Undifferentiated round cell sarcoma of the broad ligament

Sarcomas of the broad ligament are very uncommon. To our knowledge, there are no cases published of undifferentiated round cell sarcoma of the broad ligament. Round cell sarcomas are a rare and very aggressive variant, which due to their sensitivity to chemotherapy, have an acceptable prognosis. We report the case of a 27-year-old woman who presented with a pelvic mass with a 7-cm diameter placed on the right broad ligament. After surgery, she was diagnosed with undifferentiated round cell sarcoma of the broad ligament. The patient received adjuvant chemotherapy and radiotherapy, and after 12 years of follow-up, she still remains asymptomatic. Proper differential diagnoses as well as an appropriate adjuvant therapy after surgical treatment seem to be essential to obtain good oncological outcomes in this rare entit

CIC Rearrangement Sarcoma: A Case Report and Literature Review

Background: CIC-rearranged sarcoma (capicua transcriptional repressor- rearranged sarcoma, CRS) is a rare type of undifferentiated small round-cell sarcoma. There are few reported cases of CRS; in 2017, 115 cases were reported abroad and 10 cases were reported in China. Case summary: The patient is a 41-year-old male who presented with a mass in the left lumbar region for more than 1 month. Tumor excision was performed at another hospital. Pathology results indicated CRS. PET-CT indicated changes in the left lumbar region, and postoperative tissue repair changes were considered. However, combined with the medical history and imaging features, the clinical diagnosis was considered recurrence of tumor in the left lumbar region. Postoperatively, the patient was transferred to the burn department for pedicled skin-flap repair. Conclusion: CRS is rare, and the prognosis of these patients is poor. Surgical resection of the lesion is the first choice for patients without metastasis

Evaluation of fine needle aspiration cytology in the diagnosis of soft tissue tumors and its correlation with histopathological findings

Background: Fine Needle Aspiration Cytology (FNAC) is a useful, safe and cost effective tool that is used in the diagnosis of lesions in various organs. Even though the literature on FNAC of soft tissue masses is relatively scarce, a large amount of interest has developed in this area in the last few years due to the low cost of the procedure, low incidence of complications, feasibility and high therapeutic efficiency. So the present study was undertaken to study the nature of various soft tissue tumors by FNAC and to compare the cytological diagnosis with the results obtained by biopsy.  Methods: All cases 713 (Prospective-217 and Retrospective 496) included in present series were taken up for study. Aspiration of soft tissue lesions were performed following the technique of Zajicek et al. Smear was prepared, stained and examined. Histopathological details were available in 140 cases. Data was compiled in MS excel and checked for its completeness, correctness and then it was analyzed.Results: Total number of 11,560 FNAC was done in six and half year’s period from 1st April 2003 to 30th September 2009. Out of the total 713 cases of soft tissue tumors, 71.25% were reported as benign and 28.75% as malignant. Soft tissue tumors were more common in males in comparison to females, with M: F-1.63:1. Accuracy of FNAC for benign and malignant soft tissue tumours was 88.1% and 92.9% respectively.  Conclusion: The overall diagnostic accuracy of FNAC in the present study was found 90%. FNAC is a safe and reliable method of recognizing benign and malignant soft tissue tumors and in most instances histological sub typing is possible. Cytological diagnosis must be based on strict cytological criteria and well controlled ancillary techniques.

NTRK3 overexpression in undifferentiated sarcomas with YWHAE and BCOR genetic alterations

The BCOR family of tumors includes a number of undifferentiated sarcomas, occurring in various age groups and anatomic sites, characterized by a spindle and round cell phenotype and diffuse immunoreactivity for BCOR. Prior RNA sequencing data revealed that NTRK3 was a top-upregulated gene in BCOR-CCNB3 sarcomas. In this study, we investigate a large cohort of tumors harboring BCOR/YWHAE genetic alterations for NTRK3 upregulation at both the mRNA and protein levels, compared with other sarcoma types. Pan-Trk immunohistochemistry was assessed for intensity and extent. A correlation between NTRK3 expression and the type of BCOR alteration and BCOR immunoreactivity was also performed. Most soft tissue undifferentiated round cell sarcomas with YWHAE or BCOR rearrangements or BCOR internal tandem duplications (ITD) showed NTRK3, but not NTRK1 or NTRK2, upregulation by RNA sequencing data analysis. Cytoplasmic pan-Trk immunoreactivity was also observed in most soft tissue round cell sarcomas with YWHAE rearrangements (100%), BCOR ITD (80%), and BCOR-CCNB3 fusions (67%), as well as clear cell sarcomas of kidney (75%), another BCOR family tumor, and ossifying fibromyxoid tumors with ZC3H7B-BCOR fusion (100%), with variable staining intensity and extent. Pan-Trk staining was also seen in solitary fibrous tumors (100%) and less frequently in synovial sarcoma and Ewing sarcoma, but rarely in other sarcomas tested. Tumors harboring rare fusion variants of BCOR, such as BCOR-CHD9, a novel fusion identified by targeted RNA sequencing, and KMT2D-BCOR, were also positive for pan-Trk staining and NTRK3 overexpression. In conclusion, NTRK3 upregulation resulting in pan-Trk overexpression is common in the BCOR family of tumors as well as in subsets of BCOR-expressing sarcomas through alternative mechanisms. The therapeutic implication of this finding awaits further investigation

Diagnostic Accuracy of Fine Needle Aspiration Cytology in Soft Tissue Sarcomas

Background: To study diagnostic validity of fineneedle aspiration cytology in soft tissue sarcoma.Methods: In this descriptive study patients of allages and both gender with obvious soft tissueswellings and patient in whom both FNAC followedby histopathology, were included. Cases withobvious inflammatory lesions were excluded fromthe study. Immunohistochemistry was employedwhere required.The diagnosis made on FNAC wasthen compared with the diagnosis made onhistopathology.Results: Mean age of the patients was 28 years±16yrs. Lower extremity tumours were seen in 65%.Most common soft tissue sarcoma on FNAC wasspindle cell sarcoma (11 cases) followed by roundcell sarcoma(10 cases). Subsequent histopathology ofcases showed 55% of benign soft tissue tumours and45% of soft tissue sarcomas. Hence there were 4 falsenegative cases and one false positive case oncytology. On comparing with histopathologicalcorrelation diagnostic accuracy of FNAC indiagnosing soft tissue sarcoma came out to be92.3%.The sensitivity and specificity of FNAC in softtissue sarcoma was 86.2 % and 97.2% respectively.Conclusion: FNAC is a useful procedure in preoperativediagnosis of benign and malignant softtissue tumours , with a high sensitivity, specificityand diagnostic accuracy in soft tissue sarcomas

The utility of ETV1, ETV4 and ETV5 RNA inâ situ hybridization in the diagnosis of CICâ DUX sarcomas

Peer Reviewedhttps://deepblue.lib.umich.edu/bitstream/2027.42/136260/1/his13112_am.pdfhttps://deepblue.lib.umich.edu/bitstream/2027.42/136260/2/his13112.pd

Undifferentiated round cell sarcoma with BCOR internal tandem duplications (ITD) or YWHAE fusions:a clinicopathologic and molecular study

Until recently, undifferentiated round cell sarcomas (URCS) in infants have been considered a wastebasket diagnosis, composed of various pathologic entities and lacking consistent genetic alterations. The recent identification of recurrent BCOR internal tandem duplications (ITD) and less common alternative YWHAE\u2013NUTM2B/E fusions in half of infantile URCS and the majority of so-called primitive myxoid mesenchymal tumors of infancy (PMMTI) suggests a common pathogenesis with clear cell sarcoma of the kidney which also harbors the same genetic alterations. These tumors also share a similar morphology and immunoprofile, including positivity for BCOR, cyclin D1, and SATB2. In this study, we investigate the largest cohort to date of genetically confirmed URCS and PMMTI with BCOR ITD or YWHAE fusions to better define their morphologic spectrum and clinical behavior. Twenty-eight cases harbored BCOR ITD and five YWHAE fusions, occurring in 29 infants and 4 children, 19 males and 14 females. Microscopically, 20 were classified as URCS and 13 as PMMTI. Follow-up was available in 25 patients, with 14 (56%) succumbing to their diseases at a mean duration of 18-months follow-up (range: 2\u201362). Six patients remained with no evidence of disease at a mean follow-up of 63 months (range: 4\u2013192), four patients were still alive with disease (mean follow-up: 46 months, range: 4\u2013120), and one died of other causes. Local recurrence and distant metastasis were each observed in 11/25 (44%) of the patients. The overall survival was 42% at 3 years and 34% at 5 years (median survival: 26 months). There was no statistically significant survival difference between cases diagnosed as URCS and PMMTI and between those with BCOR ITD and YWHAE fusions

A study of the incidence of cancer in a large general hospital during 1899-1923, inclusive

Thesis (M.A.)--Boston Universit