Merkel cell carcinoma: case report
Abstract: Introduction: Merkel Cell Carcinoma (MCC) is a neuroendocrine neoplasm first described by Toker in 1972. Pathogenesis is not clear; but ultraviolet radiation, immunosuppression, and the presence of polymavirus (MCPyV) in the tumor genome seems to influence its development, i...
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Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología
2021
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| Acceso en línea: | https://revistas.unc.edu.ar/index.php/med/article/view/34839 |
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I10-R10-article-34839 |
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Universidad Nacional de Córdoba |
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R-10 |
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Revistas de la UNC |
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Artículo revista |
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skin neoplasms Merkel cell carcinoma polymavirus infections neoplasias de la piel carcinoma de células de Merkel infecciones por poliomavirus . |
| spellingShingle |
skin neoplasms Merkel cell carcinoma polymavirus infections neoplasias de la piel carcinoma de células de Merkel infecciones por poliomavirus . Martinello , M Villata , AC Alcain , WL Szlabi, S Pérez , HJ Cremonezzi, DC Merkel cell carcinoma: case report |
| topic_facet |
skin neoplasms Merkel cell carcinoma polymavirus infections neoplasias de la piel carcinoma de células de Merkel infecciones por poliomavirus . |
| author |
Martinello , M Villata , AC Alcain , WL Szlabi, S Pérez , HJ Cremonezzi, DC |
| author_facet |
Martinello , M Villata , AC Alcain , WL Szlabi, S Pérez , HJ Cremonezzi, DC |
| author_sort |
Martinello , M |
| title |
Merkel cell carcinoma: case report |
| title_short |
Merkel cell carcinoma: case report |
| title_full |
Merkel cell carcinoma: case report |
| title_fullStr |
Merkel cell carcinoma: case report |
| title_full_unstemmed |
Merkel cell carcinoma: case report |
| title_sort |
merkel cell carcinoma: case report |
| description |
Abstract:
Introduction: Merkel Cell Carcinoma (MCC) is a neuroendocrine neoplasm first described by Toker in 1972. Pathogenesis is not clear; but ultraviolet radiation, immunosuppression, and the presence of polymavirus (MCPyV) in the tumor genome seems to influence its development, it usually occurs in people over 60 years of age, preferably in the extremities and head and neck area. Clinically it looks like a single protruding lump with a hard consistency, very similar to other skin tumors. It is often accompanied by regional lymphadenopathy. Overall survival of stage I patients is 80%. On the contrary once distant metastases have developed, it is only of five months.
The objective of this presentation is to report a rare case of skin neoplasia with a very poor prognosis, its diagnostic approach, differential diagnoses and its viral etiology.
Presentation of the case: 55-year-old woman who consults for a tumor lesion in her elbow. Biopsy was decided: a 6 x 5cm skin lozenge that, on the surface, presented a raised, brown and ulcerated tumor lesion. When cut it had a whitish gray color, firm consistency and a maximum thickness of 4,5 cm. Sample was processed with the usual technique for staining with hematoxylin/eosin. Histologically, a proliferation of invasive neoplasic cells of neuroendocrine lineage was observed, arranged in large nests separated by desmoplasic stroma. It presented small, round and blue cells, with little and poorly defined cytoplasm and nuclei with finely granular “salt and pepper” chromatin. Mitosis count was high and extensive areas of necrosis were observed. The tumor compromised the entire thickness of the sample, contacting the surgical margin. This study is complemented with immunohistochemistry: CK 20 +, AE1/AE3 +, synaptophysin +, INSIM.1 +, polyoma virus +.
Conclusion: the MCC constitutes the skin neoplasm with the worst prognosis. Our case was related to viral etiology confirmed by immunohistochemistry. It is necessary to distinguish it from cutaneous lymphomas, which have a better prognosis. Therefore, its rapid diagnosis with immunohistochemical confirmation is essential for timely treatment.
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| publisher |
Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología |
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2021 |
| url |
https://revistas.unc.edu.ar/index.php/med/article/view/34839 |
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I10-R10-article-348392022-06-07T14:43:30Z Merkel cell carcinoma: case report Carcinoma de células de Merkel: comunicación de un caso A Martinello , M Villata , AC Alcain , WL Szlabi, S Pérez , HJ Cremonezzi, DC skin neoplasms Merkel cell carcinoma polymavirus infections neoplasias de la piel carcinoma de células de Merkel infecciones por poliomavirus . Abstract: Introduction: Merkel Cell Carcinoma (MCC) is a neuroendocrine neoplasm first described by Toker in 1972. Pathogenesis is not clear; but ultraviolet radiation, immunosuppression, and the presence of polymavirus (MCPyV) in the tumor genome seems to influence its development, it usually occurs in people over 60 years of age, preferably in the extremities and head and neck area. Clinically it looks like a single protruding lump with a hard consistency, very similar to other skin tumors. It is often accompanied by regional lymphadenopathy. Overall survival of stage I patients is 80%. On the contrary once distant metastases have developed, it is only of five months. The objective of this presentation is to report a rare case of skin neoplasia with a very poor prognosis, its diagnostic approach, differential diagnoses and its viral etiology. Presentation of the case: 55-year-old woman who consults for a tumor lesion in her elbow. Biopsy was decided: a 6 x 5cm skin lozenge that, on the surface, presented a raised, brown and ulcerated tumor lesion. When cut it had a whitish gray color, firm consistency and a maximum thickness of 4,5 cm. Sample was processed with the usual technique for staining with hematoxylin/eosin. Histologically, a proliferation of invasive neoplasic cells of neuroendocrine lineage was observed, arranged in large nests separated by desmoplasic stroma. It presented small, round and blue cells, with little and poorly defined cytoplasm and nuclei with finely granular “salt and pepper” chromatin. Mitosis count was high and extensive areas of necrosis were observed. The tumor compromised the entire thickness of the sample, contacting the surgical margin. This study is complemented with immunohistochemistry: CK 20 +, AE1/AE3 +, synaptophysin +, INSIM.1 +, polyoma virus +. Conclusion: the MCC constitutes the skin neoplasm with the worst prognosis. Our case was related to viral etiology confirmed by immunohistochemistry. It is necessary to distinguish it from cutaneous lymphomas, which have a better prognosis. Therefore, its rapid diagnosis with immunohistochemical confirmation is essential for timely treatment. Resumen: Introducción: El carcinoma de células de Merkel (CCM) es una neoplasia neuroendocrina descripta por primera vez por Toker en1972. La patogénesis no está clara, pero la radiación ultravioleta, la inmunosupresión y la presencia del poliomavirus (MCPyV) en el genoma del tumor parecen influir en su desarrollo. Se presenta habitualmente en mayores de 60 años preferentemente en extremidades y región de cabeza y cuello. Clínicamente se ve como una tumoración única, protruyente de consistencia dura, muy similar a otros tumores cutáneos. Suele acompañarse de linfadenopatías regionales. La supervivencia global de los pacientes en estadio I es del 80%. Por el contrario, una vez que se han desarrollado metástasis a distancia, es de sólo cinco meses. El objetivo de esta presentación es comunicar un caso infrecuente de neoplasia de piel de muy mal pronóstico, su abordaje diagnóstico, los diagnósticos diferenciales y su etiología viral. Presentación del caso: mujer de 55a que consultó por una lesión tumoral en el codo. Se decidió realizar biopsia: losange de piel de 6 x 5 cm que, en superficie, presentó una lesión tumoral sobreelevada, de color pardo y ulcerada. Al corte tuvo colorido blanquecino-grisáceo, consistencia firme y un espesor máximo de 4,5 cm. El material se procesó con técnica habitual para coloración con hematoxilina/eosina. Histológicamente se observó una proliferación de células neoplásicas invasoras de estirpe neuroendocrina, dispuestas en grandes nidos separados por estroma desmoplásico. Presentó células pequeñas, redondas y azules, con escaso citoplasma mal definido y núcleos con cromatina finamente granular “en sal y pimienta”. El conteo de mitosis fue alto y se observaron extensas áreas de necrosis. El tumor comprometió todo el espesor de la muestra, contactando con el margen quirúrgico. El estudio se complementó con inmunohistoquímica: CK 20 +, AE1/AE3 +, Sinaptofisina +, INSM1 +, polioma virus +. Conclusión: El CCM, constituye la neoplasia de piel de peor pronóstico. Nuestro caso se relacionó con etiología viral confirmado por inmunihistoquímica. Es necesario distinguirlo de linfomas cutáneos, que poseen mejor pronóstico. Por lo tanto su rápido diagnóstico histológico con confirmación inmunohistoquímica es indispensable para un tratamiento oportuno. . Universidad Nacional Cba. Facultad de Ciencias Médicas. Secretaria de Ciencia y Tecnología 2021-11-03 info:eu-repo/semantics/article info:eu-repo/semantics/publishedVersion Texto https://revistas.unc.edu.ar/index.php/med/article/view/34839 Revista de la Facultad de Ciencias Médicas de Córdoba.; Vol. 78 No. Suplemento (2021): Suplemento JIC XXII Revista de la Facultad de Ciencias Médicas de Córdoba; Vol. 78 Núm. Suplemento (2021): Suplemento JIC XXII Revista da Faculdade de Ciências Médicas de Córdoba; v. 78 n. Suplemento (2021): Suplemento JIC XXII 1853-0605 0014-6722 http://creativecommons.org/licenses/by-nc/4.0 |