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Demyelinating disease versus tumor in surgical neuropathology. Clues to a correct pathological diagnosis
D Zagzag1, D C Miller, G M Kleinman
1Department of Pathology, New York University Medical Center, NY 10016.
Abstract:
Clinical presentations as well as radiological and histopathological findings in biopsies from patients with multiple sclerosis (MS) or other demyelinating disorders of the central nervous system are sometimes misleading, resulting in an erroneous diagnosis of brain or spinal cord tumor. We report 17 patients who presented with symptoms mimicking those of brain (14 cases) or spinal cord (three cases) tumors. Computerized tomography or magnetic resonance imaging studies or both were interpreted as consistent with a tumor in each case. All patients underwent surgery, and all 17 pathological specimens were eventually diagnosed as showing demyelinating disease, usually consistent with MS. In each case we examined a variety of histological features and immunohistochemical studies and addressed their relative importance in considering the diagnosis of MS. All cases showed perivascular lymphocytic inflammation with variable amounts of macrophage infiltration, necrosis, and edema. The hypercellularity of the lesions and the presence of atypical reactive astrocytes with mitotic figures were the disturbing features that might have led to the erroneous diagnosis of an astrocytic neoplasm. Immunohistochemistry for astrocytic (glial fibrillary acidic protein) and macrophage (HAM-56) markers are helpful in evaluating biopsies. Our results emphasize the need to perform special stains (i.e., for myelin and axons) that demonstrate myelin loss and relative preservation of axons and allow a correct diagnosis.
Insights
Misleading clinical and imaging findings can cause misdiagnosis of brain tumors in multiple sclerosis (MS) patients. Special stains are crucial for differentiating demyelinating disease from neoplasms, ensuring accurate diagnosis and treatment.
Area of Science:
- Neurology
- Pathology
- Oncology
Background:
- Clinical, radiological, and histopathological findings in demyelinating disorders can mimic central nervous system tumors.
- Misdiagnosis can lead to unnecessary surgical interventions and delayed appropriate treatment for conditions like multiple sclerosis (MS).
Purpose of the Study:
- To analyze cases where demyelinating diseases were misdiagnosed as brain or spinal cord tumors.
- To identify key histological and immunohistochemical features that aid in distinguishing demyelinating lesions from neoplasms.
- To emphasize the importance of specific diagnostic techniques for accurate differentiation.
Main Methods:
- Review of 17 patient cases with initial misdiagnosis of CNS tumors.
- Histopathological examination of surgical biopsy specimens.
- Immunohistochemical analysis using glial fibrillary acidic protein (GFAP) and HAM-56 markers.
- Special stains for myelin and axons.
Main Results:
- All 17 patients initially suspected of having tumors were diagnosed with demyelinating disease, primarily MS.
- Histological features like perivascular lymphocytic inflammation, macrophage infiltration, necrosis, and edema were observed.
- Atypical reactive astrocytes and mitotic figures contributed to the initial misdiagnosis of astrocytic neoplasms.
- Immunohistochemistry and special stains were vital for correct diagnosis, showing myelin loss with relative axonal preservation.
Conclusions:
- Demyelinating diseases, particularly MS, can present with features mimicking CNS tumors.
- Careful histopathological evaluation, including immunohistochemistry and special stains, is essential to avoid misdiagnosis.
- Accurate differentiation ensures appropriate management and prevents unnecessary treatments for patients with demyelinating disorders.
