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Primary renal primitive neuroectodermal tumour causing Budd-Chiari syndrome: a rare case report
Biswajit Mishra1, S H Chandrashekhara1, Smita Manchanda1
1Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi, India.
BJR Case Reports
|November 22, 2018
Summary
A rare kidney cancer, Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET), can aggressively present as Budd-Chiari syndrome. This case highlights an unusual manifestation of this rare renal neoplasm.
Area of Science:
- Oncology
- Pathology
Background:
- Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) is a rare and aggressive neoplasm.
- ES/PNET commonly originates in the chest wall and paraspinal regions, with less frequent occurrences in limbs or the genitourinary tract.
Observation:
- This report details an exceptionally rare instance of primary renal ES/PNET.
- The patient's presentation mimicked Budd-Chiari syndrome, a condition typically associated with hepatic venous outflow obstruction.
Findings:
- The study identifies a unique case of kidney ES/PNET.
- The neoplasm manifested clinically as Budd-Chiari syndrome, an atypical presentation for renal tumors.
Implications:
- This case expands the known clinical spectrum of renal ES/PNET.
- Recognizing this rare presentation is crucial for timely diagnosis and management of kidney ES/PNET, potentially improving patient outcomes.
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