A 10-year-old child has a cerebellar cyst with a mural nodule. Histology shows a biphasic pattern with Rosenthal fibers and eosinophilic granular bodies. Which syndrome is most associated with this tumor?
- A Von Hippel-Lindau disease
- B Tuberous sclerosis
- C Neurofibromatosis type 1 ✓
- D Li-Fraumeni syndrome
Correct answer: C. Neurofibromatosis type 1
Explanation
The tumor is pilocytic astrocytoma, a WHO grade 1 glioma. It is strongly associated with neurofibromatosis type 1, especially when located in the optic pathway. Tuberous sclerosis is linked to subependymal giant cell astrocytoma, Von Hippel-Lindau to hemangioblastoma, and Li-Fraumeni to TP53-mutant gliomas.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
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