scholarly journals Primary Ewing’s Sarcoma of the Sinonasal Tract: A Case Report

2017 ◽  
Vol 10 (1) ◽  
pp. 91-97 ◽  
Author(s):  
Tomoharu Suzuki ◽  
Ryuji Yasumatsu ◽  
Torahiko Nakashima ◽  
Shuji Arita ◽  
Hidetaka Yamamoto ◽  
...  
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Maxillary Antrum ◽  
Chromosomal Translocation ◽  
Microscopic Analysis ◽  
Complete Removal ◽  
Mri Scans ◽  
History Of ◽  
Mitotic Figures ◽  
Contiguous Spread

A 23-year-old male presented with a 3-month history of left purulent rhinorrhea, progressive nasal obstruction, and intermittent epistaxis. A fiberoptic examination revealed a large vascular polypoid mass completely filling the left nasal cavity. CT and MRI scans showed a large hypervascular mass involving the left nasal airway, maxillary antrum, and the anterior ethmoid cells. There was no bony erosion or contiguous spread, and the remaining sinuses, orbit, and cranial fossa were uninvolved. The patient underwent complete removal of the mass via an external lateral rhinotomy approach. The soft mass was large and vascular. A microscopic analysis revealed an undifferentiated tumor consisting of a solid sheet of small, round blue cells. Mitotic figures were also present. Immunohistochemically, the tumor cells were strongly positive for CD99. Molecular studies using a PCR confirmed the chromosomal translocation of FLI1 (exon 6). These findings were considered diagnostic for Ewing’s sarcoma. Postoperatively, the patient was treated with combined chemotherapy and radiotherapy. Adjuvant chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide (total: 7 cycles) was commenced. He also received radiation therapy for local control (total dose: 50.4 Gy). The patient is currently alive without any evidence of recurrence or metastasis.

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 Primary Occipital Ewing’s Sarcoma with Subsequent Spinal Seeding

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Ali Alqahtani ◽  
Roaa Amer ◽  
Eman Bakhsh
Keyword(s):  
Posterior Fossa ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Bone Cancer ◽  
Surgical Excision ◽  
Good Prognosis ◽  
Bone Lesions ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
Whole Spine

Ewing’s sarcoma is a primary bone cancer that mainly affects the long bones. This malignancy is particularly common in pediatric patients. Primary cranial involvement accounts for 1% of cases, with occipital involvement considered extremely rare. In this case study, primary occipital Ewing’s sarcoma with a posterior fossa mass and subsequent relapse resulting in spinal seeding is reported. A 3-year-old patient presented with a 1-year history of left-sided headaches, localized over the occipital bone with progressive torticollis. Computed tomography (CT) imaging showed a mass in the left posterior fossa compressing the brainstem. The patient then underwent surgical excision followed by adjuvant chemoradiation therapy. Two years later, the patient presented with severe lower back pain and urinary incontinence. Whole-spine magnetic resonance imaging (MRI) showed cerebrospinal fluid (CSF) seeding from the L5 to the S4 vertebrae. Primary cranial Ewing’s sarcoma is considered in the differential diagnosis of children with extra-axial posterior fossa mass associated with destructive permeative bone lesions. Although primary cranial Ewing’s sarcoma typically has good prognosis, our patient developed metastasis in the lower spine. Therefore, with CNS Ewing’s sarcoma, screening of the entire neural axis should be taken into consideration for early detection of CSF seeding metastasis in order to decrease the associated morbidity and mortality.

Two rare presentations and treatments of Ewing's sarcoma: A need for a standard treatment approach.

2013 ◽  
Vol 31 (15_suppl) ◽  
pp. e21000-e21000
Author(s):  
Kanyalakshmi Ayyanar ◽  
Nitya Narayan ◽  
Karen Moeller ◽  
William Gump ◽  
Thomas Moriarty ◽  
...  
Keyword(s):  
Local Control ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Residual Disease ◽  
Standard Of Care ◽  
Diagnostic Techniques ◽  
Salvage Chemotherapy ◽  
Molecular Diagnostic ◽  
Molecular Diagnostic Techniques ◽  
Mri Scans

e21000 Background: We describe two Caucasian boys with rare presentations of non-metastatic Ewings sarcoma (ES) with atypical primary sites. One was extradural but FISH negative for 22q11.2. Another was intradural but extramedullary, and FISH positive. Literature search showed few publications which describe such cases, and there is currently no standard of care. Methods: MG, an 11 year old male, presented with a frontal skull mass, with an intracranial epidural extension of about 5 mm. Biopsy confirmed ES. MG was treated with Vincristine, Doxorubicin, and Cyclophosphamide, alternating with Ifosfamide and Etoposide every 2 weeks, for 6 weeks. He underwent tumor excision for local control with good response. Chemotherapy continued, completing 14 cycles total. He is in remission 15 months after completion. AH, a 13 year old male, presented with lower back pain and paraesthesias of feet. MRI revealed an intradural, extra-medullary L1-L3 mass. Intra-operatively, all visible tumor peeled off readily, except for the L2 nerve root. The tumor was FISH positive for EWSR1. He was treated with the same regimen as MG for 6 cycles. Local control was done with radiation. He then continued chemotherapy, completing a total of 14 cycles. MRI scans 3 months later revealed enhancing intradural lesions along the spinal cord (involving C5, T5 and T8) and two lesions along the cerebellum. He was started on cyclophosphamide and topotecan and has completed 8 cycles without disease progression. Results: MG was diagnosed based solely on histo-pathological diagnosis. While MG’s lesion was extra-osseus in nature, more unusual presentations demonstrate the importance of molecular diagnostic techniques. AH was diagnosed based on chromosomal analysis. Progression is presumably from microscopic residual disease in intradural space that invaded CSF. He is presently on salvage chemotherapy. Nine months after relapse, disease is stable, with plans to minimize disease and consolidate. Conclusions: Intradural tumors tend to progress rapidly, maybe more so than extradural tumors. Future suggestions include developing a standard approach that will improve both treatment of Ewing’s sarcoma and prognostic outcomes for rare initial presentations.

scholarly journals Delayed Diagnosis of Ewing's Sarcoma of the Right Humerus Initially Treated as Chronic Osteomyelitis: A Case Report

2005 ◽  
Vol 13 (1) ◽  
pp. 88-92 ◽  
Author(s):  
BPB Tow ◽  
MH Tan
Keyword(s):  
Case Report ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Chronic Osteomyelitis ◽  
Delayed Diagnosis ◽  
Delay In Diagnosis ◽  
Limited Stage ◽  
History Of ◽  
Anti Inflammatory ◽  
The Right

We report a case of limited stage Ewing's sarcoma which was initially treated as chronic osteomyelitis for 3 years. A 24-year-old man presented with a one-week history of pain in the right arm and fever, with histology suggestive of osteomyelitis of the affected humerus. He developed multiple relapses of pain and fever; each episode responded to antibiotic treatment. A second biopsy was performed 3 years later and confirmed a diagnosis of Ewing's sarcoma. Despite a 3-year delay in diagnosis, the disease remained localised. This case report highlights an atypical facet of the natural history of Ewing's sarcoma: a response to antibiotic and anti-inflammatory agents, and the limited stage of the disease despite a misdiagnosis of 3 years. This suggests the possibility that anti-inflammatory agents exert an inhibitory effect on the tumour growth. We also highlight the newer histologic and immunologic staining used in the diagnosis of Ewing's sarcoma.

scholarly journals Very Late Local Relapse of Ewing's Sarcoma of the Head and Neck treated with Aggressive Multimodal Therapy

Sarcoma ◽  
2008 ◽  
Vol 2008 ◽  
pp. 1-4 ◽  
Author(s):  
J. Thariat ◽  
A. Italiano ◽  
F. Peyrade ◽  
I. Birtwisle-Peyrottes ◽  
L. Gastaud ◽  
...  
Keyword(s):  
Multimodal Therapy ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
High Dose Chemotherapy ◽  
Local Relapse ◽  
High Dose ◽  
Curative Intent ◽  
First Occurrence ◽  
History Of ◽  
Left Maxillary Sinus

Ewing's sarcoma's relapse rarely occurs more than two years after the initial diagnosis. We report the case of a 26-year-old man with a history of Ewing's sarcoma of the left maxillary sinus at the age of 10 who presented with a very late local relapse, 16 years after the first occurrence of disease. Ultimate control was achieved after multimodal therapy including surgery, high-dose chemotherapy, and radiotherapy. This report indicates that local relapses of Ewing's sarcoma can be treated with curative intent in selected cases.

scholarly journals Primary Ewing’s sarcoma of the petroclival bone: A case report and literature review

2020 ◽  
Vol 11 ◽  
pp. 6
Author(s):  
Derrek Schartz ◽  
Prashanthi Divakar ◽  
Laura Tafe ◽  
Joseph Paydarfar
Keyword(s):  
Skull Base ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Petrous Apex ◽  
Adjuvant Radiation ◽  
Posterior Aspect ◽  
History Of ◽  
Magnetic Resonance Imaging Mri ◽  
Stage Iii Breast Cancer ◽  
Mri Surveillance

Background: Primary Ewing’s sarcoma (ES) is typically seen within the long bones, vertebrae, or pelvis. Uncommonly, it can be found within the cranium among the rarest locations for primary ES are the skull base, in particular, the petroclival bone. Case Description: The patient is a 68-year-old female with past medical history of Stage III breast cancer who presented with severe retro-orbital headache and diplopia due to a cranial nerve VI palsy. Magnetic resonance imaging (MRI) revealed a mass at the left petroclival bone with extension into the adjacent left petrous apex and into the posterior aspect of the left cavernous sinus proximal to the carotid artery. The patient subsequently underwent an endoscopic transsphenoidal biopsy. Pathological and molecular analysis supported a diagnosis of ES. The patient then underwent neoadjuvant chemotherapy and radiotherapy. At 12 month-follow-up, her petroclival ES demonstrated significant interval decrease in size on MRI surveillance imaging. Conclusions: This is the third case of primary ES of the petroclival bone to be reported in the literature. In this patient, management consisted of surgical incisional biopsy followed by adjuvant radiation and chemotherapy. Knowledge and awareness of this type of tumor is important for the skull base surgeon.

scholarly journals Ewing's Sarcoma: Development of RNA Interference-Based Therapy for Advanced Disease

ISRN Oncology ◽  
2012 ◽  
Vol 2012 ◽  
pp. 1-13 ◽  
Author(s):  
Olivia Simmons ◽  
Phillip B. Maples ◽  
Neil Senzer ◽  
John Nemunaitis
Keyword(s):  
Rna Interference ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Advanced Disease ◽  
Chromosomal Translocation ◽  
Transcription Factor Gene ◽  
Factor Gene ◽  
Systemic Rnai ◽  
Ets Transcription Factor ◽  
Target Sequences

Ewing's sarcoma tumors are associated with chromosomal translocation between the EWS gene and the ETS transcription factor gene. These unique target sequences provide opportunity for RNA interference(i)-based therapy. A summary of RNAi mechanism and therapeutically designed products including siRNA, shRNA and bi-shRNA are described. Comparison is made between each of these approaches. Systemic RNAi-based therapy, however, requires protected delivery to the Ewing's sarcoma tumor site for activity. Delivery systems which have been most effective in preclinical and clinical testing are reviewed, followed by preclinical assessment of various silencing strategies with demonstration of effectiveness to EWS/FLI-1 target sequences. It is concluded that RNAi-based therapeutics may have testable and achievable activity in management of Ewing's sarcoma.

scholarly journals Cell Cycle Deregulation in Ewing's Sarcoma Pathogenesis

Sarcoma ◽  
2011 ◽  
Vol 2011 ◽  
pp. 1-10 ◽  
Author(s):  
Ashley A. Kowalewski ◽  
R. Lor Randall ◽  
Stephen L. Lessnick
Keyword(s):  
Cell Cycle ◽  
Transcription Factor ◽  
Target Genes ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Cell Cycle Control ◽  
Chromosomal Translocation ◽  
Published Data ◽  
Pediatric Tumor ◽  
Cell Cycle Deregulation

Ewing's sarcoma is a highly aggressive pediatric tumor of bone that usually contains the characteristic chromosomal translocation t(11;22)(q24;q12). This translocation encodes the oncogenic fusion protein EWS/FLI, which acts as an aberrant transcription factor to deregulate target genes necessary for oncogenesis. One key feature of oncogenic transformation is dysregulation of cell cycle control. It is therefore likely that EWS/FLI and other cooperating mutations in Ewing's sarcoma modulate the cell cycle to facilitate tumorigenesis. This paper will summarize current published data associated with deregulation of the cell cycle in Ewing's sarcoma and highlight important questions that remain to be answered.

scholarly journals Pediatrics Ewing’s Sarcoma of the Sinonasal Tract: A Case Report and Literature Review

2019 ◽  
Vol 2019 ◽  
pp. 1-3
Author(s):  
Ahmed Aldandan ◽  
Ali ‎ Almomen ◽  
Abdulrahman Alkhatib ◽  
Ghaleb Alazzeh
Keyword(s):  
Case Report ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Neck Region ◽  
Endoscopic Biopsy ◽  
Cell Tumor ◽  
Sinonasal Tract ◽  
Histopathological Analysis ◽  
History Of ◽  
Blue Cell

Ewing’s sarcoma (ES) is a highly malignant, small, round cell tumor that originates from the primitive neuroectodermal cells. Primary ES commonly occurs in early childhood or adolescence. It may present with skeletal and extraskeletal forms. The extraskeletal form is rarely encountered in the head and neck region and is extremely rare in the sinonasal tract. This is a case report of sinonasal ES in a 13-year-old female patient who presented with a 7-month history of right nasal obstruction, anosmia, intermittent epistaxis, snoring, and hearing loss. Clinical examination revealed a right nasal mass pushing the septum to the left side and extending to the nasopharynx. Endoscopic biopsy and histopathological analysis showed a small blue cell tumor suggestive of ES. The patient was treated with surgery, radiotherapy, and chemotherapy. After a follow-up of 5 years, the patient remains recurrence-free with excellent functional status and quality of life.

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