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• IHC with CD99 over expression and cytogenetic analysis like translocations involving the EWS locus EWSR1 gene rearrangement are required for definitive diagnosis of Primary Ewing sarcoma of Kidney which is very rare entity
  professor Pranab Kumar Bhattacharya
  Published on: 28 January 2019

• Published on: 28 January 2019

  IHC with CD99 over expression and cytogenetic analysis like translocations involving the EWS locus EWSR1 gene rearrangement are required for definitive diagnosis of Primary Ewing sarcoma of Kidney which is very rare entity

  • professor Pranab Kumar Bhattacharya, Professor of Pathology calcutta school of Tropical Medicine 108 CR Avenue kolkata 73 West Bengal India

  Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumours typically found in children and adolescents. These tumours belong to the family of small round cell tumours and are of neuro ectodermal origin Primary Ewing sarcoma (EWS) of the kidney is a rare tumor in adults. It was first described in 1975 by Seemayer and colleagues, and has since been sporadically documented in the literature Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, although both share common stem-cell precursor and unique chromosomal abnormality. Because of their similar histologic and cytogenetic characteristics, these tumors are now considered part of a spectrum of neoplastic diseases now known as Ewing’s sarcoma family tumors (ESFT), which also includes other malignancies The ESFT are most common in bone. Extraskeletal ESFT are less common and can affect the skin, soft tissue, or viscera So Renal primary sarcomas are a rare group of renal tumours. Ewing sarcoma/PNET of the kidney is distinctly rare, with more than 100 cases reported globally. Among these, leiomyo sarcoma is the most common (40–60%) followed by lipo sarcoma (10–15%) Sources of renal EWS include neural cells that invaginate into the kidney during its development some authors theorize that embryonic neural crest cells migrate into the kidney and undergo tumorigenesis It is primarily a genetic disease with case...

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  Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) are high-grade malignant tumours typically found in children and adolescents. These tumours belong to the family of small round cell tumours and are of neuro ectodermal origin Primary Ewing sarcoma (EWS) of the kidney is a rare tumor in adults. It was first described in 1975 by Seemayer and colleagues, and has since been sporadically documented in the literature Ewing sarcoma and primitive peripheral neuroectodermal tumor (PNET) were originally described as two distinct pathologic entities, although both share common stem-cell precursor and unique chromosomal abnormality. Because of their similar histologic and cytogenetic characteristics, these tumors are now considered part of a spectrum of neoplastic diseases now known as Ewing’s sarcoma family tumors (ESFT), which also includes other malignancies The ESFT are most common in bone. Extraskeletal ESFT are less common and can affect the skin, soft tissue, or viscera So Renal primary sarcomas are a rare group of renal tumours. Ewing sarcoma/PNET of the kidney is distinctly rare, with more than 100 cases reported globally. Among these, leiomyo sarcoma is the most common (40–60%) followed by lipo sarcoma (10–15%) Sources of renal EWS include neural cells that invaginate into the kidney during its development some authors theorize that embryonic neural crest cells migrate into the kidney and undergo tumorigenesis It is primarily a genetic disease with cases affected sporadically, mainly a translocation type of mutation causing a fusion between the EWS gene on chromosome 22 and FLI1 on chromosome
  Mean age at diagnosis of 30.4 years with the majority being male. Presentation of such tumors reported in the literature is non-specific and frequently involves localised pain which usually involves flank, subcostal or abdominal area with or without hematuria Differential diagnosis include Renal cell carcinoma and Wilm’s tumor ; neuroblastoma; malignant lymphoma ;metastatic renal involvement from sarcoma elsewhere in the body
  No specific signs of PNET have been described in ultra sonography, computed tomography (CT), or magnetic resonance imaging (MRI). The imaging characteristics of most renal sarcomas are indistinguishable from those of RCC . These tumors appears as ill-defined, large heterogeneous masses with necrotic and hemorrhagic areas. Using ultrasonography, the tumour appears isoechogenic or hyperechogenic to the renal parenchyma. The CT findings include areas of internal haemorrhage or necrosis, peripheral hyper vascularity, and diffuse calcification. On MRI, the tumour appeared iso intense or hypo intense on T1-weighted images but heterogeneous intermediate to high T2 signal intensity . Venous extension into the renal vein, inferior vena cava, and right heart has been described .
  Biopsy of the kidney mass show that Ewing sarcoma/primitive neuroectodermal tumor The pathologic findings shows usually multiple areas of necrosis and haemorrhage. In some cases, the tumour may exhibits calcifications and can be surrounded by a fibrous capsule. Microscopic features associated with ESFT/PNET are considered however non-specific as they can be seen in many other tumors, hence, the use of immunochemistry with CD99 over expression and cytogenetic analysis for definitive diagnosis Diagnosis is to be confirmed by fluorescence in situ hybridization (FISH) technique, which showed translocations involving the EWS locus (EWSR1 gene rearrangement).

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  Conflict of Interest:
  None declared.

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