Omental desmoplastic small round cell tumor with metastasis
We report a case of a huge omental DSRCT with lymph node metastasis which was initially misdiagnosed as gastrointestinal stromal tumor on radiology. A 26-year-old male presented with complaints of upper abdominal swelling associated with constant dull pain. On examination there was a large 15 × 12 cm intraabdominal mass in the epigastric and umbilical region. Imaging studies were suggestive of neoplastic mesenchymal etiology. Image-guided fine-needle aspiration cytology (FNAC) was suggestive of mesenchymal neoplastic etiology. On laparotomy, there was a huge 20 × 15 cm mass arising from omentum with multiple om...
Source: Indian Journal of Pathology and Microbiology - January 25, 2022 Category: Pathology Authors: Raghav Yelamanchi Ekta Yadav Nikhil Gupta Arvind Ahuja D S Chauhan Source Type: research

Omental desmoplastic small round cell tumor with metastasis
We report a case of a huge omental DSRCT with lymph node metastasis which was initially misdiagnosed as gastrointestinal stromal tumor on radiology. A 26-year-old male presented with complaints of upper abdominal swelling associated with constant dull pain. On examination there was a large 15 × 12 cm intraabdominal mass in the epigastric and umbilical region. Imaging studies were suggestive of neoplastic mesenchymal etiology. Image-guided fine-needle aspiration cytology (FNAC) was suggestive of mesenchymal neoplastic etiology. On laparotomy, there was a huge 20 × 15 cm mass arising from omentum with multiple om...
Source: Indian Journal of Pathology and Microbiology - January 25, 2022 Category: Pathology Authors: Raghav Yelamanchi Ekta Yadav Nikhil Gupta Arvind Ahuja D S Chauhan Source Type: research

Lobular Carcinoma of the Breast Metastatic to the Ovary: A Clinicopathologic Study of 38 Cases
We evaluated the clinicopathologic features of 38 cases of metastatic lobular (n=33) or predominantly lobular (n=5) carcinoma involving the ovary. The patients were from 39 to 91 years of age (mean: 53 y). In 2 cases, the breast primary and ovarian metastasis were diagnosed synchronously, and in 5, the breast primary was only discovered after the metastatic carcinoma in the ovary was found. In the majority of cases (79%), both ovaries were involved; the mean ovarian tumor size was 5.9 cm. The ovarian tumors demonstrated a range of architectural patterns including macronodular (71%), diffuse/solid growth (87%)...
Source: The American Journal of Surgical Pathology - January 18, 2022 Category: Pathology Tags: Original Articles Source Type: research

Phase 2 Study of ONC201 in Neuroendocrine Tumors including Pheochromocytoma-Paraganglioma and Desmoplastic Small Round Cell Tumor
CONCLUSIONS: Oral ONC201 was well tolerated in patients with metastatic neuroendocrine tumors and associated with clinical benefit including tumor responses, particularly in some DSRCT patients and the majority of PC-PG patients.PMID:35022321 | DOI:10.1158/1078-0432.CCR-21-4030 (Source: Clinical Cancer Research)
Source: Clinical Cancer Research - January 13, 2022 Category: Cancer & Oncology Authors: Peter M Anderson Matteo M Trucco Rohinton S Tarapore Stacey Zahler Stefanie Thomas Janette Gortz Omar Mian Martin Stoignew Varun Prabhu Sara Morrow Joshua E Allen Source Type: research

Phase 2 Study of ONC201 in Neuroendocrine Tumors including Pheochromocytoma-Paraganglioma and Desmoplastic Small Round Cell Tumor
CONCLUSIONS: Oral ONC201 was well tolerated in patients with metastatic neuroendocrine tumors and associated with clinical benefit including tumor responses, particularly in some DSRCT patients and the majority of PC-PG patients.PMID:35022321 | DOI:10.1158/1078-0432.CCR-21-4030 (Source: Clinical Cancer Research)
Source: Clinical Cancer Research - January 13, 2022 Category: Cancer & Oncology Authors: Peter M Anderson Matteo M Trucco Rohinton S Tarapore Stacey Zahler Stefanie Thomas Janette Gortz Omar Mian Martin Stoignew Varun Prabhu Sara Morrow Joshua E Allen Source Type: research

Primary desmoplastic small round cell tumor of the submandibular gland: a case report and literature review
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... (Source: Diagnostic Pathology)
Source: Diagnostic Pathology - January 7, 2022 Category: Pathology Authors: Jiayu Zhou, Qingling Li, Baihua Luo, Xiaodan Fu, Chunlin Ou, Xiaomei Gao, Zhijie Xu, Deyun Feng and Keda Yang Tags: Case Report Source Type: research

Desmoplastic Small Round Cell Tumor in a Pregnant Woman: A Case Report and Literature Review
Conclusion: The treatment of DSRCT in pregnant women requires a multidisciplinary consultation, and the treatment and examination during pregnancy are subject to many constraints, which may have a negative impact on the patient's prognosis. Also, a review of the literature found that there is still no standard treatment protocol for DSRCT, and its prognosis in female patients is independent of age and tissue origin.PMID:34970100 | PMC:PMC8686780 (Source: The Yale Journal of Biology and Medicine)
Source: The Yale Journal of Biology and Medicine - December 31, 2021 Category: Universities & Medical Training Authors: Haiying Zhou Ahmad Alhaskawi Qingrong Sun Yanzhao Dong Vishnu Goutham Kota Mohamed Hasan Abdulla Hasan Abdulla Sohaib Hasan Abdullah Ezzi Zewei Wang Hui Lu Source Type: research

Paraduodenal/pancreatic Ewing sarcoma is very rare and therefore may be mistaken for neuroendocrine carcinoma
A diagnosis of Ewing sarcoma (ES) is suggested by certain morphological and immunohistochemical findings and supported by demonstration of EWSR1 translocation,1 as is usually demonstrated generically with a break-apart fluorescence in situ hybridisation (FISH) probe. However, because there are differential diagnoses that also harbour EWSR1 translocations (eg, desmoplastic small round cell tumour, DSRCT), ES can only be confirmed by demonstrating a fusion partner regarded to be ES specific.1 The most common of these partners are FLI1 (85% of ES) and ERG (10% of ES).1 Most ESs arise in bone or soft tissues1 and here, patholo...
Source: Journal of Clinical Pathology - December 15, 2021 Category: Pathology Authors: Wong, N. A. C. S., Suortamo, S., George, E., Beavers, S. Tags: PostScript Source Type: research

Cancers, Vol. 13, Pages 6072: EWSR1-WT1 Target Genes and Therapeutic Options Identified in a Novel DSRCT In Vitro Model
This study underscores the usefulness of preclinical in vitro models for studying molecular mechanisms and potential therapeutic options. (Source: Cancers)
Source: Cancers - December 2, 2021 Category: Cancer & Oncology Authors: Margit Bleijs Corine Pleijte Sem Engels Femke Ringnalda Friederike Meyer-Wentrup Marc van de Wetering Hans Clevers Tags: Article Source Type: research

The contribution of Juan Rosai to the pathology of soft tissue tumors
Pathologica. 2021 Oct;113(5):396-409. doi: 10.32074/1591-951X-551.ABSTRACTThe conceptual evolution in the field of soft tissue tumor pathology has been mostly driven by a relatively small group of individuals that includes giants of the past and the present such as James Ewing, Raffaele Lattes, Arthur Purdy Stout, Franz Enzinger, Sharon Weiss, Lennart Angervall, Harry Evans, Marku Miettinen, and Christopher Fletcher. Juan Rosai, not only exerted an immense impact on surgical pathology in general, but in consideration of his unique talent in identifying novel clinicopathologic entities, has also contributed remarkably to cu...
Source: Pathologica - November 27, 2021 Category: Pathology Authors: Marta Sbaraglia Elena Bellan Thomas Mentzel Angelo P Dei Tos Source Type: research

A rare cause of bilateral pleural effusion - desmoplastic small round cell tumor
We report a case of DSRCT of pleura presenting as bilateral pleural effusion in a young adolescent male who was treated with both surgery and chemotherapy. However patient succumbed to death after one year of diagnosis.PMID:34802222 | DOI:10.4081/monaldi.2021.1874 (Source: Monaldi Archives for Chest Disease)
Source: Monaldi Archives for Chest Disease - November 22, 2021 Category: Respiratory Medicine Authors: Archana Baburao Poonam Maurya Amulya Chakenahalli Huliraj Narayanswamy Source Type: research

Intraabdominal and ganglionic desmoplastic small round cell tumor: a  case series
ConclusionTreatment of desmoplastic small round cell tumor is based on multidisciplinary therapy. Despite high-dose chemotherapy, extensive surgical resection, and radiotherapy, desmoplastic small round cell tumor remains lethal. (Source: Journal of Medical Case Reports)
Source: Journal of Medical Case Reports - October 12, 2021 Category: General Medicine Source Type: research

Presentation of a  rare, highly aggressive peritoneal disease: desmoplastic small round cell tumor and its therapeutic options
ConclusionOur case report demonstrates a  very rare cause of recurrent abdominal pain. Desmoplastic small round cell tumor is a rare and highly aggressive undifferentiated sarcoma, which mainly affects young men. Treatment options include chemotherapy, radiotherapy, cytoreductive surgery, and/or hyperthermic intraperitoneal chemotherapy. Standardized treatment protocols are still lacking because only a few cases have been described so far. Differential diagnoses include all malignancies with peritoneal masses. (Source: European Surgery)
Source: European Surgery - October 1, 2021 Category: Surgery Source Type: research

Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives
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 m...
Source: Frontiers in Oncology - September 15, 2021 Category: Cancer & Oncology Source Type: research

Surgical management and outcomes of paediatric ovarian tumours —a 25-year UK single centre experience
ConclusionsFindings from this study show an excellent survival (97%) for paediatric ovarian neoplasms. Rare malignant neoplasms though do carry a poor prognosis. In the modern era of care, all efforts should be made to preserve future hormonal health and fertility with ‘ovarian sparing’ operation(s) where appropriate. (Source: Pediatric Surgery International)
Source: Pediatric Surgery International - September 2, 2021 Category: Surgery Source Type: research

Investigation of spleen CXCR4 expression by [ < sup > 68 < /sup > Ga]Pentixafor PET in a cohort of 145 solid cancer patients
CONCLUSION: Spleen [68Ga]Pentixafor uptake was not associated with stage of disease and clinical outcomes in solid tumor patients. We identified positively associated platelet and/or leukocyte counts with spleen [68Ga]Pentixafor uptake in neuroendocrine tumors, non-small cell lung cancer and small cell lung cancer, suggesting that splenic CXCR4 expression could possibly play a role in systemic immunity/inflammation in some types of solid tumors or a subgroup of patients within solid tumor entities.PMID:34417915 | DOI:10.1186/s13550-021-00822-6 (Source: Cell Research)
Source: Cell Research - August 21, 2021 Category: Cytology Authors: Richard Lewis Stefan Habringer Malte Kircher Maike Hefter Caroline Anna Peuker Rudolf Werner Val ëza Ademaj-Kospiri Alexander G äble Wolfgang Weber Hans-J ürgen Wester Andreas Buck Peter Herhaus Constantin Lapa Ulrich Keller Source Type: research

Investigation of spleen CXCR4 expression by [68Ga]Pentixafor PET in a cohort of 145 solid cancer patients
ConclusionSpleen [68Ga]Pentixafor uptake was not associated with stage of disease and clinical outcomes in solid tumor patients. We identified positively associated platelet and/or leukocyte counts with spleen [68Ga]Pentixafor uptake in neuroendocrine tumors, non-small cell lung cancer and small cell lung cancer, suggesting that splenic CXCR4 expression could possibly play a role in systemic immunity/inflammation in some types of solid tumors or a subgroup of patients within solid tumor entities. (Source: EJNMMI Research)
Source: EJNMMI Research - August 21, 2021 Category: Radiology Source Type: research

Mesenchymal/non-epithelial mimickers of neuroendocrine neoplasms with a focus on fusion gene-associated and SWI/SNF-deficient tumors
AbstractMimickers of neuroendocrine neoplasms (NEN) include a number of important pitfall tumors. Here, we describe our experience with mesenchymal mimics of NENs to illustrate their spectrum and draw the attention particularly to a group of mesenchymal/non-epithelial neoplasms (MN) that combine epithelioid histology with neuroendocrine (NE-) features and peculiar genetic abnormalities. In a consultation series of 4498 cases collected between 2009 and 2021, 2099 neoplasms expressing synaptophysin and/or chromograninA were reviewed and analyzed. A total of 364 (18%) were diagnosed as non-NENs, while the remaining tumors wer...
Source: Virchows Archiv - August 5, 2021 Category: Pathology Source Type: research

The clear cell sarcoma functional genomic landscape
Clear cell sarcoma (CCS) is a deadly malignancy affecting adolescents and young adults. It is characterized by reciprocal translocations resulting in expression of the chimeric EWSR1-ATF1 or EWSR1-CREB1 fusion proteins, driving sarcomagenesis. Besides these characteristics, CCS has remained genomically uncharacterized. Copy number analysis of human CCSs showed frequent amplifications of the MITF locus and chromosomes 7 and 8. Few alterations were shared with Ewing sarcoma or desmoplastic, small round cell tumors, which are other EWSR1-rearranged tumors. Exome sequencing in mouse tumors generated by expression of EWSR1-ATF1...
Source: Journal of Clinical Investigation - August 3, 2021 Category: Biomedical Science Authors: Emanuele Panza, Benjamin B. Ozenberger, Krystal M. Straessler, Jared J. Barrott, Li Li, Yanliang Wang, Mingchao Xie, Anne Boulet, Simon W.A. Titen, Clinton C. Mason, Alexander J. Lazar, Li Ding, Mario R. Capecchi, Kevin B. Jones Source Type: research

Multimodal Therapy Including Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy Can Result in Long-term Disease-free Survival in Pediatric Desmoplastic Small Round Cell Tumor With Extraperitoneal Disease
Desmoplastic small round cell tumor is a rare sarcoma with 5-year overall survival of 15%. An 8-year-old female presented with diffuse abdominal/pelvic desmoplastic small round cell tumor including numerous liver metastasis. She underwent neoadjuvant chemotherapy followed by cytoreductive surgery (CRS) and hyperthermic intraperitoneal chemotherapy (HIPEC). Residual disease was found shortly after CRS/HIPEC which was resected, followed by whole abdomen/pelvic radiation and autologous hematopoietic cell transplant. Previous papers have reported dismal survival in patients with liver metastasis and residual disease arguing ag...
Source: Journal of Pediatric Hematology Oncology - August 1, 2021 Category: Hematology Tags: Clinical and Laboratory Observations Source Type: research

Desmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review
We presented a rare case of DSRCT of the right submandibular in a 25-year-old man. MRI revealed a 3 × 2-cm solid nodule located in the right submandibular, and physical examination showed no other occupying lesion elsewhere. Histologically, the tumor was composed of various-sized small round cell nests, embedded in an abundant desmoplastic stroma. Immunohistochemically, the tumor cells were typic ally positive for epithelial (CK and EMA), mesenchymal (vimentin and desmin), and neuroendocrine (CD56, NSE, Syn, and CgA) markers, but negative for WT1. Fluorescence in situ hybridization revealed the presence of a break ap...
Source: ORL - July 28, 2021 Category: ENT & OMF Source Type: research

Desmoplastic Small Round-cell Tumor: Retrospective Review of Institutional Data and Literature Review
CONCLUSION: While aggressive multimodality treatment is always warranted for DSCRT, the options are limited by the multicentric presentation, short-lived initial response and lack of established subsequent therapy portending a poor prognosis. Consolidative cytoreductive surgery following first-line therapy may improve survival.PMID:34281846 | DOI:10.21873/anticanres.15179 (Source: Cell Research)
Source: Cell Research - July 20, 2021 Category: Cytology Authors: Thejus Jayakrishnan Ryan Moll Ariel Sandhu Angela Sanguino Gurveen Kaur Shifeng Mao Source Type: research

Uncommon and peculiar soft tissue sarcomas: multidisciplinary review and practical recommendations. Spanish Group for Sarcoma research (GEIS –GROUP). Part II
In this second part of the “Uncommon and peculiar soft tissue sarcomas: multidisciplinary review and practical recommendations” from Spanish Group for Research on Sarcoma (GEIS –group), we will review, in a multidisciplinary way, another group of uncommon sarcoma subtypes, with the same objectives and rules we set up in the first part. We will analyze here the following subtypes: alveolar soft part sarcoma, epithelioid sarcoma, clear cell sarcoma, desmoplastic small round cell tumor, rhabdoid tumor, phyllodes tumor, tenosynovial giant cell tumors, myoepithelial tumor, perivascular epithelioid cell neoplas...
Source: Cancer Treatment Reviews - July 15, 2021 Category: Cancer & Oncology Authors: Javier Mart ínez-Trufero, Josefina Cruz Jurado, C. Nieves Hernández-León, Raquel Correa, Jose Manuel Asencio, Daniel Bernabeu, Rosa Alvarez, Nadia Hindi, Cristina Mata, Gloria Marquina, Virginia Martínez, Andres Redondo, Luis Javier Floría, M. Carmen Source Type: research

Sclerosing Encapsulating Peritonitis in a Pediatric Patient Treated With Cytoreductive Surgery and Hyperthermic Intraperitoneal Chemotherapy
Conclusion: Although there have been published reports of adult patients experiencing cases of SEP following CRS/HIPEC, this is the first published case of secondary SEP occurring in a pediatric oncology patient. (Source: Journal of Pediatric Hematology Oncology)
Source: Journal of Pediatric Hematology Oncology - July 1, 2021 Category: Hematology Tags: Online Articles: Clinical and Laboratory Observations Source Type: research

Clinicopathological features of desmoplastic small round cell tumors: clinical series and literature review
Desmoplastic small round cell tumor (DSRCT) is a highly malignant sarcoma that occurs in the abdominopelvic cavities of adolescents. The accurate diagnosis of DSRCT is challenging owing to limited literatures.... (Source: World Journal of Surgical Oncology)
Source: World Journal of Surgical Oncology - June 30, 2021 Category: Cancer & Oncology Authors: Ling-Ling Wang, Zhong-He Ji, Ying Gao, Hong Chang, Ping-Ping Sun and Yan Li Tags: Research Source Type: research

EWSR1-WT1 gene fusions in neoplasms other than desmoplastic small round cell tumor: a report of three unusual tumors involving the female genital tract and review of the literature
Modern Pathology, Published online: 07 June 2021; doi:10.1038/s41379-021-00843-5EWSR1-WT1 gene fusions in neoplasms other than desmoplastic small round cell tumor: a report of three unusual tumors involving the female genital tract and review of the literature (Source: Modern Pathology)
Source: Modern Pathology - June 7, 2021 Category: Pathology Authors: J. Kenneth Schoolmeester Andrew L. Folpe Asha A. Nair Kevin Halling Bobbie Collett Sutton Emily Landers Anthony N. Karnezis Brendan C. Dickson Marisa R. Nucci David L. Kolin Source Type: research

Desmoplastic Small Round Cell Tumor of the Kidney with Mainly Pulmonary Symptoms by 18F-FDG PET/CT
Desmoplastic small round cell tumor (DSRCT) is a rare malignancy believed to originate from the serous membranes and it is highly aggressive with 5-year overall survival of 18.4%. Only a small number of DSRCT cases have been documented. Here, we report findings of DSRCT of the kidney on 18F-FDG PET/CT in a 30-year-old woman who presented with repetitive pulmonary infection and spontaneous pneumothorax. (Source: Urology)
Source: Urology - May 4, 2021 Category: Urology & Nephrology Authors: Ya Liu, Yi Tao, FeiFei Wang, Zhanwen Huang Tags: Clinical Challenges in Urology Source Type: research