Occam Rings True (video)

Brain and orbit MRI with fat suppression demonstrated a left olfactory groove contrast-enhancing mass, bilateral papilledema with enhancing right optic nerve head, longitudinally extensive perineural enhancement of the meninges surrounding both optic nerves, sparing the optic nerve fascicles and chiasm, as well as meningeal enhancement along the ventral pons and surrounding the cervical spinal cord. No intraparenchymal lesions were identified. Computed tomography of the chest, abdomen and pelvis was normal. Sedimentation rate and C-reactive protein, complete blood count and complete metabolic panels were normal other than a mild elevation in ALT to 71 units/L. Lumbar puncture demonstrated elevated opening pressure at 31 cm H2O, 28 nucleated cells/uL (79% lymphocytes), 0 erythrocytes/uL, hypoglycorrhachia with glucose 27 mg/dL, elevated protein at 162 mg/dL. Infectious studies, including syphilis, tuberculosis and fungal testing as well as serum and CSF angiotensin converting enzyme levels were normal. CSF cytology and flow cytometry was nondiagnostic. After infectious studies returned negative, he was treated empirically with 1000 mg intravenous methylprednisolone every 24 hours. After the first dose, his visual acuity remained 2/400 OD but improved to 20/40 -2 OS. However, after the second dose his funicular pain and his vision worsened to admission baseline. Biopsy of the olfactory groove lesion revealed an adenocarcinoma with signet ring cell features. The CSF cytology was re-evaluated after the tissue diagnosis was established and felt to be consistent with metastatic adenocarcinoma with signet ring features. As body positron emission tomography did not reveal another source of his cancer, the tumor is consistent with an intestinal type sinonasal adenocarcinoma with diffuse meningeal metastases. The patient was evaluated by oncology and radiation oncology and given a very poor prognosis. Palliative whole-brain irradiation was given and he was discharged with home hospice.