Paediatric Peripheral Primitive Neuroectodermal Tumour - A Clinico-Pathological Study from Southern India

Area of Science:

• Pediatric Oncology
• Skeletal Muscle Tumors
• Pediatric Pathology

Background:

• Primitive Neuroectodermal Tumors (PNET) and Ewing Sarcomas (ES) are aggressive childhood cancers.
• These malignancies exhibit neuroectodermal differentiation.

Purpose of the Study:

• To analyze the clinical presentation, morphology, immunohistochemistry (IHC), management, and outcomes of pediatric PNET/ES.
• To evaluate factors influencing survival in this patient cohort.

Main Methods:

• Retrospective study of biopsy-proven pediatric PNET/ES cases (<18 years) over six years.
• Clinical data, treatment, and follow-up were retrieved and analyzed.
• Survival rates were calculated, and Kaplan-Meier curves were generated.

Main Results:

• Eleven cases of pediatric peripheral PNET/ES were identified.
• The mean age at presentation was 94.08 months; male/female ratio was 1.2:1.
• 81.8% survival rate observed; metastasis at diagnosis was associated with significantly shorter survival (9.66 months vs. 25 months for localized disease).

Conclusions:

• Metastasis at diagnosis is the primary determinant of poor prognosis in pediatric PNET/ES.
• Immunohistochemistry (IHC) is crucial for accurate diagnosis of these small round blue cell tumors.
• Multidisciplinary management and supportive care enhance survival for localized disease.