(13 citation statements)

References 40 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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“…Moreover, the tumor can have intermittent areas of primitive tubules or rosette-like structures [18]. Immunohistochemically, tumor cells show a pattern of multi-phenotypic differentiation [30,[39][40][41]. This multiple antigen expression pro le is a characteristic of DSRCT and can be used to distinguish DSRCT from the other histologically related small round cell tumors.…”

Section: Discussionmentioning

confidence: 99%

“…There is no standardized approach for the treatment of this malignant disease. Effective cytoreduction combined with comprehensive therapies, as the best treatment strategy presented in most studies, may prolong patient survival [27,[37][38][39][40][41]. With the in-depth analysis of molecular genetics of DSRCT, targeted therapy, immunotherapy, and other methods have been used for the treatment of DSRCT in recent years [28,29].…”

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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“…Imaging ndings are non-speci c, but they can indicate the location, size, and the number of tumors, thereby contributing to biopsy, surgery, and radiotherapy. [30,[39][40][41]. This multiple antigen expression pro le is a characteristic of DSRCT and can be used to distinguish DSRCT from the other histologically related small round cell tumors.…”

Section: Discussionmentioning

confidence: 99%

“…There is no standardized approach for the treatment of this malignant disease. Effective cytoreduction combined with comprehensive therapies, as the best treatment strategy presented in most studies, may prolong patient survival [27,[37][38][39][40][41]. With the in-depth analysis of molecular genetics of DSRCT, targeted therapy, immunotherapy and other methods have been used for the treatment of DSRCT in recent years [28,29].…”

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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Subcutaneous desmoplastic small round‐cell tumor: An unusual primary location expanding the differential of superficial round‐cell tumors

Cited by 9 publications

References 40 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 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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