Renal primitive neuroectodermal tumor in an adolescent: a case report and review of literature

Abul Hasan Shadali Abdul Khader; Ramya C. T.; Sudeep Gaddam; Priyathersini Nagarajan; Natarajan K.; Dhaarani Jayaraman; Gokulakrishnan Periakaruppan

doi:10.18203/2349-3291.ijcp20240738

Authors

Abul Hasan Shadali Abdul Khader Department of Pediatric Hematology and Oncology, Sri. Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Ramya C. T. Department of Pediatrics, Sri. Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Sudeep Gaddam Department of Pediatric Hematology and Oncology, Sri. Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Priyathersini Nagarajan Department of Pathology, Sri. Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Natarajan K. Department of Urology, Sri. Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Dhaarani Jayaraman Department of Pediatric Hematology and Oncology, Sri. Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India
Gokulakrishnan Periakaruppan Department of Radiology, Sri. Ramachandra Institute of Higher Education and Research, Chennai, Tamil Nadu, India

DOI:

https://doi.org/10.18203/2349-3291.ijcp20240738

Keywords:

Primitive neuroectodermal tumor, Non-Wilms renal tumors, Small round blue cell tumors

Abstract

Renal primitive neuroectodermal tumor is a highly malignant neoplasm that commonly affects young adults and infrequently presented in children and adolescents. We report a case of a 14-year-old female child who presented with abdominal mass. PET-CT showed an FDG avid cystic lesion in the inter and lower polar regions of the right kidney, extending into the right renal fat and renal vein, with multiple FDG avid lesions in the axial and appendicular skeleton, suggesting renal mass with bony metastases. Biopsy confirmed the diagnosis of primitive neuroectodermal tumor of the kidney. The patient completed six cycles of chemotherapy with partial response and subsequently the patient defaulted and lost to follow-up. To emphasize the critical significance of renal PNET in the differential diagnosis of renal tumors in children.

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