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PNET of kidney: Report of four cases

Palash Kumar Mandal1, Supti Mukherjee, Sravasti Roy

  • 1Department of Pathology, Cancer Center Welfare Home and Research Institute, Kolkata, West Bengal, India.

Indian Journal of Medical and Paediatric Oncology : Official Journal of Indian Society of Medical & Paediatric Oncology
|September 19, 2012
PubMed

Insights

Primitive neuroectodermal tumor (PNET) of the kidney is a rare and aggressive cancer. Early diagnosis and differentiation from other small round cell tumors are crucial for effective treatment and improved patient outcomes.

Area of Science:

  • Oncology
  • Pathology

Background:

  • Primitive neuroectodermal tumor (PNET) of the kidney is an exceptionally rare malignancy.
  • Limited case reports exist, highlighting the need for further clinical and pathological understanding.

Observation:

  • Four adult cases (30-50 years) presented with vague loin pain and palpable masses.
  • Renal masses were identified on imaging; one patient experienced hematuria.
  • All cases were diagnosed at advanced stages.

Findings:

  • Pathological examination confirmed PNET, with CD99 membrane positivity on immunohistochemistry.
  • EWS-FLI-1 gene fusion analysis was not performed.
  • Despite advanced stage, patients survived post-treatment with radiotherapy and chemotherapy.

Implications:

  • Timely reporting of rare renal PNET cases is vital due to their aggressive nature and distinct therapeutic needs.
  • Accurate differentiation from other small blue round cell tumors (e.g., neuroblastoma, nephroblastoma, NHL) is essential.
  • Immunohistochemistry, cytogenetics, and molecular studies are critical for precise diagnosis and tailored treatment strategies.
Keywords:
CD99immunohistochemistryprimitive neuroectodermal tumor of kidney

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