Combined treatment with radiotherapy, chemotherapy and avelumab results in regression of metastatic Merkel cell carcinoma and improvement of associated Lambert‑Eaton myasthenic syndrome: A case report

Green,Caroline; Mettävainio,Martin Isaksson; Kjellman,Christina; Ramqvist,Torbjörn; Dalianis,Tina; Israelsson,Pernilla; Lindquist,David

doi:10.3892/ol.2022.13513

Combined treatment with radiotherapy, chemotherapy and avelumab results in regression of metastatic Merkel cell carcinoma and improvement of associated Lambert‑Eaton myasthenic syndrome: A case report

Authors:
- Caroline Green
- Martin Isaksson Mettävainio
- Christina Kjellman
- Torbjörn Ramqvist
- Tina Dalianis
- Pernilla Israelsson
- David Lindquist
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Affiliations: Department of Clinical Sciences, Umeå University, SE‑90187 Umeå, Sweden, Department of Medical Biosciences, Umeå University, SE‑90187 Umeå, Sweden, Department of Oncology‑Pathology, Karolinska Institute, SE‑17177 Stockholm, Sweden, Department of Radiation Sciences, Umeå University, SE‑90187 Umeå, Sweden
Published online on: September 21, 2022 https://doi.org/10.3892/ol.2022.13513
Article Number: 393
Copyright: © Green et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Merkel cell carcinoma (MCC) is a rare and highly aggressive neuroendocrine malignancy arising from mechanoreceptors in the basal epidermis. Due to a pronounced risk of spread and a high propensity for recurrence after treatment, immediate treatment is of utmost importance. Lambert‑Eaton myasthenic syndrome (LEMS) is a paraneoplastic phenomenon affecting the muscles with autoimmune pathophysiology, and >50% of known cases are associated with an underlying malignancy. In the present report, the case of a 67‑year‑old man presenting with progressive proximal muscle weakness, autonomic dysfunction and involuntary weight loss is described. Symptoms and detection of voltage‑gated calcium channel antibodies were consistent with LEMS. Distant metastases were found in the inguinal and iliac lymph nodes, and these were immunohistochemically confirmed to be of epithelial and neuroendocrine origin, consistent with MCC. Local radiotherapy and chemotherapy improved the symptoms; however, a change of treatment was required due to the side effects of the chemotherapy. Avelumab, an immune checkpoint inhibitor, was therefore introduced, and within a year the patient did not only experience tumor remission but also exhibited marked improvements in muscle strength and mobility. At present, 2 years later, the MCC is still in remission. To the best of our knowledge, the present report is the first to describe MCC with associated LEMS, which was successfully treated with avelumab after previous radiotherapy and chemotherapy, with both improved functional motor recovery and tumor reduction. In conclusion, the present case report demonstrated that the present treatment strategy is a potential treatment option and could thus be considered in similar cases.

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