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      Tratamiento quirúrgico del carcinoma de Merkel facial avanzado. Presentación de un caso Translated title: Surgical treatment of advanced facial Merkel cell carcinoma. A case report

      case-report

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          Abstract

          Resumen Introducción. El carcinoma de Merkel es un tumor maligno poco frecuente, que afecta principalmente a la población caucásica y cuya etiología guarda relación con el poliomavirus de las células de Merkel. Conlleva mal pronóstico, especialmente en estadios finales. Caso clínico. Se expone el caso de una paciente que presentaba un tumor primario facial de grandes dimensiones, con avanzado grado de extensión, afectación linfática cervical y metástasis parotídea derecha. Fue tratada mediante exéresis de la lesión primaria y cobertura con injerto de piel parcial, linfadenectomía cervical y parotidectomía ipsilateral. Resultados. Se logró mejoría importante en la calidad de vida de la paciente y sobrevida de al menos seis meses. Conclusión. Aunque no está claro el manejo óptimo del carcinoma de Merkel avanzado debido a su mal pronóstico, la cirugía favorece una mejoría en la calidad de vida del paciente y puede tener un papel clave en el manejo del carcinoma de Merkel en los estadios avanzados.

          Translated abstract

          Abstract Introduction. Merkel carcinoma is a rare malignant tumor that mainly affects the Caucasian population and whose etiology is related to the Merkel cell polyomavirus. It has a poor prognosis, especially in the final stages. Clinical case. The case of a patient who presented a large primary facial tumor, with an advanced degree of extension, cervical lymphatic involvement and right parotid metastasis is described. She was treated surgically by excision of the primary lesion and coverage with partial skin graft, cervical lymphadenectomy, and ipsilateral parotidectomy. Results. A significant improvement was achieved in the patient’s quality of life and survival of at least six months. Conclusion. Although the optimal management of advanced Merkel carcinoma is unclear due to its poor prognosis, surgery improves the patient’s quality of life and it can play a key role in the management of Merkel carcinoma in advanced stages.

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          Most cited references10

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          Clonal integration of a polyomavirus in human Merkel cell carcinoma.

          Merkel cell carcinoma (MCC) is a rare but aggressive human skin cancer that typically affects elderly and immunosuppressed individuals, a feature suggestive of an infectious origin. We studied MCC samples by digital transcriptome subtraction and detected a fusion transcript between a previously undescribed virus T antigen and a human receptor tyrosine phosphatase. Further investigation led to identification and sequence analysis of the 5387-base-pair genome of a previously unknown polyomavirus that we call Merkel cell polyomavirus (MCV or MCPyV). MCV sequences were detected in 8 of 10 (80%) MCC tumors but only 5 of 59 (8%) control tissues from various body sites and 4 of 25 (16%) control skin tissues. In six of eight MCV-positive MCCs, viral DNA was integrated within the tumor genome in a clonal pattern, suggesting that MCV infection and integration preceded clonal expansion of the tumor cells. Thus, MCV may be a contributing factor in the pathogenesis of MCC.
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            Merkel cell carcinoma

            Merkel cell carcinoma (MCC) is a rare but highly aggressive skin cancer with neuroendocrine features. MCC pathogenesis is associated with either the presence of Merkel cell polyomavirus or chronic exposure to ultraviolet light (UV), which can cause a characteristic pattern of multiple DNA mutations. Notably, in the Northern hemisphere, the majority of MCC cases are of viral aetiology; by contrast, in areas with high UV exposure, UV-mediated carcinogenesis is predominant. The two aetiologies share similar clinical, histopathological and prognostic characteristics. MCC presents with a solitary cutaneous or subcutaneous nodule, most frequently in sun-exposed areas. In fact, UV exposure is probably involved in both viral-mediated and non-viral-mediated carcinogenesis, by contributing to immunosuppression or DNA damage, respectively. Confirmation of diagnosis relies on analyses of histological features and immunological marker expression profiles of the lesion. At primary diagnosis, loco-regional metastases are already present in ~30% of patients. Excision of the tumour is the first-line therapy; if not feasible, radiotherapy can often effectively control the disease. Chemotherapy was the only alternative in advanced-stage or refractory MCC until several clinical trials demonstrated the efficacy of immune-checkpoint inhibitors.
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              Trabecular Carcinoma of the Skin

                Author and article information

                Journal
                rcci
                Revista Colombiana de Cirugía
                rev. colomb. cir.
                Asociación Colombiana de Cirugía (Bogotá, Distrito Capital, Colombia )
                2011-7582
                2619-6107
                February 2024
                : 39
                : 1
                : 141-147
                Affiliations
                [1] Córdoba orgnameHospital Universitario Reina Sofía España
                [2] Andalucía orgnameUniversidad de Jaén orgdiv1Servicio de Cirugía general y digestiva Spain
                [3] Córdoba orgnameHospital Universitario Reina Sofía orgdiv1Servicio de Anatomía patológica España
                Author information
                https://orcid.org/0000-0001-6021-7365
                https://orcid.org/0000-0003-1501-5937
                https://orcid.org/0000-0001-7847-6701
                https://orcid.org/0000-0002-0014-3816
                Article
                S2011-75822024000100141 S2011-7582(24)03900100141
                10.30944/20117582.2337
                f7274bf7-1c16-43f9-ba3b-b46ce9ffd4b8

                This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.

                History
                : 04 February 2023
                : 15 March 2023
                Page count
                Figures: 0, Tables: 0, Equations: 0, References: 10, Pages: 7
                Product

                SciELO Colombia

                Categories
                Presentación de caso

                plastic surgery,skin transplantation,neuroendocrine carcinoma,head and neck neoplasms,Merkel cell carcinoma,cirugía plástica,trasplante de piel,carcinoma neuroendocrino,neoplasias de cabeza y cuello,carcinoma de células de Merkel

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