In a recent issue, the prestigious journal Neurology, Neuroimmunology, and Neuroinflammation, has published the article ‘Paraneoplastic Neurological Syndromes Associated with Merkel Cell Carcinoma’, being Dr. Nicolás Lundahl Ciano Petersen one of the main authors.
This work has been featured on the Neurology Podcast of the American Academy of Neurology, hosted by Dr. Justin Abbatemarco, a neuroimmunologist at the Cleveland Clinic. You can listen to the podcast by clicking here.
Below is a brief summary of the article, which can be read in full at the following link.
Neuroendocrine tumors are a rare and heterogenous group of tumors that arise from nervous and endocrine cells throughout the body, therefore they characteristically express neuroendocrine biomarkers. The most characteristic features is that the frequently produce paraneoplastic neurological syndromes, which are immune mediated responses erroneously targeting the nervous system.
Merkel cell carcinomas (MCC) are rare neuroendocrine skin tumors that presents as rapidly growing, painless, pinky/violaceous nodule or plaque, arising in:
- Elderly Caucasian patients
- Usually in sun-exposed skin areas like the head, neck, arms or legs, since UV radiation is of the most important risk factors.
- It was recently found that most MCC are caused by Merkel cell polyomavirus (MCPyV), an human oncovirus considered the responsible of the majority of cases of MCC.
This etiological relation with MCPyV, and its neuroendocrine nature likely play a role in the immunogenicity of MCC, and the immune-tolerance breakdown leading to PNS.
Paraneoplastic neurological syndromes associated with Merkel cell carcinomas are extremely rare conditions, with only 30 previously reported cases, and 17 additional in our cohort.
- From a clinical point of view, they are characterized by an heterogeneous clinical spectrum, mainly represented by Lambert-Eaton myasthenic syndrome, rapidly progressive cerebellar syndromes, an overlapping of Lambert-Eaton myasthenic syndrome and rapidly progressive cerebellar syndromes, and encephalomyelitis.
- From an immunological points of view we observed as well an heterogeneous antibody-profile mainly represented by antibodies against Hu, CV2/CMRP5, and VGCC-Abs, but also other atypical/poorly characterized antibodies targeting different neurofilaments chains.
- Lastly, and likely one of the most intriguing feature of MCC-related PNS is the fact that 60% patients had spontaneous primary tumor regression and Merkel cell carcinoma lymph node metastasis at PNS onset, especially among patients with VGCC-Abs and Lambert-Eaton Myasthenic syndrome.
- Firstly, they will raise awareness about the potential paraneoplastic origin of neurological disorders in patients with rare malignancies like MCC.
- AND, THEREFORE facilitate their recognition, ESPECIALLY in patients with atypical presentations, like a lymph node metastasis of small cell cancer without an identifiable primary tumor.
- ADITIONALLY, by improving the diagnosis of this extremely rare disease, we will enable treatment administration to try to improve patient outcomes.
Dr. Nicolás Lundahl Ciano-Petersen
Neurólogo en HC Marbella International Hospital
February 21, 2025
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