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[Primitive neuroectodermal tumor with Wilms' tumor. Case report].

T Kato1, T Aida, H Abe

  • 1Department of Neurosurgery, Hokkaido University, Sapporo.

Neurologia Medico-Chirurgica
|December 1, 1991
PubMed
Summary
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This study reports a rare case of central nervous system-renal neoplasia in an infant, highlighting the co-occurrence of primitive neuroectodermal tumors and Wilms' tumors. The aggressive nature of these combined pediatric cancers led to metastasis and patient expiration.

Area of Science:

  • Pediatric Oncology
  • Neuro-oncology
  • Nephrology

Background:

  • Primitive neuroectodermal tumors (PNETs) and Wilms' tumors are rare pediatric malignancies.
  • Central nervous system-renal neoplasia describes the co-occurrence of these tumor types.
  • The embryological origins and potential shared pathways of PNETs and Wilms' tumors remain an area of investigation.

Observation:

  • A case report of an infant diagnosed with PNETs in the brain (lateral ventricle and cerebellar vermis).
  • Simultaneous discovery of bilateral Wilms' tumors in the kidneys.
  • Despite treatment with chemotherapy and radiation, the patient experienced tumor recurrence, lung metastasis, and peritoneal dissemination.

Findings:

  • Autopsy confirmed recurrent PNET in the brain and widespread Wilms' tumor recurrence with metastasis.

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  • The study found no definitive evidence for a shared histogenetic or cytogenetic origin for the co-occurring tumors.
  • However, the presence of embryonal cells in neural tissue suggests a potential neuroepithelial origin for Wilms' tumors.

    • Implications:

    • This case underscores the complexity of rare pediatric cancer syndromes.
    • Further research into the developmental biology of embryonal cells may elucidate the link between central nervous system and renal tumors.
    • Understanding these associations is crucial for improving diagnostic and therapeutic strategies in pediatric oncology.