scholarly journals Myxoid/round cell and pleomorphic liposarcomas

Cancer ◽  
2007 ◽  
Vol 109 (12) ◽  
pp. 2522-2531 ◽  
Author(s):  
Marco Fiore ◽  
Federica Grosso ◽  
Salvatore Lo Vullo ◽  
Elisabetta Pennacchioli ◽  
Silvia Stacchiotti ◽  
...  
Keyword(s):  
Round Cell ◽  
Pleomorphic Liposarcomas

scholarly journals Managing Liposarcomas: Cutting Through the Fat

2016 ◽  
Vol 12 (3) ◽  
pp. 221-227 ◽  
Author(s):  
Gulam A. Manji ◽  
Gary K. Schwartz
Keyword(s):  
Treatment Plan ◽  
Soft Tissue Sarcomas ◽  
Definitive Treatment ◽  
Round Cell ◽  
Myxoid Liposarcomas ◽  
Well Differentiated ◽  
Third Line ◽  
Line Setting ◽  
Pleomorphic Liposarcomas ◽  
Histologic Subtypes

Liposarcomas are one of the most common of more than 50 histologic subtypes of soft tissue sarcomas that, themselves, are heterogeneous. Liposarcomas fall into four distinct histologic subtypes: atypical lipomatous tumor/well-differentiated liposarcoma, dedifferentiated liposarcoma, myxoid (round cell) liposarcoma, and pleomorphic liposarcoma. Definitive treatment remains surgical resection with negative margins for resectable disease. However, well-differentiated liposarcomas that are large or difficult to operate upon should be followed with close surveillance as long as there is no radiologic concern for a dedifferentiated component. In contrast, first-line chemotherapy with anthracycline with or without ifosfamide, or gemcitabine and docetaxel should be used for inoperable myxoid (round cell) or pleomorphic liposarcomas, which are relatively responsive to chemotherapy. In the second- and third-line setting, myxoid liposarcomas, in particular, seem to be sensitive to trabectedin, which was recently approved by the US Food and Drug Administration (FDA). Eribulin offered a survival benefit when compared with dacarbazine in the third-line setting in liposarcomas (other than the well-differentiated subtype) and is now FDA approved. Recent studies have identified distinct genetic aberrations that not only aid in the diagnosis of liposarcoma subtypes but represent actionable targets. Cyclin-dependent kinase 4 and murine double minute 2 are overexpressed in well-differentiated and dedifferentiated liposarcomas and offer opportunities that are being pursued in clinical trials. It is critical that liposarcomas are not approached by oncologists as one disease entity but rather subclassified into distinct subtypes using histologic and molecular tools before formalizing a treatment plan.

scholarly journals DDIT3 Expression in Liposarcoma Development

Sarcoma ◽  
2014 ◽  
Vol 2014 ◽  
pp. 1-6 ◽  
Author(s):  
Christina Kåbjörn Gustafsson ◽  
Katarina Engström ◽  
Pierre Åman
Keyword(s):  
Transcription Factor ◽  
Round Cell ◽  
Mesenchymal Tumors ◽  
Cell Subpopulations ◽  
Well Differentiated ◽  
Pleomorphic Liposarcomas ◽  
Lipomatous Tumors ◽  
Genetic Rearrangements

Liposarcomas are mesenchymal tumors containing variable numbers of lipoblasts or adipocytes. The most common entities, well differentiated/dedifferentiated liposarcoma (WDLS/DDLS) and myxoid/round cell liposarcoma (MLS/RCLS), are both characterized by genetic rearrangements that affect the expression of the transcription factor DDIT3. DDIT3 induces liposarcoma morphology when ectopically expressed in a human fibrosarcoma. The role of DDIT3 in lipomatous tumors is, however, unclear. We have analyzed the expression of DDIT3 in 37 cases of liposarcoma (WDLS/DDLSn= 10, MLS/RCLSn= 16, and pleomorphic liposarcomas (PLS)n= 11) and 11 cases of common benign lipomas. Major cell subpopulations of WDLS/DDLS and MLS/RCLS tumors were found to express DDIT3 or the derived fusion protein, whereas PLS cases showed only a few positive cells. The lipomas contained large subpopulations expressing DDIT3. No correlation between numbers of DDIT3 expressing cells and numbers of lipoblasts/adipocytes was found. In vitro adipogenic treatment of two DDIT3 expressing cell lines induced lipid accumulation in small subpopulations only. Our results suggest a dual, promoting and limiting, role for DDIT3 in the formation of lipoblasts and liposarcoma morphology.

Desmoplastic small round cell tumor showing solid proliferation with limited desmoplasia and confusing immunohistochemical findings: an autopsy report

2020 ◽  
Vol 53 (3) ◽  
pp. 177-182
Author(s):  
Atsushi Kihara ◽  
Kazuya Takahashi ◽  
Ayataka Ishikawa ◽  
Yusuke Amano ◽  
Daisuke Matsubara ◽  
...  
Keyword(s):  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Autopsy Report

scholarly journals Early radical cystectomy with negative margins in a 57-year-old female with myxoid/round cell liposarcoma of the bladder suggests prolonged overall survival

2021 ◽  
Vol 27 (1) ◽  
Author(s):  
Johannes Eduard Delport ◽  
Khanyisa Makamba
Keyword(s):  
Overall Survival ◽  
Surgical Intervention ◽  
Radical Surgery ◽  
Bladder Tumour ◽  
Eastern Cape ◽  
Round Cell ◽  
Female Population ◽  
Case Presentation ◽  
Rural Town ◽  
Common Cancer

Abstract Background Bladder cancer is the 17th most common cancer in the female population. Most bladder cancers are of urothelial origin. Sarcomas of the bladder are very uncommon. Case presentation This case concerns a 57-year-old female from a rural town in the Eastern Cape who was diagnosed with myxoid/round cell liposarcoma of the bladder. After the initial transurethral resection of the bladder tumour, she underwent an anterior exenteration with Bricker’s diversion (ileal conduit) and negative margins were achieved. Conclusions Sarcomas of the bladder are known to have a poor prognosis. Our patient is alive with good stoma function more than 24 months since her radical surgery. We are of the opinion that her favourable overall survival is attributable to early radical surgical intervention with negative margins.

scholarly journals CIC-DUX4 rearranged uterine cervix round-cell sarcoma exhibiting near-complete pathologic response following radiation and neoadjuvant chemotherapy: A case report

2021 ◽  
Vol 36 ◽  
pp. 100745
Author(s):  
Aline Cristini Vieira ◽  
Camila Bragança Xavier ◽  
Thiago Dieb Vieira ◽  
Filomena Marino Carvalho ◽  
Mariana Scaranti ◽  
...  
Keyword(s):  
Case Report ◽  
Neoadjuvant Chemotherapy ◽  
Uterine Cervix ◽  
Pathologic Response ◽  
Complete Pathologic Response ◽  
Round Cell ◽  
Cell Sarcoma

scholarly journals FDG PET/CT imaging of desmoplastic small round cell tumor: findings at staging, during treatment and at follow-up

2015 ◽  
Vol 45 (9) ◽  
pp. 1308-1315 ◽  
Author(s):  
Austin Ostermeier ◽  
M. Beth McCarville ◽  
Fariba Navid ◽  
Scott E. Snyder ◽  
Barry L. Shulkin
Keyword(s):  
Fdg Pet ◽  
Ct Imaging ◽  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Pet Ct ◽  
Fdg Pet Ct

scholarly journals Desmoplastic Small Round Cell Tumor of the Submandibular Gland: A Case Report and Literature Review

ORL ◽  
2021 ◽  
pp. 1-6
Author(s):  
Qingjiao Li ◽  
Xiaolu Yuan
Keyword(s):  
Submandibular Gland ◽  
Young Patients ◽  
Neck Region ◽  
Cell Tumor ◽  
Pelvic Cavity ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
The Right

Desmoplastic small round cell tumor (DSRCT) is a rare and aggressively malignant tumor mostly occurring in the abdominal and pelvic cavity of young patients. However, few cases had been reported concerning DSRCT occurring in the head and neck region. 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 typically 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 apart involving the <i>Ewing sarcoma</i> (<i>EWS</i>) gene. The patient received chemotherapy and radiotherapy and relapsed after 19 months of follow-up. DSRCT of the submandibular gland is rare, and the diagnosis of this tumor in an uncommon location relies on the histomorphology, immunophenotype, and <i>EWS</i> gene translocation detection. Differential diagnosis including primary salivary gland tumors and the other small round cell tumors needs to be excluded.

scholarly journals Sinonasal FUS-ERG-Rearranged Ewing’s Sarcoma Mimicking Glomangiopericytoma

2020 ◽  
Vol 13 (3) ◽  
pp. 1393-1396
Author(s):  
Maggie Zhou ◽  
Yen Chen Kevin Ko ◽  
Gregory W. Charville ◽  
Kristen N. Ganjoo
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Staining Pattern ◽  
Gene Arrangement ◽  
Round Cell ◽  
The Family ◽  
History Of ◽  
Blue Cell ◽  
Aggressive Tumor ◽  
Membranous Staining

Ewing’s sarcoma is a rare and aggressive tumor that typically arises in the long bones of the extremities. It belongs in the family of small round blue cell tumors and is characterized immunohistochemically by diffuse CD99 expression and molecularly by one of several oncogenic translocations, most commonly t(11;22)(q24;q12) between the <i>EWSR1</i> gene and the <i>FLI1</i> gene. Here we present a rare case of Ewing’s sarcoma in the sinonasal tract with <i>FUS-ERG</i> gene arrangement that was regarded for almost a decade as a sinonasal-type hemangiopericytoma (glomangiopericytoma). This case illustrates the surprisingly prolonged natural history of Ewing’s sarcoma that did not receive therapy for many years and the importance of considering alternative genetic translocations. Our experience suggests that the presence of diffuse CD99 membranous staining pattern in a small blue round cell tumor with morphology typical for Ewing’s sarcoma but FISH negative for <i>EWSR1</i> rearrangement should prompt consideration of <i>FUS-ERG</i> fusion.

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