Background: Extrapulmonary small cell carcinoma (EPSCC) involving the brain is a rare manifestation of an uncommon tumor type. Case presentation: We report a 59 year-old Caucasian female diagnosed with an EPSCC involving the left parietal lobe without detectable extracranial primary tumor followed by serial positron emission tomography/computed tomography (PET/CT) imaging. Histopathological examination at both initial presentation and recurrence revealed small cell carcinoma. Serial PET/CT scans of the entire body failed to reveal any extracranial [18F]2-fluoro-2-deoxy-D-glucose (FDG) avid lesions at either diagnosis or follow-up. Conclusion: Chemotherapy may show a transient response in the treatment of EPSCC. Further studies are needed to help identify optimal treatment strategies. Combination PET/CT technology may be a useful tool to monitor EPSCC and assess for an occult primary malignancy.
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