Authors : Anitha Padmanabhan, Anitha Padmanabhan, Rucha K Kanhe, Rucha K Kanhe, Nitin M Gadgil, Nitin M Gadgil
DOI : 10.18231/j.jdpo.2020.022
Volume : 5
Issue : 1
Year : 2020
Page No : 108-111
Introduction: Primitive neuroectodermal tumor (PNET) / Ewing sarcoma of kidney are rare aggressive
tumor with poor prognosis. It occurs in young adults generally in 2nd to 3rd decade of life
Case Reports: We report three cases of PNET/Ewing sarcoma of kidney in patients of age 25and 35 years
with complain of hematuria and abdominal pain .CT scan revealed heterogeneously enhancing lesion in
kidney and we received radical nephrectomy specimen with variegated appearance on gross. Microscopic
examination showed tumor with malignant cells in sheets and pseudorosettes .The diagnosis was given as
PNET/ES and was confirmed with immunohistochemistry.
Discussion: Renal PNET/ES is a member of Ewing sarcoma family. They are rare tumor originating
from the neural crest and are more aggressive than PNET at any other site. The presence of Homer
Wright rosettes is less common in extra-osseous Ewing sarcoma and their presence is a sure diagnosis
of PNET/ES. The differential diagnosis for these small round cell tumor of kidney other than PNET
include malignant lymphoma, renal neuroblastoma,,embryonalrhanbdomyosarcoma, wilms tumor. The
immunohistochemical marker CD99 positivity is diagnostic. Other markers positive are S-100, vimentin
and NSE (neuron specific enolase).
Conclusion: Histopathology and immunohistochemistry remains gold standard for confirming the
diagnosis.
Keywords: Ewing sarcoma (ES) / kidney/, Primitive neuroectodermal tumor, (PNET).