Ewings Sarcoma of the Kidney: A Rare Entity

Choubey, Surya Kant; Pipara, Gotam; Kumar, Anurag

doi:10.14740/wjnu304w

Cited by 3 publications

(5 citation statements)

References 13 publications

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“…Primary ESFT of the kidney is rarely reported. 33 - 41 However, in the present series, involvement of kidney was seen in 7 (median age of presentation: 30 years) patients. Further ESFTs in bladder and prostate noted in this cohort were rarely reported.…”

Section: Discussioncontrasting

confidence: 62%

Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade’s Experience From a Single Centre in India

Murthy

Challa

Raju

et al. 2020

Clin Med�Insights�Pathol

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Introduction: The diagnosis of Ewing sarcoma family of tumours (ESFT) is challenging, especially in adults and in extra-skeletal or visceral location. Several morphologic mimics with varied treatment options and prognosis confer diagnostic dilemmas. Application of ancillary diagnostic modalities in surgical pathology in clinical routine has enabled accurate diagnosis of ESFT in bone, soft tissues, and viscera. Aim: The study aims to assess the clinicopathological features including molecular test results of ESFT with emphasis on sex, age, and location, especially extra-skeletal soft tissue and visceral location. Material and Methods: Data of clinicopathological, molecular tests (wherever performed), diagnosis rendered in 302 ESFT over a decade from our centre were reviewed. Statistical comparison of skeletal and extra-skeletal tumours with reference to age and sex was done using SPSS package. The P value of <.05 was considered significant. Results: The cohort included 302 ESFTs with 49% skeletal and 51% extra-skeletal tumours. Thigh was most common site among skeletal tumours; chest wall, paraspinal location, and retroperitoneum among soft tissues (39.4%); and kidney, ovary, and cervix among visceral tumours (11.3%). Fluorescence in situ hybridisation for EWSR1 gene rearrangement was positive in 54 patients and reverse-transcriptase polymerase chain reaction in 19 patients. Predominance of male sex, younger age and location in extremities among skeletal tumours and lack of gender predilection, higher age and axial location in extra-skeletal tumours were noted, which were statistically significant. Molecular tests were performed more frequently in extra-skeletal tumours, especially in visceral tumours to establish the diagnosis. Conclusions: The study showed statistically significant differences in the age, sex, and location between skeletal and extra-skeletal ESFT. The increased percentage of extra-skeletal tumours especially in viscera was attributed to the increased awareness and availability of ancillary techniques.

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Section: Discussioncontrasting

confidence: 62%

Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade’s Experience From a Single Centre in India

Murthy

Challa

Raju

et al. 2020

Clin Med�Insights�Pathol

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“…The clinical presentations of these patients include flank/abdominal pain, palpable mass, and hematuria [6]. Usually, they remain asymptomatic for a long time, until the mass reaches a palpable size, as seen in our case.…”

Section: Discussionmentioning

confidence: 59%

“…Primary ES of the kidney typically manifests in children and adolescents with a strong male predilection [ 4 ]. The clinical presentations of these patients include flank/abdominal pain, palpable mass, and hematuria [ 6 ]. Usually, they remain asymptomatic for a long time, until the mass reaches a palpable size, as seen in our case.…”

Section: Discussionmentioning

confidence: 99%

Primary Adult Renal Ewing’s Sarcoma: A Rare Entity

Shangpliang¹,

Rangad²,

Das³

et al. 2022

Cureus

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Ewing's sarcoma/primitive neuroectodermal tumors are high-grade small round blue cell tumors traditionally found in children and adolescents. These tumors primarily affect the bone and soft tissue, with extraskeletal sites rarely being affected. The clinical presentation and imaging findings are non-specific and are not characteristic. The diagnosis is essentially based on the histopathologic findings assisted by immunohistochemistry and/or cytogenetic molecular studies. Proper diagnoses and timely management of this tumor are essential owing to the aggressive nature and poor prognosis of the disease.

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“…Cut surface of the kidney showed pale, white, fleshy, infiltrating mass noted in the middle and lower pole measuring 7 Â 5.5 Â 5cm, and cystic, hemorrhagic, and necrotic areas were noted (►Fig. 1).…”

Section: Case Reportmentioning

confidence: 99%

“…Ewing sarcoma is highly malignant tumor of bone mostly seen in children and young adults. 1 Nearly 6% of cases are extraosseous. Trunk, extremities, head and neck, and retroperitoneum are the most common extraosseous site.…”

Section: Introductionmentioning

confidence: 99%

Ewing Sarcoma of Kidney: A Rare Entity

Aithal A,

K.,

Mathias

2024

Journal of Health and Allied Sciences NU

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Primary renal origin of Ewing sarcoma/PNET (primitive neuroectodermal tumor) is a rare entity in the adult population and has an aggressive outcome. The entity was first coined by Arthur Purdy stout in 1918 and recognized under family of small round cell tumor. Radiologically it is difficult to distinguish from primary clear cell carcinoma of kidney from Ewing/PNET. Diagnosis of this requires histopathology, immunohistochemistry (IHC), and cytogenetics studies.A 40-year-old female presented with hematuria and radiology found a mass lower pole of left kidney with extension into renal hilum. Nephrectomy was done for the case and the histopathology diagnosis of small round cell tumor of left kidney was given. Followed by IHC, diagnosis of malignant round cell tumor was suggestive of Ewing sarcoma.Microscopy showed cells with small, round, hyperchromatic nuclei, scant cytoplasm, and inconspicuous nucleoli. Increased mitosis was noted (15/10 high power field). Multiple foci of pseudorosettes, areas of hemorrhage, and necrosis lymphovascular emboli were seen.IHC done outside showed NKX 2-2, synaptophysin positivity, CD99 diffuse membranous positivity, cytokeratin perinuclear dot like positivity, and negative for CD20, CD3, desmin, CD34, S100, and Pax8. Impression of malignant round cell tumor was suggestive of Ewing sarcoma.Ewing sarcoma is one of the rare yet highly aggressive tumors. This should be kept as differential diagnosis in young adults with renal mass, as on radiologically it cannot be differentiated from renal cell carcinoma. Diagnosis of Ewing sarcoma is done using histopathology, IHC, and cytogenetic study. Early diagnosis helps in initiation of surgery, chemotherapy, and radiotherapy and helps in increasing the survival rate.

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Ewings Sarcoma of the Kidney: A Rare Entity

Cited by 3 publications

References 13 publications

Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade’s Experience From a Single Centre in India

Ewing Sarcoma With Emphasis on Extra-skeletal Ewing Sarcoma: A Decade’s Experience From a Single Centre in India

Primary Adult Renal Ewing’s Sarcoma: A Rare Entity

Ewing Sarcoma of Kidney: A Rare Entity

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