scholarly journals A Rare Presentation of Ewing Sarcoma as Supraclavicular Swelling in an Adolescent Male – A Case Report

2020 ◽  
Vol 7 (11) ◽  
pp. C155-159
Author(s):  
Arun Gopal ◽  
Shivali Awasthi ◽  
Vikas Dagar ◽  
Prateek Kinra
Keyword(s):  
Ewing Sarcoma ◽  
Age Groups ◽  
Cell Tumor ◽  
Adolescent Male ◽  
Primary Malignancy ◽  
Rare Presentation ◽  
Blue Cell ◽  
Trucut Biopsy ◽  
Almost All ◽  
Round Blue Cell Tumor

            Supraclavicular swellings can have numerous differential diagnosis ranging from infections to malignancy, in almost all age groups. Here we present a case of 15 year old boy who presented with left supraclavicular swelling and clinically diagnosed as a case of Tuberculosis, underwent FNAC which was suggestive of Small Round Blue Cell Tumor favouring Ewing sarcoma. Later, trucut biopsy of the swelling confirmed the diagnosis of Ewing sarcoma. Ewing sarcoma is one of the most common primary malignancy of bone in children but can also arise from extraskeletal region like soft tissue.

scholarly journals Highly aggressive undifferentiated small round blue cell tumor of foot with unique SMARCA1, KAT6A and NAV3 mutations

2019 ◽  
Vol 2019 (9) ◽  
Author(s):  
Cyrus Parsa ◽  
Robert Orlando ◽  
Michael Marcus ◽  
Jin Guo ◽  
Ravin Rupani
Keyword(s):  
Cancer Progression ◽  
Cell Tumor ◽  
Chromosome 22 ◽  
High Grade ◽  
Mutational Pattern ◽  
Blue Cell ◽  
Ets Family ◽  
Round Blue Cell Tumor ◽  
Ewing Family Of Tumors ◽  
Different Tissues

ABSTRACT Malignancies characterized histologically by high-grade monotonous small round blue cells (SRBCs) belong to a heterogeneous group of neoplasms often referred to as Ewing family of tumors. The most common molecular confirmation of these neoplasms is by fusions between EWSR1 gene on chromosome 22 and the ETS family of transcription factors, including FLI1 gene (11q24) and the ERG (21q22), that are implicated in the development of different tissues as well as cancer progression. In this article, we present a case of highly aggressive extraskeletal SRBC tumor involving the foot of a 24-year-old male with sole molecular findings of mutations in KAT6A, NAV3 and SMARCA1 genes with high expression of soft tissue markers (COL1A1, COL1A2, COL3A1) and MYC mRNA. To our knowledge, this unique mutational pattern has not previously been described in SRBCs.

Small cell carcinoma of the submandibular gland: a rare small round blue cell tumor

2007 ◽  
Vol 28 (2) ◽  
pp. 118-121 ◽  
Author(s):  
David M. Walters ◽  
Stewart C. Little ◽  
Richard B. Hessler ◽  
Christine G. Gourin
Keyword(s):  
Cell Carcinoma ◽  
Submandibular Gland ◽  
Small Cell Carcinoma ◽  
Small Cell ◽  
Cell Tumor ◽  
Blue Cell ◽  
Round Blue Cell Tumor

Expression of C-kit in Ewing Family of Tumors: A Comparison of Different Immunohistochemical Protocols

2004 ◽  
Vol 7 (4) ◽  
pp. 342-347 ◽  
Author(s):  
Atif Ahmed ◽  
Enid Gilbert-Barness ◽  
Atilano Lacson
Keyword(s):  
Monoclonal Antibodies ◽  
Ewing Sarcoma ◽  
Polyclonal Antibodies ◽  
Growth Factor Receptor ◽  
Myelogenous Leukemia ◽  
Antigen Retrieval ◽  
Tyrosine Kinase Receptor ◽  
Ki 67 ◽  
Blue Cell ◽  
Round Blue Cell Tumor

Ewing sarcoma is a small round blue cell tumor with a high incidence of metastasis and poor survival. The tyrosine kinase receptor, c-kit, is a growth factor receptor that is expressed in a variety of tumors including Ewing sarcoma. Blockade of c-kit by imatinib mesylate (Gleevec; Novartis Pharmaceuticals Corp, East Hanover, NJ) has been successfully used in the treatment of chronic myelogenous leukemia and gastrointestinal tumors. Detection of c-kit expression in Ewing sarcoma indicates a possible role of c-kit in tumor progression and a potential use of anti-c-kit therapy in Ewing sarcoma. Ki-67 is a proliferation marker found at all stages of the cell cycle. Expression of c-kit and Ki-67 was studied in 17 patients with Ewing sarcoma. Sections from paraffin-embedded tumor samples were immunostained, using standard immunohistochemical protocols, with c-kit and Ki-67 monoclonal antibodies, polyclonal c-kit antibody without antigen retrieval, and c-kit polyclonal antibody with antigen retrieval. Eleven out of 17 cases (65%) stained with c-kit monoclonal antibody; the staining was diffuse in 6/17 (35%) cases. C-kit expression did not correlate with Ki-67 proliferation rates. Using the polyclonal c-kit-antibody without antigen retrieval methods, c-kit expression was demonstrated in 1/11 (9%) cases. Incorporating antigen retrieval methods, c-kit expression increased to 53%. Concordance between monoclonal antibodies in detecting c-kit expression was observed in 12/17 cases (71%). We conclude that c-kit is variably expressed in Ewing sarcoma, using either monoclonal or polyclonal antibodies. Detection of c-kit expression in Ewing sarcoma improves with the use of antigen retrieval methods.

scholarly journals Title-An Extrapolation to Small Round Blue Cell Tumor Diagnosed as Esthesioneuroblastoma –Acase Series

2016 ◽  
Vol 15 (08) ◽  
pp. 43-47
Author(s):  
Dr. Arnav Sahu ◽  
Dr.Anu Singh ◽  
Dr Abhishek Verma ◽  
Dr Trilochan Singh ◽  
Dr. R.K. Srivastava ◽  
...  
Keyword(s):  
Cell Tumor ◽  
Blue Cell ◽  
Round Blue Cell Tumor

scholarly journals Ectopic ACTH Syndrome and Hypothyroidism Due to Right Renal Small Round Blue Cell Tumor: A Case Report

2020 ◽  
Author(s):  
Weipu Mao ◽  
Jiajia Xu ◽  
Haowen Lu ◽  
Yali Wang ◽  
Lihua Zhang ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Thyroid Hormone ◽  
Soft Tissues ◽  
Cell Tumor ◽  
Lumbar Pain ◽  
Ectopic Acth Syndrome ◽  
Histopathological Analysis ◽  
Ectopic Acth ◽  
Blue Cell ◽  
Round Blue Cell Tumor

Abstract Background: Small round blue cell malignancies are rare and highly aggressive tumors that are commonly located in the soft tissues or axial bones of the bone or trunk and are particularly rare in the kidney. The common cause of ectopic ACTH syndrome is pulmonary neuroendocrine tumors, such as small cell carcinomas and carcinoid tumors. Here, we present an unusual case of ectopic ACTH syndrome and hypothyroidism caused by small round blue cell tumor of the right kidney.Case presentation: A 19-year-old girl presented with a history of right lumbar pain and discomfort for 2 months, aggravated for 2 days. Abdominal contrast-enhanced computed tomography and computed tomography angiography showed right suprarenal pole occupancy with subepithelial hemorrhage. Preoperative hormone levels including plasma total cortisol (PTC), adrenocorticotrophic hormone (ACTH) and thyroid hormone measurements were abnormal, indicating that the patient had Cushing syndrome and hypothyroidism. The patient underwent right radical nephrectomy. Histopathological analysis revealed a renal small round blue cell tumor (consistent with a primitive neuroectodermal tumor), with positive immunohistochemistry for CD99 and Ki67 (about 10%) and molecular pathology for EWSR1 gene fusions. PTC, ACTH and thyroid hormone returned to normal after surgery.Conclusions: We report a rare ectopic ACTH syndrome and hypothyroidism due to renal small round blue cell tumor. The clinical manifestation of renal small round blue cell tumor is non-specific and the diagnosis relies on pathological morphology, immunohistochemistry and fusion gene detection. At present, surgery combined with radiotherapy and chemotherapy is used in the treatment, but the prognosis is still not optimistic.

scholarly journals Primary primitive neuroectodermal tumor of the adrenal gland: A unique tumor at an unusual site

2021 ◽  
Vol 8 (3) ◽  
pp. 429-432
Author(s):  
Rakhi V Jagdale ◽  
Jaydeep N Pol
Keyword(s):  
Primitive Neuroectodermal Tumor ◽  
English Literature ◽  
Young Female ◽  
Cell Tumor ◽  
Neuroectodermal Tumor ◽  
Unusual Site ◽  
Non Hodgkin Lymphoma ◽  
Blue Cell ◽  
Neuroectodermal Origin ◽  
Round Blue Cell Tumor

Ewing sarcoma/Primitive Neuroectodermal Tumor (ES/PNET) is a malignant small round blue cell tumor of neuroectodermal origin that affects bones and soft tissue in children and young adults. ES/PNET is very uncommon in parenchymal organs. We report a case of primary adrenal PNET in a young female having pregnancy induced persistent hypertension. She underwent right adrenalectomy for a large adrenal mass. On histopathology, it was typical malignant round cell tumor (MRCT). Immunohistochemistry confirmed the diagnosis of ES/PNET with expression of CD99, FLI-1 and NKX2.2. Until now, only 38 cases of primary adrenal ES/PNET have been reported in the English literature with just 5 cases from India. Adrenal ES/PNET can be mistaken with other MRCTs like Non-Hodgkin lymphoma and neuroblastoma. Since the biology, treatment and prognosis of these tumors is entirely different, we emphasize that pathologists must be aware of this possibility and try to differentiate them using ancillary techniques.

scholarly journals Desmoplastic Infantile Astrocytoma and Desmoplastic Infantile Ganglioglioma – not so infantile anymore

2020 ◽  
Vol 6 (7) ◽  
pp. 440-448
Author(s):  
Dr. Jyotsna Sahai ◽  
◽  
Dr. Shilpi Sahu ◽  
Keyword(s):  
Spindle Cell ◽  
Age Groups ◽  
Good Prognosis ◽  
Differentiated Cells ◽  
Desmoplastic Infantile Astrocytoma ◽  
Meningeal Involvement ◽  
Blue Cell ◽  
Dural Attachment ◽  
Poorly Differentiated ◽  
Round Blue Cell Tumor

DIA and DIG are rare, infantile, supratentorial neoplasms that usually occur in children before 2years of age and are exceedingly rare in older age groups. They appear as hypodense, cystic masseswith solid components showing dural attachment on neuroimaging. They are characterized byreticulin-rich spindle cell stroma containing connective tissue due to meningeal involvement,microscopically. These tumors have potential for misdiagnosis because they contain varyingproportions of neoplastic glial, neuronal and poorly differentiated cells, which causes them to have a“small round blue cell tumor” like appearance, though they have a good prognosis if correctlydiagnosed. The current study report two cases diagnosed at our institution that had very latepresentation with varying complaints which challenged the normally believed dictum of these tumorsbeing entirely infantile.

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