scholarly journals EWSR1 Rearrangement and CD99 Expression as Diagnostic Biomarkers for Ewing/PNET Sarcomas in a Moroccan Population

2018 ◽  
Vol 2018 ◽  
pp. 1-5 ◽  
Author(s):  
Sara Louati ◽  
Nadia Senhaji ◽  
Laila Chbani ◽  
Sanae Bennis
Keyword(s):  
Differential Diagnosis ◽  
Sensitivity And Specificity ◽  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Neuroectodermal Tumor ◽  
Fish Analysis ◽  
Round Cell ◽  
Diagnostic Biomarkers ◽  
Moroccan Population ◽  
Round Cell Tumors

Ewing sarcoma/primitive neuroectodermal tumor (Ewing/PNET sarcomas or EPS) are a group of round cell tumors. Malignant round cell tumors form a large and diverse group that includes rhabdomyosarcoma, synovial sarcoma, non-Hodgkin’s lymphoma, neuroblastoma, hepatoblastoma, Wilm’s tumor, desmoplastic small round cell tumor, and other morphologically similar entities. Differential diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (Ewing/PNET sarcomas or EPS) is difficult. In addition to morphology and immunohistochemistry (IHC), differential diagnosis of these tumors is based on molecular analysis of the EWSR1 gene rearrangement using fluorescent in situ hybridization (FISH) technique. We investigated the diagnostic value of combined CD99 immunostaining and EWSR1 t(22q12) alteration using a dual-color, break-apart rearrangement probe in forty-one formalin-fixed paraffin-embedded (FFPE) tissue samples from pediatric and adult patients diagnosed with EPS. IHC was performed in all cases using the CD99 antibody and showed a positivity of 92.7% in the enrolled cases (38/41) followed by FISH analysis where 48.8% of the cases (20/41) were rearranged. Sensitivity and specificity for IHC assays were 88% and 58%, respectively. Notably, FISH had a sensitivity of 100% and a specificity of 87%. In addition, CD99 positivity was found to correlate with EWSR1 rearrangement (p<0.05). This report shows that FISH has better sensitivity and specificity than IHC in the Moroccan population, and supports its combination with CD99 immunostaining as diagnostic biomarkers for this rare malignant entity.”

Primitive Neuroectodermal Tumor of the Gallbladder

2004 ◽  
Vol 128 (5) ◽  
pp. 571-573 ◽  
Author(s):  
Dong Eun Song ◽  
Ga Won Choi ◽  
Sun-Young Jun ◽  
Jae Y. Ro
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Soft Tissues ◽  
Neuron Specific Enolase ◽  
Neuroectodermal Tumor ◽  
Chromosomal Translocations ◽  
Common Antigen ◽  
Round Cell ◽  
Small Round Cell ◽  
Positive Immunoreactivity ◽  
Round Cell Tumors

Abstract Ewing sarcoma/primitive neuroectodermal tumor (ES/ PNET) most commonly occurs in the soft tissues of the extremities and the paravertebral areas. To our knowledge, no case of PNET has been reported in the gallbladder to date. We report a case of PNET of the gallbladder occurring in a 53-year-old woman. Microscopically, the monotonous small round cells with a formation of Homer-Wright rosettes showed intense membranous positive immunoreactivity for MIC2/CD99, as well as for neuron-specific enolase and synaptophysin. Other small round cell tumors, such as lymphoma, rhabdomyosarcoma, or melanoma, were excluded in light of negative immunoreactivity for leukocyte common antigen, desmin, and S100. The diagnosis of PNET was rendered based on the characteristic morphology and immunohistochemical findings, despite an absence of chromosomal translocations, such as t(11; 22)(q24;q12) and t(21;22)(q22;q12).

Pelvic primitive neuroectodermal tumor associated with a cluster of small round cell tumors: case report and review of current literature☆

2003 ◽  
Vol 91 (1) ◽  
pp. 247-253 ◽  
Author(s):  
Bobbie S Gostout ◽  
Noralane M Lindor ◽  
Connie S DiMarco ◽  
Prema P Peethambaram ◽  
Amy C Clayton
Keyword(s):  
Case Report ◽  
Current Literature ◽  
Primitive Neuroectodermal Tumor ◽  
Neuroectodermal Tumor ◽  
Round Cell ◽  
Small Round Cell ◽  
Round Cell Tumors

scholarly journals WT1 Staining Reliably Differentiates Desmoplastic Small Round Cell Tumor From Ewing Sarcoma/Primitive Neuroectodermal Tumor

2000 ◽  
Vol 114 (3) ◽  
pp. 345-353 ◽  
Author(s):  
D. Ashley Hill ◽  
John D. Pfeifer ◽  
Edith F. Marley ◽  
Louis P. Dehner ◽  
Peter A. Humphrey ◽  
...  
Keyword(s):  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Cell Tumor ◽  
Neuroectodermal Tumor ◽  
Round Cell ◽  
Round Cell Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Melanotic Neuroectodermal Tumor of Infancy: A Molecular Genetic Study

1998 ◽  
Vol 1 (4) ◽  
pp. 295-299 ◽  
Author(s):  
Maliheh Khoddami ◽  
Jeremy Squire ◽  
Maria Zielenska ◽  
Paul Thorner
Keyword(s):  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Molecular Genetic ◽  
Small Cell ◽  
Cell Tumor ◽  
Neuroectodermal Tumor ◽  
Round Cell ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Small Round Cell Tumor ◽  
Small Round Cell

Melanotic neuroectodermal tumor of infancy is a rare but well-recognized entity in pediatric pathology. However, the relationship of this tumor to other pediatric small cell tumors with neuroectodermal features (such as neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, and desmoplastic small round cell tumor) is undetermined. Molecular genetic studies of melanotic neuroectodermal tumor of infancy have not been reported. We studied three typical cases of mela-notic neuroectodermal tumor of infancy in an attempt to link this tumor to other small cell tumors with well-characterized molecular genetic changes. Tests performed included: detection of MYCN gene amplification and deletion of 1p (all 3 cases), and presence of the t(11;22)(q24;q12) and the t(11;22)(p13;q12) translocations (2 of 3 cases). None of these tests yielded positive results. Thus, there is no genetic basis at present to link melanotic neuroectodermal tumor of infancy to neuroblastoma, Ewing sarcoma/peripheral primitive neuroectodermal tumor, or desmoplastic small round cell tumor.

scholarly journals Primary gastric Ewing sarcoma/primitive neuroectodermal tumor

2021 ◽  
Vol 49 (2) ◽  
pp. 030006052098668
Author(s):  
Yu Ye ◽  
Xiaoming Qiu ◽  
Jixin Mei ◽  
Dongyun He ◽  
Ailing Zou
Keyword(s):  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Distal Gastrectomy ◽  
Histopathological Examination ◽  
Posterior Wall ◽  
Neuroectodermal Tumor ◽  
Whole Body ◽  
Round Cell ◽  
Ulcerative Lesion ◽  
Enhanced Ct

A rare and highly malignant small round cell tumor, Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) usually occurs in the pelvis, long-axis bones, and femur. In contrast, extraosseous ES is more often found in the paraspinal region, limbs, and retroperitoneum, but is extremely rare in the stomach. We report a case of a 55-year-old woman who presented with fatigue, fever, and black stool. Preoperative computed tomography (CT) imaging showed a large ulcerative lesion of approximately 5.5 × 5.0 cm in the stomach and irregular thickening of the ulcer wall. Upper endoscopy revealed a large, irregular ulcer in the posterior wall of the stomach. Histopathological examination suggested that the mass with the largest diameter (7.5 cm) was ES. Immunohistochemistry indicated positivity for CD99. Enhanced CT of the whole body was performed but no definite masses were found in other organs, and the patient was diagnosed with primary gastric ES. The patient underwent radical distal gastrectomy with Roux-en-Y gastrojejunostomy, but refused chemoradiotherapy.

scholarly journals Epithelioid Leiomyosarcoma in a Non-Immunocompromised Infant: Additional Differential Diagnosis of Pediatric “Round Cell Tumors”

2000 ◽  
Vol 13 (10) ◽  
pp. 1156-1160 ◽  
Author(s):  
Keisuke Kato ◽  
Kokoro Arai ◽  
Yukichi Tanaka ◽  
Rieko Ijiri ◽  
Yoshikazu Kato ◽  
...  
Keyword(s):  
Differential Diagnosis ◽  
Round Cell ◽  
Round Cell Tumors

Primitive Neuroectodermal Tumor of the Stomach

2005 ◽  
Vol 129 (1) ◽  
pp. 107-110 ◽  
Author(s):  
Raoulin Soulard ◽  
Valère Claude ◽  
Philippe Camparo ◽  
Jean-Philippe Dufau ◽  
Patrick Saint-Blancard ◽  
...  
Keyword(s):  
Polymerase Chain Reaction ◽  
Young Adults ◽  
Soft Tissue ◽  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Neuron Specific Enolase ◽  
Neuroectodermal Tumor ◽  
Chain Reaction ◽  
Children And Young Adults ◽  
Polymerase Chain

Abstract Ewing sarcoma/primitive neuroectodermal tumor is classically a tumor of the soft tissue or bone in children and young adults, but several cases have been described in patients of all ages. Within the last decade, the clinicopathologic spectrum of Ewing sarcoma/primitive neuroectodermal tumor has been markedly expanded by recognition that the tumor may also have a visceral origin. We describe a case of primitive neuroectodermal tumor arising in the stomach of a 66-year-old woman. The neoplasm was excised using a radical surgical procedure. Microscopically, the tumor was made up of solid nests and sheets of round cells. Immunohistochemically, the tumor cells showed immunoreactivity for CD99, S100, neuron-specific enolase, and vimentin. A multiplex real-time polymerase chain reaction assay detected an EWS-ERG fusion. To our knowledge, this is the first description of a primitive neuroectodermal tumor arising in the stomach.

scholarly journals Primary Ewing sarcoma/primitive neuroectodermal tumor of the lung

2020 ◽  
Vol 10 (3) ◽  
Author(s):  
Devika Gupta ◽  
Tathagata Chatterjee ◽  
Rohit Tewari ◽  
Arti Trehan ◽  
Anuradha Ahuja
Keyword(s):  
Ewing Sarcoma ◽  
Primitive Neuroectodermal Tumor ◽  
Neuroectodermal Tumor
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