Important Recently Characterized Non-Ewing Small Round Cell Tumors

Carter, Cody S.; Patel, Rajiv M.

doi:10.1016/j.path.2018.10.008

Cited by 21 publications

(40 citation statements)

References 61 publications

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“…In addition, several reports showed that CIC-rearranged sarcoma tended to have a greater amount of eosinophilic Fig. 4 a-c Computed tomography scan shows a greater than 16 cm recurrent tumor with distant metastases in the posterior segment of the liver and the lingula of the lung cytoplasm and occasional clear cell change compared with conventional Ewing Sarcoma, and lack any evidence of neuroectodermal differentiation such as Homer Wright rosettes [8]. In rare cases, appearance of a minor component of rhabdoid or plasmacytoid morphology has been reported [5,7,10].…”

Section: Discussionmentioning

confidence: 99%

“…Nearly 90% of cases arise in soft tissues, occur equally in the extremities and trunk/pelvis, and arise in visceral organs is approximately 10%. Though Ewing sarcoma arises commonly in bone, most CIC-rearranged sarcoma arises in soft tissue and bone development is less than 5% [8]. Very rare cases have been reported in deep organs such as the gastrointestinal tract, kidney, and brain [4,6,7,9].…”

Section: Discussionmentioning

confidence: 99%

“…CIC-rearranged sarcoma is also commonly positive for calretinin (approximately 70%), resulting in immunohistochemical overlap with mesothelioma [5]. In limited cases, focal desmin and cytokeratin AE1/AE3 and S100 expressed [4,5,7,8]. Several neuroendocrine markers and some cell surface markers used for diagnosing lymphoma are generally negative [4,5].…”

Section: Discussionmentioning

confidence: 99%

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Pancreaticoduodenectomy for a primary duodenal capicua transcriptional repressor (CIC) -rearranged sarcoma with severe bleeding: a case report

et al. 2020

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Background: Capicua transcriptional repressor (CIC) -rearranged sarcoma is characterized by small round cells, histologically similar to Ewing sarcoma. However, CIC-rearranged sarcoma has different clinical, histological, and immunohistochemical features from Ewing sarcoma. It is important to differentiate between these tumors. Case presentation: The patient is a 44-year-old man with a duodenal tumor diagnosed in another hospital who presented with a history of melena. Laboratory studies showed anemia with a serum hemoglobin of 6.0 g/dL. He was hospitalized and gastrointestinal bleeding was controlled successfully with endoscopy. However, he suffered from appetite loss and vomiting and progression of anemia a few weeks after presentation. Upper gastrointestinal endoscopy showed a circumferential soft tumor in the second portion of the duodenum and the endoscope could not pass distally. Computed tomography scan showed a greater than 10 cm tumor in the duodenum, with compression of the inferior vena cava and infiltrating the ascending colon. A definitive pathologic diagnosis could not be established despite four biopsies from the tumor edge. Due to gastrointestinal obstruction and progression of anemia, a pylorus-preserving pancreaticoduodenectomy with partial resection of the inferior vena cava and right hemicolectomy was performed as a complete tumor resection. The tumor was diagnosed as a CIC-rearranged sarcoma, but 2 months postoperatively local recurrence and distant metastases to the liver and lung were found. The patient died 3 months after surgery. Conclusions: Although the only definitive treatment for CIC-rearranged sarcoma is surgical resection, the CICrearranged sarcoma is highly malignant with a poor prognosis even after radical resection. More research is needed to establish optimal treatment strategies.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Pancreaticoduodenectomy for a primary duodenal capicua transcriptional repressor (CIC) -rearranged sarcoma with severe bleeding: a case report

et al. 2020

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“…They can present over a wide age range, but tend to occur in younger individuals (mean age of 30 years). Approximately 90% of such tumors are present in the deep soft tissues of the extremities, trunk, and/or pelvis, with only a minority of cases occurring in the bone . This pattern of anatomic localization shows a marked contrast with conventional EWS, more than 90% of which present in the bone.…”

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confidence: 98%

“…Cellular components that include rhabdoid, spindled, or plasmacytoid morphology can be encountered in such tumors. The degree of cytologic atypia in CIC ‐rearranged sarcomas is also higher than in conventional EWS, particularly the presence of a prominent nucleoli . Other features that allow one to consider the possibility of CIC ‐tumors include more abundant eosinophilic cytoplasm and the presence of clear‐cell changes.…”

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confidence: 99%