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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

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 s...

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Autores principales: Green, Caroline, Isaksson Mettävainio, Martin, Kjellman, Christina, Ramqvist, Torbjörn, Dalianis, Tina, Israelsson, Pernilla, Lindquist, David
Formato: Online Artículo Texto
Lenguaje:English
Publicado: D.A. Spandidos 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9533363/
https://www.ncbi.nlm.nih.gov/pubmed/36276497
http://dx.doi.org/10.3892/ol.2022.13513
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author Green, Caroline
Isaksson Mettävainio, Martin
Kjellman, Christina
Ramqvist, Torbjörn
Dalianis, Tina
Israelsson, Pernilla
Lindquist, David
author_facet Green, Caroline
Isaksson Mettävainio, Martin
Kjellman, Christina
Ramqvist, Torbjörn
Dalianis, Tina
Israelsson, Pernilla
Lindquist, David
author_sort Green, Caroline
collection PubMed
description 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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spelling pubmed-95333632022-10-20 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 Isaksson Mettävainio, Martin Kjellman, Christina Ramqvist, Torbjörn Dalianis, Tina Israelsson, Pernilla Lindquist, David Oncol Lett Articles 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. D.A. Spandidos 2022-09-21 /pmc/articles/PMC9533363/ /pubmed/36276497 http://dx.doi.org/10.3892/ol.2022.13513 Text en Copyright: © Green et al. https://creativecommons.org/licenses/by-nc-nd/4.0/This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License (https://creativecommons.org/licenses/by-nc-nd/4.0/) , which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.
spellingShingle Articles
Green, Caroline
Isaksson Mettävainio, Martin
Kjellman, Christina
Ramqvist, Torbjörn
Dalianis, Tina
Israelsson, Pernilla
Lindquist, David
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
title 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
title_full 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
title_fullStr 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
title_full_unstemmed 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
title_short 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
title_sort 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
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9533363/
https://www.ncbi.nlm.nih.gov/pubmed/36276497
http://dx.doi.org/10.3892/ol.2022.13513
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