Renal Primitive Neuroectodermal Tumour: Case Report of a Rare Entity
Sai Chandana Gali1, B N Kumarguru2, Balachandra Bhat3
1Tutor and Postgraduate, Department of Pathology, PESIMSR , Kuppam, Andhra Pradesh, India .
Journal of Clinical and Diagnostic Research : JCDR
|April 8, 2017
Summary
Peripheral Primitive Neuroectodermal Tumour (PNET) of the kidney is an aggressive small round cell cancer. Diagnosis requires histopathology and immunophenotyping, as renal PNET often shows poor treatment response.
Area of Science:
- Oncology
- Pathology
Background:
- Peripheral Primitive Neuroectodermal Tumour (PNET) is a rare and aggressive small round cell malignancy.
- Renal PNET is notably more aggressive than PNET in other anatomical locations, often presenting in pediatric and adolescent populations.
Observation:
- A 24-year-old female presented with left abdominal swelling, revealing a heterogeneous left renal mass via ultrasound.
- Nephrectomy and oophorectomy were performed, exposing a large, multi-lobular tumor involving most of the kidney with focal hemorrhage.
Findings:
- Histological examination revealed diffuse sheets of small round cells with characteristic Homer-Wright and perivascular pseudo-rosettes, suggestive of PNET.
- Immunohistochemistry confirmed tumor cells expressing MIC 2, supporting the diagnosis of renal PNET.
Implications:
- Accurate differentiation of renal PNET from other renal round cell tumors is critical for appropriate management.
- The aggressive nature and poor response to standard treatments highlight the need for further research into novel therapeutic strategies for renal PNET.
