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Case Reports
. 2025 May 8:17:11795735251340051.
doi: 10.1177/11795735251340051. eCollection 2025.

Paraneoplastic Tumefactive Demyelination With Underlying Anaplastic Thyroid Cancer: A Case Report and Review of the Literature

Chaloulos-Iakovidis Panagiotis  1 , Arsany Hakim  2 , Stork Lidia  3 , Stadelmann-Nessler Christine  3 , Kollár Attila  4 , Chan Andrew  1   2   3   4   5 , De Beukelaer Sophie  1 , Salmen Anke  1   5 , Hoepner Robert  1 , Helly Hammer  1
Affiliations
Case Reports

Paraneoplastic Tumefactive Demyelination With Underlying Anaplastic Thyroid Cancer: A Case Report and Review of the Literature

Chaloulos-Iakovidis Panagiotis et al. J Cent Nerv Syst Dis. .

Abstract

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.

Keywords: MRI; anaplastic thyroid cancer; paraneoplastic syndrome; tumefactive demyelination.

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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Conflict of interest statement

The author(s) declared the following potential conflicts of interest with respect to the research, authorship, and/or publication of this article: Chaloulos-Iakovidis P. has no conflict of interest to declare. • Hakim A. has no conflict of interest to declare relevant to this topic. • Stork L. has no conflict of interest to declare. • Stadelmann-Nessler C. has nothing to disclose related to this work. The team received research support by Novartis, Roche, Biogen, and Genzyme. CS served on advisory boards of Merck, Novartis and Roche and received honoraries for educational lectures from Roche and Novartis. • Kollár A. has no conflict of interest to declare relevant to this topic. • De Beukelaer S A-L. has no conflict of interest to declare. • Chan A. has served on advisory boards for, and received funding for travel or speaker honoraria from, Actelion-Janssen, Almirall, Bayer, Biogen, Celgene, Sanofi-Genzyme, Merck, Novartis, Roche, and Teva, all for hospital research funds; and research support from Biogen, Genzyme and UCB. Chan A is associate editor of the European Journal of Neurology and serves on the editorial board for Clinical and Translational Neuroscience and as topic editor for the Journal of International Medical Research. He reports no conflicts of interest related to this manuscript. • Salmen A. received speaker honoraria and/or travel compensation for activities with Almirall Hermal GmbH, Biogen, Merck, Novartis, Roche, and Sanofi Genzyme and research support by Baasch Medicus Foundation and the Swiss MS Society. She serves on the Editorial Board of Frontiers in Neurology - Multiple Sclerosis and Neuroimmunology. All not related to this work. • Hoepner R. received speaker/advisor honorary from Merck, Novartis, Roche, Biogen, Alexion, Sanof, Janssen, Bristol-Myers Squibb, Teva/Mepha and Almirall. He received research support within the last 5 years from Roche, Merck, Sanof, Biogen, Chiesi, and Bristol-Myers Squibb. He also received research grants from the Swiss MS Society and the SITEM Insel Support Fund and is a member of the Advisory Board of the Swiss and International MS Society. He also serves as vice editor-in-chief for the Journal of Central Nervous System Disease, was not involved in the reviewing process of, or decisions related to, this manuscript. • Hammer H. received research support and travel grants within the last 5 years from Biogen, Merck, Roche & BMS, not related to this work.

Figures

Figure 1.
Figure 1.
Structured Literature Search Presented in a Flow Chart.
Figure 2.
Figure 2.
Initial MRI. Axial T2 (A), T1 Post Contrast (B), DWI (C) and ADC Map (D) Show a Left Frontoparietal Well Defined Lesion With Peripheral Spiculated and Linear Enhancement With Peripheral Diffusion Restriction Representing Active Demyelination (Advancing Front). The Lesion on the Left Side was Also Extending to the Corpus Callosum (Not Shown). No Significant Space Occupying Character. There is Also Punctate Enhancement on the Right.
Figure 3.
Figure 3.
(A) H/E -Staining Showing a Macrophage-Rich Lesion, With Reactive Astrocytes. No Evidence of Malignant cells. (B) Bielschowsky Silver Impregnation Shows Preserved Axons. No Evidence of Necrotic lesions. (C) CD8 Staining Shows Single CD8 + Cytotoxic lymphocytes. (D) Pax-5-Staining Shows No Evidence of Pax5 + B Cells, Arguing Against Lymphoma. A, B, C, D Scale bar 100 µm.
Figure 4.
Figure 4.
(A, B) Proteolipid Protein Staining (PLP) Shows Demyelination (A) and Macrophages Phagocyting PLP (B). (C) Myelin Oligodendrocyte Glycoprotein Staining (MOG) Shows Macrophages Phagocyting MOG (Evidence of Early Active Demyelinating). (A) Scale bar = 100 µm, B and C Scale bar = 50 µm.
Figure 5.
Figure 5.
(A) Computer Tomography of the Neck Revealing a Large Heterogeneous Lesion Measuring 47 mm With Peripheral Enhancement and Small Calcifications in the Left Thyroid Lobe Resulting in a Mild Displacement of the Trachea to the Right. No Pathologically Enlarged Thoracic Lymph nodes. (B) Thyroid Ultrasonography Showing a Heterogeneous, Well-Defined, Solid, Predominantly Hypoechoic Mass With Macrocalcifications and Minimal Vascularization (Not Shown) of the Left Thyroid Lobe Measuring 51 × 39 × 41 mm.
Figure 6.
Figure 6.
Timeline of the Relevant Clinical Events, Diagnostic Examinations (in Light Blue) and Treatment (in Green).
Figure 7.
Figure 7.
Pie Chart Representation of all the Published Cases of Paraneoplastic Tumefactive Demyelination.
Figure 8.
Figure 8.
Longitudinal Follow-Up With Coronal T1 Post Contrast Showing the Lesion Dynamic. On the Initial Presentation (A) the Lesion Presented on the Left Side With Peripheral Spiculated and Linear Enhancement With Infiltration of the Corpus Callosum Reaching the Subependymal Region. Linear Enhancement on the Right Side was Also Seen Representing Enhancement of the Perivascular Spaces, which Increased on the Follow-Up Exams (B) Later on (C) Further Extension of the Enhancement in the Subependymal Region was Seen on the Left Side, as Well as New Lesions in the White Matter. This Pattern of Involvement of the Perivascular Spaces Represents Spreading of the Inflammation via the Glymphatic Pathway. The Subependymal Region Also Plays an Important Role in Brain Fluid Dynamics and Clearance. No Central Vein Sing or Hypointence Rim Lesions where Seen.
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