Long-term survival in a patient with Li-Fraumeni syndrome-associated giant cell glioblastoma treated with nivolumab: illustrative case.

Background: Li-Fraumeni syndrome (LFS) is characterized by p53 germline mutations and a high predisposition to cancers including glioblastoma (GBM), the most common and aggressive primary malignant brain tumor in adults. Despite current therapies, the 5-year survival rate is 5%-10%. The authors report a case with a durable long-term response to immunotherapy with checkpoint inhibition in a patient with LFS-associated GBM.

Nivolumab with radiation therapy in a glioblastoma patient with Lynch syndrome.

Lynch syndrome is an autosomal dominant disorder leading to cancer predisposition caused by mutations in mismatch repair genes. There is minimal published experience treating glioblastoma in patients with Lynch syndrome. We report a patient with Lynch syndrome who was initially diagnosed with a left occipital isocitrate dehydrogenase (IDH) wild-type glioblastoma.

Atypical central neurocytoma with sarcomatous differentiation.

We report a case of an intraventricular tumor with features of atypical central neurocytoma with a sarcomatous component in a 44-year-old woman who presented with headaches and vomiting. Magnetic resonance imaging revealed a 3.4-cm lobulated enhancing mass in the occipital horn of the left lateral ventricle, and the patient subsequently underwent a left occipital-parietal craniotomy for debulking.

Addison's Disease and Pituitary Enlargement.

A 60-year-old man with Addison's disease, primary hypothyroidism and type 2 diabetes mellitus who was treated with stable doses of hydrocortisone and fludrocortisone developed increasing skin pigmentation and a bitemporal hemianopia. The plasma ACTH level was 14,464 pg/mL, and an invasive pituitary macroadenoma with suprasellar extension was found on magnetic resonance imaging leading to transnasal-transsphenoidal adenomectomy. The tumor demonstrated features of an eosinophilic adenoma and stained uniformly for ACTH.

Improvement of therapeutic index for brain tumors with daily image guidance.

Background: Image-guidance maximizes the therapeutic index of brain irradiation by decreasing setup uncertainty. As dose-volume data emerge defining the tolerance of critical normal structures responsible for neuroendocrine function and neurocognition, minimizing clinical target volume (CTV) to planning target volume (PTV) expansion of targets near these structures potentially lessens long-term toxicity.

Methods: We reviewed the treatment records of 29 patients with brain tumors, with a total of 517 fractions analyzed.