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Paraneoplastic vasculitis of nerve: a remote effect of cancer
Abstract:
The development of mononeuritis multiplex brought three men to medical attention weeks to months prior to the diagnosis of a malignancy. In each case, sural nerve biopsy exhibited active wallerian degeneration with vasculitis. Two of the men died and at autopsy were found to have clinically unrecognized oat cell carcinoma and carcinomatous sensory neuropathy with brainstem encephalitis. The third patient is still alive but was found to have a lymphoma and has subsequently developed a liposarcoma. The vasculitis in all 3 cases seems limited mainly to the peripheral nervous system. Mononeuritis multiplex due to vasculitis may represent a new remote effect of cancer on the nervous system.
Insights
Mononeuritis multiplex, a nerve disorder, preceded cancer diagnosis in three men. Vasculitis affecting peripheral nerves may signal an unrecognized remote effect of malignancy.
Area of Science:
- Neurology
- Oncology
- Pathology
Background:
- Mononeuritis multiplex is a neurological condition characterized by damage to multiple individual peripheral nerves.
- Cancer can manifest with various neurological paraneoplastic syndromes, but vasculitis as a presenting sign is less common.
Purpose of the Study:
- To investigate the association between mononeuritis multiplex and underlying malignancy.
- To characterize the pathological findings in patients presenting with mononeuritis multiplex preceding cancer diagnosis.
Main Methods:
- Case series review of three male patients presenting with mononeuritis multiplex.
- Clinical evaluation, neurological examination, and sural nerve biopsy.
- Autopsy findings in deceased patients to identify underlying pathologies.
Main Results:
- All three patients developed mononeuritis multiplex weeks to months before malignancy diagnosis.
- Sural nerve biopsies revealed active Wallerian degeneration and vasculitis, primarily affecting the peripheral nervous system.
- Two patients had unrecognized small cell lung cancer (oat cell carcinoma) and carcinomatous sensory neuropathy with brainstem encephalitis; the third had lymphoma and liposarcoma.
Conclusions:
- Mononeuritis multiplex associated with vasculitis may be a novel remote effect of cancer.
- This presentation highlights the importance of considering occult malignancy in patients with vasculitic neuropathy.
- Further research is needed to elucidate the mechanisms linking cancer and vasculitic neuropathy.