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 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 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.

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.

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 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

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 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 Ewing’s Sarcoma of Kidney- a Rare Case

2016 ◽  
Vol 14 (1) ◽  
pp. 46-48
Author(s):  
Bikash Bukram Thapa ◽  
Narayan Thapa ◽  
Bharat Bahadur Bhandari ◽  
Bhairab Kumar Hamal
Keyword(s):  
Young Adult ◽  
Rare Case ◽  
Ewing’S Sarcoma ◽  
Ewing's Sarcoma ◽  
Clinical Presentations ◽  
Aggressive Tumor ◽  
Multimodality Approach

Ewing’s sarcoma of kidney is rare and aggressive tumor of young adult with diagnostic challenges. Clinical presentations and imaging are non specific. The diagnosis depends on histopathology and immunohistochemistry and  treatment encompass multimodality approach. We present here a case of Ewing’s sarcoma kidney of a young adult managed in our institution.

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.

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