scholarly journals Desmoplastic small round cell tumors of the pleura: a review of the clinical literature

2017 ◽  
Vol 12 ◽  
Author(s):  
Alessandro Giuseppe Fois ◽  
Pietro Pirina ◽  
Antonella Arcadu ◽  
Francesca Becciu ◽  
Sandra Manca ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Cell Tumor ◽  
Chest Roentgenogram ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
First Case ◽  
Rare Malignancy ◽  
Round Cell Tumors

Desmoplastic small round cell tumor of the pleura is a rare malignancy, with only a few cases reported in the scientific literature. The aim of the present review is to discuss the demographic, pathological, clinical, and therapeutic features of this rare tumor. English-language articles published since 1989, when the first case of desmoplastic small round cell tumor of the pleura was described, were retrieved, and fifteen cases included in fourteen articles were revised. The mean age of the patients was 25.5 years, out of them 60% were males. Chest pain, pleural effusion, and dyspnea were the most common clinical manifestations, while chest roentgenogram and computed tomography were the imaging techniques most commonly used. Surgical biopsy was employed in 80% of the cases for diagnosis. A multidisciplinary approach consisting in a combination of surgery with chemotherapy and radiation therapy was adopted in most cases. Only two patients (13.3%) were alive at 3 years from diagnosis, reflecting the aggressiveness of the disease, and the poor outcomes of the treatments currently available. Desmoplastic small round cell tumors of the pleura are extremely aggressive and challenging to diagnose, because of their rarity and unspecific demographic, clinical, and radiological features. An in-depth knowledge of such features is necessary for the optimal management of patients with this rare malignancy.

Variant EWS-WT1 Chimeric Product in the Desmoplastic Small Round Cell Tumor

1999 ◽  
Vol 2 (2) ◽  
pp. 188-192 ◽  
Author(s):  
Agnes S. Chan ◽  
Sue MacNeill ◽  
Paul Thorner ◽  
Jeremy Squire ◽  
Maria Zielenska
Keyword(s):  
Chromosomal Translocation ◽  
Cell Tumor ◽  
Round Cell ◽  
Chromosome 11 ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Binding Domains ◽  
First Case ◽  
Hybrid Genes

Chromosome translocations found in neoplasms often result in the creation of hybrid genes encoding chimeric proteins. Desmoplastic small round cell tumor (DSRCT) is a recently described aggressive malignancy associated with a unique chromosomal translocation t(11;22)(p13; q12). This translocation has recently been characterized, revealing the rearrangement and fusion of the WT1 gene on chromosome 11 to the EWS gene on chromosome 22. Fusion of these two genes results in the production of a putative oncogenic protein composed of the zinc finger DNA-binding domains of WT1 linked to the potential transcriptional regulatory domains of EWS. The typical chimeric transcript consists of the first 7 exons of EWS and the last 3 exons of WT1. We report here the first case of DSRCT with a variant EWS-WT1 chimeric product that includes 9 exons of EWS and 3 exons of WT1.

scholarly journals Intra-Abdominal Desmoplastic Small Round Cell Tumor: Current Treatment Options and Perspectives

2021 ◽  
Vol 11 ◽  
Author(s):  
Guixia Wei ◽  
Xinyao Shu ◽  
Yuwen Zhou ◽  
Xia Liu ◽  
Xiaorong Chen ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Cell Tumor ◽  
High Dose ◽  
Rapid Progression ◽  
Round Cell ◽  
Differentiation Markers ◽  
Soft Tissue Neoplasm ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

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.

scholarly journals A Rare Malignancy in an Adolescent: Desmoplastic Small Round Cell Tumor

2021 ◽  
Vol 29 (1) ◽  
pp. 45-50
Author(s):  
Güleç Mert DOĞAN ◽  
Ahmet SIĞIRCI ◽  
Arzu AKYAY ◽  
Sema UĞURALP ◽  
Merve Nur GÜVENÇ
Keyword(s):  
Cell Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Rare Malignancy

scholarly journals Blue Round Small Cell tumor: A Surgical Update of DSRCT with review of literature ‘A Grim Affair’

2021 ◽  
pp. 31
Author(s):  
Keyword(s):  
Clinical Manifestations ◽  
Small Cell ◽  
Abdominal Discomfort ◽  
Primary Site ◽  
Cell Tumor ◽  
Round Cell ◽  
Review Of Literature ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Desmoplastic small round cell tumor (DSRCT) is a tumor derived from the Greek desmos referring to knot and plasis to formation an uncommon soft tissue malignant tumor, mesenchymal in origin and aggressive with a prelidiction for males and advanced at presentation. It was first described as a distinct clinical entity by Gerald WL and Rosai J (7). There are fewer than 200 reported to date. Depending on the primary site of location the Clinical manifestations vary. As most arise from the abdomen and pelvis they remain asymptomatic till they attain a huge size. Other reported sites are the skull, thorax, and paratesticular region (10,13). We report the case of a 19 yr old male who had non specific abdominal discomfort with asthenia for a period of six months and was referred to us for evaluation of left supraclavicular nodes. The prognosis of Desmoplastic small round cell tumor (DSRCT) is poor with few surviving less than two years.

Primary Desmoplastic Small round Cell Tumor of the Kidney: A Case Report in a 14-Year-Old Girl with Molecular Confirmation

2007 ◽  
Vol 10 (4) ◽  
pp. 320-324 ◽  
Author(s):  
Sophie Collardeau-Frachon ◽  
Dominique Ranchère-Vince ◽  
Olivier Delattre ◽  
Stelly Hoarau ◽  
Philippe Thiesse ◽  
...  
Keyword(s):  
Fusion Transcript ◽  
Cell Tumor ◽  
Round Cell ◽  
Young Males ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell ◽  
Molecular Features ◽  
The Subject ◽  
Round Cell Tumors

We report a case of desmoplastic small round cell tumor (DSRCT) arising in the kidney of a 14-year-old female. The subject presented with gross hematuria. Medical imaging uncovered a left renal mass without regional or metastatic extension. The tumor showed morphological, immunohistochemical, and molecular features of DSRCT. Immunostaining revealed polyphenotypic differentiation. Molecular analysis detected the fusion transcript resulting from the t(11;22)(p13;q12) reciprocal translocation, which characterized this neoplasm. Desmoplastic small round cell tumor is a rare, aggressive neoplasm that mainly affects young males and that usually presents with widespread abdominal serosal involvement. This unusual localization should lead one to consider this tumor in the differential diagnosis of small blue round cell tumors of the kidney.

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 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.

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