| Sumario: | We present a case of paraneoplastic tumefactive demyelination in a 55-year-old female with an underlying anaplastic thyroid carcinoma (ATC), alongside a review of the literature on all cases of tumefactive demyelination associated with non-CNS neoplasia. In the presented case the patient developed a right-sided subacute sensorimotor hemiparesis. The initial cerebral MRI revealed a bilateral frontoparietal tumefactive mass lesion with marked gadolinium uptake and mass effect. Cerebrospinal fluid revealed CSF-specific oligoclonal bands type III, with negative cell count, protein and pathogen testing. Brain biopsy indicated demyelination and T-cell infiltrates and foamy macrophages. A body CT revealed an anaplastic thyroid carcinoma. Despite steroids, plasma exchange, rituximab, and cancer treatment, the patient died due to clinical fluctuation and cancer progression. In addition to our case 9 cases of tumefactive demyelinating have been reported in patients with newly diagnosed extracranial neoplasia, most commonly seminoma germ cell tumour (7/10). 8/10 (80%) of patients were male, with mean age at diagnosis was 52.9 years 95% C.I. [43.8, 62.0]. 5/10 patients presented with sensorimotor hemiparesis and/or confusion/neurocognitive deficits. 4/10 with visual deficits and 2/10 with aphasia. In all cases neoplasia was diagnosed simultaneously or after neurological manifestations. All cases presented initially as solitary lesions. A malignancy specific-treatment as well as steroid treatment in different regiments were applied. In addition in 2/10 plasmapheresis was implemented and 1/10 patients received intravenous immunoglobulins. In the majority of cases including the presented case partial neurological improvement was documented whereas malignancy usually progressed. To our knowledge, this is the first report of paraneoplastic tumefactive demyelination associated with an ATC highlighting the importance of a thorough workup in these patients. This is the first reported case of paraneoplastic tumefactive demyelination associated with ATC, underscoring the necessity of a comprehensive diagnostic approach in similar patients. Plain Language Summary: Why was this study done? We reported a case of a rare brain condition called tumefactive demyelination in a patient with an aggressive type of thyroid cancer (anaplastic thyroid cancer, or ATC). Tumefactive demyelination is when the immune system attacks the protective covering of nerve fibers in the brain called myelin, creating large, tumor-like lesions. This condition is typically not seen in patients with cancer, so we wanted to explore this rare connection and review similar cases. What did the researchers do? We examined the case of a 55-year-old woman who developed weakness on one side of her body. Brain scans showed a large lesion in her brain, and further tests indicated myelin damage. A body scan later revealed she also had thyroid cancer. Despite treatment for both the brain lesion and the cancer, the patient's condition worsened, and she passed away due to cancer progression. We also reviewed 9 similar cases of this brain condition occurring alongside cancer. What did the researchers find? In our case and the other cases reviewed, patients developed brain lesions around the time they were diagnosed with cancer. Most patients showed improvement in their neurological symptoms after treatment, but the cancer itself usually progressed. This highlights the difficulty in treating both conditions simultaneously. What do the findings mean? This is the first report of a patient with thyroid cancer developing this rare brain condition, and it suggests that thorough testing should be done for cancer patients who develop unusual neurological symptoms. Early detection and treatment of both conditions are important, but the prognosis remains challenging due to the aggressive nature of both cancer and brain lesions with inflamation.
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