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Published on: October 15, 2021
Surgical treatment of intracranial Erdheim-Chester disease
Alex Alfieri1, Roberto Gazzeri, Marcelo Galarza
1Department of Neurological Surgery, Martin-Luther University Halle-Wittenberg, Halle, Germany. alex.alfieri@medizin.uni-halle.de
Insights
Erdheim-Chester disease (ECD) is a rare non-Langerhans histiocytosis. Intracranial ECD, though rare, presents with neurological symptoms and its treatment remains controversial.
Area of Science:
- Neurology
- Oncology
- Histiocytosis
Background:
- Erdheim-Chester disease (ECD) is a rare systemic non-Langerhans histiocytosis affecting multiple organs.
- Intracranial involvement in ECD is infrequent, with limited cases documented in international literature.
- Clinical manifestations include leg pain, exophthalmos, and diabetes insipidus (DI), with neurological symptoms often involving DI and cerebellar signs.
Purpose of the Study:
- To review the clinical presentation, radiological and histological characteristics, and natural history of intracranial Erdheim-Chester disease.
- To summarize the diagnostic and therapeutic approaches for central nervous system ECD.
- To highlight the role of surgical interventions in the diagnosis and management of intracranial ECD.
Main Methods:
- Literature review of intracranial Erdheim-Chester disease cases.
- Analysis of clinical presentation, radiological findings, and histological characteristics.
- Evaluation of treatment strategies, including surgical resection and radiation therapy.
Main Results:
- Central nervous system involvement in ECD is rare, with only 27 reported patients.
- Diabetes insipidus and cerebellar signs are the most common neurological symptoms.
- Intracranial surgical procedures were performed in 11 patients, serving as the initial diagnostic method in seven.
Conclusions:
- Intracranial Erdheim-Chester disease is a rare manifestation of a multi-systemic disorder.
- The diagnosis and management of intracranial ECD, particularly surgical interventions, require careful consideration due to controversial treatment options.
- Further research is needed to establish optimal treatment protocols for intracranial ECD.
Abstract:
We review the clinical presentation, radiological and histological characteristics, and the natural history, of intracranial Erdheim-Chester disease (ECD). ECD is a rare form of non-Langerhans histiocytosis that affects multiple organs. It is clinically characterized by leg pain, exophthalmos and diabetes insipidus (DI). Central nervous system involvement is rare, with only 27 patients reported in the international literature. DI and cerebellar signs represent the most common neurological symptoms. Its treatment is controversial. Intracranial surgical procedures for ECD have been reported in 11 patients with a complete surgical resection performed in six, and an intracerebral biopsy performed in five patients. In seven patients the cranial procedures represented the initial diagnostic method. Surgical resection and radiation therapy have been used in the further management of these cerebral lesions.
