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.

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 the sinonasal tract with orbital extension: a rare case report and literature review

2019 ◽  
Vol 2019 (3) ◽  
Author(s):  
Omair Al Hussain ◽  
Ahmed Aldandan ◽  
Abdulrahman Alkhatib ◽  
Ghaleb Alazzeh ◽  
Ali Almomen
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Rare Case ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Sinonasal Tract ◽  
Rare Case Report

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 A rare case of Ewing’s sarcoma in the sinonasal tract

2017 ◽  
Vol 4 (1) ◽  
pp. 304 ◽  
Author(s):  
Deepthi Satish ◽  
Nandakumar R. ◽  
Balasubramanya A. M ◽  
Nitty Mathew
Keyword(s):  
Head And Neck ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Histopathological Examination ◽  
Neck Region ◽  
Malignant Tumour ◽  
Sinonasal Tract ◽  
Ewings Sarcoma ◽  
Primary Neoplasm ◽  
Immunohistochemical Studies

<p>Ewing's sarcoma (ES) first described by James Ewing in 1921, is a primary neoplasm of the skeletal system. Extraskeletal Ewing’s sarcoma (EES) is a rare, rapidly growing, round cell malignant tumour that can develop in the soft tissue at any location. Extraskeletal Ewings sarcoma of the head and neck is uncommon. Nearly 80% of patients are younger than 20 years with peak incidence in the second decade. Ewings sarcoma rarely affects the sinonasal tract. Diagnosis is after histopathological examination immunohistochemical studies and cytogenetic studies. Treatment will include a multidisciplinary approach with surgery as the first line followed by chemotherapy and radiotherapy. Ewings sarcoma in the head and neck region do not metastazise early hence carries a better prognosis. Evaluation of lesion using imaging, biopsy followed by histopathology and immunohistochemistry and cytogenetic analysis are necessary for early diagnosis and treatment. We present a case of an 18-year-old male patient with left sided epistaxis and left sided nasal obstruction, on examination a mass was seen the nasal cavity, endoscopic excision and biopsy of the mass was suggestive of an Ewings sarcoma. He received post-operative chemotherapy and radiotherapy. 2 years on regular follow up patient is fine without signs of metastasis or recurrence. We are presenting this case due to the rarity of its presentation in the sinonasal tract.</p>

scholarly journals Primary Ewing's sarcoma of the sinonasal tract, eroding the ethmoid and sphenoid sinus with intracranial extension: A rare case report

2015 ◽  
Vol 3 (4) ◽  
pp. 807-810 ◽  
Author(s):  
MARIA EMANUELA NEGRU ◽  
ANDREA PIETRO SPONGHINI ◽  
DAVID RONDONOTTI ◽  
FRANCESCA PLATINI ◽  
MARCO GIAVARRA ◽  
...  
Keyword(s):  
Case Report ◽  
Sphenoid Sinus ◽  
Rare Case ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Sinonasal Tract ◽  
Intracranial Extension ◽  
Rare Case Report

scholarly journals EUS-FNA for the Diagnosis of Retroperitoneal Primitive Neuroectodermal Tumor

2011 ◽  
Vol 2011 ◽  
pp. 1-3 ◽  
Author(s):  
Aijaz A. Sofi ◽  
Ashish D. Thekdi ◽  
Ali Nawras
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Percutaneous Biopsy ◽  
Needle Aspiration ◽  
Neuroectodermal Tumor ◽  
Novel Approach ◽  
History Of ◽  
Blue Cell ◽  
Round Blue Cell Tumor

Primitive neuroectodermal tumor (PNET) is a rare “small round blue cell tumor” that is diagnosed by open biopsy or percutaneous biopsy of the lesion under radiologic guidance. In this case report, we present a novel approach to the diagnosis of a retroperitoneal PNET by endoscopic ultrasound- (EUS-) guided fine needle aspiration (FNA). A 35-year-old man presented with the history of left-sided flank pain and swelling of 3-weeks duration. Computerized tomography (CT) scan of his abdomen revealed a 12.8 × 13 × 12.5 cm cystic and solid mass arising from the retroperitoneum and displacing the third and fourth portions of the duodenum. He underwent EUS which revealed a well-circumscribed heterogeneous mass abutting the inferior portion of the stomach. EUS-FNA of the mass revealed malignant cells consistent with primitive neuroectodermal tumor (PNET)/Ewing's sarcoma. EUS-guided FNA is an appropriate technique for diagnosing retroperitoneal PNET/Ewing's sarcoma.

Potential therapeutic genomic alterations in desmoplastic small round blue cell tumor.

2017 ◽  
Vol 35 (15_suppl) ◽  
pp. 11066-11066 ◽  
Author(s):  
Joanne Xiu ◽  
Ajaz Bulbul ◽  
Sadaf Rashad
Keyword(s):  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Statistical Significance ◽  
Low Frequency ◽  
Cell Tumor ◽  
Mutational Load ◽  
Missense Mutations ◽  
Significant Difference ◽  
Blue Cell ◽  
Round Blue Cell Tumor

11066 Background: Desmoplastic Small Round Blue Cell Tumor (DSRCT) originates from a cell with multilineage potential. A molecular hallmark of DSCRT is the EWS-WT1 reciprocal translocation. Ewing’s and DSRCT are treated similarly due to similar oncogene activation pathways and DSRCT has been represented in very limited numbers in sarcoma studies. Methods: Thirty-five DSRCT tumors were tested with a multiplatform profiling service (Caris Life Sciences, Phoenix, AZ). Specific tests performed included sequencing (NextGen), protein expression (IHC) and gene amplification (CISH or FISH). Tumor mutational load (TML) was calculated as somatic nonsynonymous missense mutations sequenced with a 592-gene panel. Molecular alterations were compared to 88 Ewing sarcomas (ES). Chi-square tests were used for comparison and statistical significance was determined as p < 0.05. Results: In the 35 DSRCT tumors, high expression of TOP2A were seen in 63%, TOPO1 in 63%, PTEN in 62%, androgen receptor (AR) in 59%, EGFR in 42% of tumors; low expression of TUBB3 was seen in 44%, MGMT in 45%, TS in 48%, RRM1 in 57% and ERCC1 in 76% of tumors. When compared to ES, no significant difference was seen in protein expressions with the exception of a significantly higher overexpressson of AR in DSRCT (59% vs. 3%, p = 1.7E-10) and TUBB3 (56% vs. 29%, p = 0.03). Tumor expression of PD-L1 (Ab: SP142) was not seen in the 4 DSRCT and 10 ES tested. NextGen revealed a TP53 mutation (7%) and a FOXO3 mutation (L382fs) in DSCRT, while 6 TP53 mutations (13%), 2 APC mutations (L1129S and I1307K), 1 BRCA1(c.301+1G > A) and 1 CTNNB1 (T41A) mutation were identified in ES. Tumor mutational load evaluated in the 3 DSRCT and 11 ES tumors averaged 6 and 5 mutations per megabase, respectively. Conclusions: Molecular profiling on 35 DSRCT tumors and comparison with Ewing’s sarcoma revealed low immunogenicity ( < 10 Mutations/MB) and low frequency of actionable mutations including PD-L1 in both tumor types. High AR expression could present as a potential therapeutic target for DSRCT while taxanes may be more effective in Ewing’s sarcoma compared to DSCRT based on TUBB3 expression. Genomic and Molecular assessment may help determine the ideal regimen that will help achieve maximal tumor debulking.

Extraskeletal Ewing’s sarcoma presenting as a neck mass - case report

ORL ro ◽  
2017 ◽  
Vol 2 (35) ◽  
pp. 24
Author(s):  
Daniel Mirea ◽  
Loredana Mitran ◽  
Daniela Safta ◽  
Cornelia Niţipir ◽  
Bogdan Mocanu ◽  
...  
Keyword(s):  
Case Report ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Neck Mass ◽  
Extraskeletal Ewing’S Sarcoma ◽  
Mass Case

scholarly journals A child with Ewing's sarcoma in scapula: A rare case report

2021 ◽  
pp. 106182
Author(s):  
Mohammad Nour Shashaa ◽  
Amr Hamza ◽  
Mohamad AlHashemi ◽  
Rama Alyousfi ◽  
Muhammad Abu Alkheer Deebo ◽  
...  
Keyword(s):  
Case Report ◽  
Rare Case ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Rare Case Report
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