scholarly journals Ewing's Sarcoma and Primary Osseous Lymphoma: Spectrum of Imaging Appearances

2019 ◽  
Vol 23 (01) ◽  
pp. 036-057 ◽  
Author(s):  
Olympia Papakonstantinou ◽  
Violeta Nikodinovska ◽  
Filip Vanhoenacker ◽  
Marc-André Weber
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Tumor Stroma ◽  
Conventional Radiography ◽  
Long Bone ◽  
Round Cell ◽  
Resonance Imaging ◽  
Tissue Component ◽  
Grade Ii ◽  
Hematoxylin And Eosin

Ewing's sarcoma (ES) is a rare, highly malignant anaplastic stem cell tumor. Histologically, the tumor consists of uniform densely packed small monomorphic cells with round nuclei. The typical appearance at hematoxylin and eosin (H&E) staining is small blue round cells without any matrix formation. On conventional radiography, ES typically presents as a permeative lesion in the diaphysis of a long bone in a child. A large soft tissue component is another characteristic feature, best depicted by magnetic resonance imaging.Primary osseous lymphomas are most commonly highly malignant B-cell lymphomas. At H&E histologic staining, the tumor stroma consists of diffuse round-cell infiltrates that resembles the appearance of ES. Although there is no typical imaging appearance of an osseous lymphoma, it should be considered in an adult presenting with a Lodwick grade II or III lesion in the metaphysis or diaphysis of a large long bone, the pelvis, or the vertebral column. Histologic confirmation is mandatory.

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.

scholarly journals Ewing’s sarcoma of sinonasal tract: a rare case

2020 ◽  
Vol 6 (3) ◽  
pp. 565
Author(s):  
Ankur Gupta ◽  
Ancy S. Sofia ◽  
Kanwar Sen
Keyword(s):  
Multidisciplinary Approach ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Histopathological Examination ◽  
Malignant Tumour ◽  
Rare Entity ◽  
Round Cell ◽  
First Line ◽  
Cytogenetic Studies ◽  
Immunohistochemical Studies

<p class="abstract">Extra skeletal Ewing’s sarcoma (EES) is a rare, rapidly growing, round cell malignant tumour that can develop in the soft tissue at any location. Involvement of the paranasal sinus is a very rare entity. Nearly 80% of patients are younger than 20 years. Diagnosis is made after histopathological examination, immunohistochemical studies and cytogenetic studies. Treatment includes a multidisciplinary approach with surgery as the first line followed by chemotherapy and radiotherapy.</p><p class="abstract"> </p>

scholarly journals Ewing's Sarcoma of the Cervical Spine

2017 ◽  
Vol 07 (02) ◽  
pp. 086-089
Author(s):  
Meenakshi Bhattacharjee ◽  
Winston Huh ◽  
Vandana Thapar ◽  
Paul Dahm
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Pediatric Population ◽  
Signs And Symptoms ◽  
Neck Mass ◽  
Neurological Deficits ◽  
Resonance Imaging ◽  
Neck Trauma ◽  
Motor Weakness ◽  
Posterior Neck

AbstractIn this article, we present the case of a 6-year-old female presented to the emergency department with progressive ascending motor weakness leading to cardiac arrest. The recent medical history included neck trauma 1 month prior to admission, 2 weeks of subjective fevers, and 1 day of urinary incontinence. After stabilization, and a review of the recent signs and symptoms, a magnetic resonance imaging of the neck revealed a posterior neck mass from C2 to T2. Neurosurgical removal of the mass was consistent with Ewing's sarcoma. Neck pain is a common presentation in the pediatric population, with the most common cause being traumatic. When coupled with neurological deficits, further studies are warranted to evaluate for organic causes.

scholarly journals Role of immunohistochemistry in the diagnosis of malignant small round cell tumors

1970 ◽  
Vol 1 (2) ◽  
pp. 87-91
Author(s):  
R Bashyal ◽  
TB Pathak ◽  
S Shrestha ◽  
CB Pun ◽  
S Banstola ◽  
...  
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Small Cell ◽  
Low Grade ◽  
Round Cell ◽  
Small Round Cell ◽  
Small Cell Variant ◽  
Valuable Adjunct ◽  
Cell Variant ◽  
Round Cell Tumors

Background: Immunohistochemistry is a key tool for the analysis of localization of target molecules within tissues. It has a significant role in the identification of tumors lacking evidence of lineage differentiation on the basis of routine light microscopic morphology alone. Approximately 90% of tumors posing diagnostic difficulties by morphology could be accurately classified by exploiting immunohistochemistry. The aim of this study is to identify the true identity of malignant small round cell tumors by immunohistochemical analysis. Materials and Methods:This was a retrospective study done in Department of Histopathology of B.P.Koirala Memorial Cancer Hospital from January 2010 to April 2011.A total of 40 cases small round cell tumors were selected for immunostaining. The immunohistochemistry technique used is the Polymer detection-EnvisionTM System, a two step staining technique based on Horse Radish Peroxidase labeled dextran polymer technology (DAKO Company). Results: Out of 40 cases of malignant small round cell tumors, there were 21 cases (52.5%) of Non- Hodgkin Lymphoma , 11 cases (27.5%) of Ewing’s Sarcoma/Primitive Neuroectodermal Tumor, 1 case (2.5%) of Lymphoblastic Lymphoma , 1 case (2.5%) of Rhabdomyosarcoma, 2 cases (5%) of Low grade neuroendocrine tumor, 1 case (2.5%) of Neuroblastoma, 2 cases (5%) of Poorly differentiated Synovial Sarcoma (small cell variant), 1case (2.5%) of Malignant Melanoma (small cell variant). Conclusion: Immunohistochemistry is a valuable adjunct to routine hematoxylin and eosin staining for adequate and accurate categorization of malignant small round cell tumors. Keywords: Immunohistochemistry; Malignant Small Round Cell Tumor; Non Hodgkin’s Lymphoma; Ewing’s Sarcoma; Rhabdomyosarcoma DOI: http://dx.doi.org/10.3126/jpn.v1i2.5398 JPN 2011; 1(2): 87-91

Ewing’s sarcoma/primitive neuroectodermal tumor of the prostate a case report and literature review

2013 ◽  
Vol 7 (5-6) ◽  
Author(s):  
Tao Wu ◽  
Tao Jin ◽  
Deyi Luo ◽  
Lin Chen ◽  
Xiang Li
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Transrectal Ultrasound ◽  
Neuroectodermal Tumor ◽  
Resonance Imaging ◽  
Mri Scan ◽  
Difficult Defecation ◽  
Magnetic Resonance Imaging Mri ◽  
Multiagent Chemotherapy

We present a case of Ewing’s sarcoma (ES) and primitive neuroectodermal tumor (PNET) of prostate. A 29-year-old male presented with difficult defecation and anus distention and was found on magnetic resonance imaging (MRI) scan of the pelvis to have a prostate tumor. A transrectal ultrasound guided needle biopsy confirmed the diagnosis. The patient underwent cystoprostatectomy and replacement ileocystoplasty and was followed by multiagent chemotherapy. PNET/EWS of the prostate is an extremely rare neoplasm. And the prognosis is very poor, so we should pay enough attention to the differential diagnosis and treatment.

Extraskeletal Ewing's Sarcoma/Primitive Neuroectodermal Tumor of the Posterior Mediastinum with t(11;22)(q24;q12)

2008 ◽  
Vol 94 (6) ◽  
pp. 888-891 ◽  
Author(s):  
Marosh Manduch ◽  
David F Dexter ◽  
Peter M Ellis ◽  
Kenneth Reid ◽  
Phillip A Isotalo
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Posterior Mediastinum ◽  
Neuroectodermal Tumor ◽  
Malignant Neoplasms ◽  
Round Cell ◽  
Mediastinal Tumors ◽  
Small Round Cell ◽  
Extraskeletal Ewing’S Sarcoma

Ewing's sarcoma/primitive neuroectodermal tumor family of tumors is part of a rare group of malignant neoplasms with small round-cell morphology. We describe a 24-year-old woman who presented with non-specific back pain. A chest radiograph and magnetic resonance imaging demonstrated an extraosseous, dumbbell-shaped mass of the posterior mediastinum with extension into the spinal canal. The patient underwent a left posterolateral thoracotomy and a T3–5 laminectomy with subsequent multi-agent chemotherapy. Histopathologic examination of the tumor demonstrated sheets of primitive small round malignant cells that showed no visible differentiation. Neoplastic cells were strongly immunoreactive for CD99 and vimentin and were negative for chromogranin, synaptophysin, CD31, CD34, calcitonin, desmin, low-molecular weight cytokeratins, wide-spectrum cytokeratins, leukocyte common antigen, S-100 protein, and thyroid transcription factor-1. The neoplasm was diagnosed as a Ewing's sarcoma/primitive neuroectodermal tumor, and cytogenetic studies confirmed a t(11;22)(q24;q12) chromosomal translocation and an associated trisomy of chromosome 2, supporting the histologic diagnosis. Extraskeletal Ewing's sarcoma/primitive neuroectodermal tumors are rare neoplasms that should be distinguished from other small round-cell tumors by morphology and ancillary laboratory techniques. Although rare, they need to be considered in the differential diagnosis of primary mediastinal tumors.

scholarly journals Novel Secondary Somatic Mutations in Ewing's Sarcoma and Desmoplastic Small Round Cell Tumors

PLoS ONE ◽  
2014 ◽  
Vol 9 (8) ◽  
pp. e93676 ◽  
Author(s):  
Yunyun Jiang ◽  
Vivek Subbiah ◽  
Filip Janku ◽  
Joseph A. Ludwig ◽  
Aung Naing ◽  
...  
Keyword(s):  
Somatic Mutations ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Round Cell ◽  
Small Round Cell ◽  
Round Cell Tumors

scholarly journals Radiation diagnostics of peripheral primitive neuroectodermal tumors (PNET) in children at the Scientific Center of Pediatrics and Pediatric Surgery (Almaty, Kazakhstan)

2020 ◽  
Vol 58 (4) ◽  
pp. 36-40
Author(s):  
U. IBRAGIMOV ◽  
S. SARGELOV ◽  
M. ENSEPBAEV ◽  
A. TAYNEKOVA
Keyword(s):  
Soft Tissue ◽  
Primitive Neuroectodermal Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Abdominal Cavity ◽  
Neuroectodermal Tumor ◽  
Peripheral Primitive Neuroectodermal Tumor ◽  
Tissue Component ◽  
Soft Tissue Component ◽  
Medical Network

Relevance: Peripheral primitive neuroectodermal tumor (primitive neuroectodermal tumor – PNET) belongs to the group of malignant tumors that develop from migrating embryonic neural crest cells. PNET includes several nosological forms: Askin’s tumor, esthesioneuroblastoma, the very peripheral primitive neuroectodermal tumor, and Ewing’s sarcoma. PNET accounts for 3–9% of all soft tissue tumors and 19% of all soft tissue sarcomas in children. In Europe and the US, PNETs account for 3.4 cases per year per 1 million children below 15 years; in Kazakhstan – 0.6-1.2 cases per 1 million child population. Rapid tumor growth, malignancy, and early metastasis to other organs and systems predetermine the PNET’s specific role in oncology. The purpose of the study was to improve the quality and availability of early sarcoma diagnostics in children at medical institutions of the general medical network. Results: 35 cases of peripheral PNET in children were analyzed. The age of the patients was 1.5 to 17 years, the average age – 9.3 years. Boys were 1.3 times more than girls. One patient (3.6%) had extra-skeletal tumor localization. Children with stage IIB prevailed – 46.4% of cases (13 children). Radiographical differentiation between Ewing’s sarcoma and primary chronic or “healed” (antibiotic) acute hematogenous osteomyelitis in the initial phase of the process is almost impossible before the extraosseous soft tissue component is formed. The bone damage process is more often localized in the bone diaphysis and subsequently spreads to its metaphyses. Conclusion: PNET is more likely to come from the chest wall, so it is advisable to start the X-ray examination from the chest. In terms of radiation semiotics, PNET is similar to Ewing’s sarcoma and Askin’s tumor; therefore, an additional immunohistochemical study of the tumor tissue is required. An important indirect diagnostic criterion in Ewing’s sarcoma is the predominance of the soft tissue component over the bone manifestations. Extended CT and MRI studies with contrast enhancement (chest, abdominal cavity, pelvis, and the primary lesion area) and skeletal scintigraphy are required to clarify the extent of changes, stage the tumor accurately, and assess the tumor dynamics after treatment. The above conclusions generally confirm the available literature data.

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