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and most of them are diagnosed coincidentally on removal of another epidermoid or basal-cell carcinoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> We report 2 cases of incipient Merkel cell carcinoma not associated with another lesion&#59; both tumors had a diameter of less than 5<span class="elsevierStyleHsp" style=""></span>mm&#46; We discuss the most appropriate treatment in these cases of small Merkel cell carcinoma and highlight the distinctive histologic aspect and unusual location&#8212;the dermoepidermal junction in one case and the junction between the dermis and the subcutaneous tissue in the other case&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case 1</span><p id="par0015" class="elsevierStylePara elsevierViewall">The first case was a 79-year-old English patient with a history of gastric ulcer and occupational exposure to asbestos&#46; The patient was being followed up in our department because of a basal cell carcinoma located on the tip of the nose&#59; 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and S-100 protein &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Once the tumor had been diagnosed as Merkel cell carcinoma&#44; treatment was completed using Mohs surgery to ensure complete excision of the malignant lesion&#59; the margin was negative after a single stage&#46; The staging study&#44; which included computed tomography &#40;CT&#41; of the chest&#44; abdomen&#44; and pelvis&#44; was negative for tumors&#59; the CT scan incidentally revealed calcified pleural plaques characteristic of asbestosis&#46; The patient was free of disease after 10 months of follow-up&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case 2</span><p id="par0025" class="elsevierStylePara elsevierViewall">The second patient was an 82-year-old woman with no relevant history&#44; who visited our department with a lesion that had appeared 2 weeks earlier on the left buttock&#46; Examination revealed a very small subcutaneous nodular lesion measuring less than a centimeter&#59; the lesion was completely nonspecific&#44; covered by normal skin&#44; and palpable but not visible&#46; A punch biopsy showed a proliferation of monomorphic round cells with large vesicular nuclei&#44; no nucleoli&#44; and abundant mitosis in the deep dermis&#44; almost reaching the hypodermis &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; This proliferation of cells was accompanied by an infiltrate of nontumorous lymphocytes&#46; Immune staining was positive for cytokeratin 20 and chromogranin A&#44; and negative for TTF-1&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">In this case&#44; the staging study included a CT scan of the chest&#44; abdomen&#44; and pelvis&#44; and positron emission tomography &#40;PET&#41;&#59; the results of all tests were negative&#46; Treatment was completed by extending the margin of excision by 2<span class="elsevierStyleHsp" style=""></span>cm in diameter and down to the fascia and studying the sentinel node&#46; Both the extended margin and the sentinel node were negative for tumors&#46; The patient was still free of disease after a year and a half of follow-up&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">Management of patients with Merkel cell carcinoma is often complex&#46; The aggressive nature of the tumor and its low frequency make it difficult to establish a reliable and uniformly accepted management protocol&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> In general&#44; the most appropriate treatment is considered to be surgery plus radiation therapy of the surgical site&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> accompanied&#44; when applicable&#44; by a study of the sentinel nodes or selective lymph node resection&#44; sometimes with radiation therapy of the lymph node drainage area&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> However&#44; the wide surgical excision recommended in the literature is not always possible&#44; and no specific recommendations exist regarding ideal surgical margins&#46; Mohs surgery is increasingly recommended for the treatment of Merkel cell carcinoma&#44; but this modality is not available in many hospitals&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> As the tumor is known to be sensitive to radiation&#44; the use of adjuvant radiation therapy after surgery would seem to be logical&#46; However&#44; in practice&#44; we should always be reticent about treating a very small tumor on the face of a patient &#40;as in our first case&#41; with aggressive initial surgery plus radiation therapy&#46; A study by Stokes et al&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> reviewed 346 cases of Merkel cell carcinoma&#44; with known lymph node staging &#40;either because the tumor had a sentinel node or because selective lymph node resection had been performed&#41; in 213 cases&#46; Those authors correlated lymph node staging with the size of the primary Merkel cell tumor and found that tumors measuring less than 1 centimeter were found in only 2 patients &#40;4&#37;&#41; with lymph node involvement&#46; Furthermore&#44; lymph node involvement in those 2 patients was not detected by means of the sentinel node&#44; as the enlarged nodes in these patients were clinically palpable&#46; The authors therefore concluded that the sentinel node technique is probably not justified in Merkel cell tumors with a diameter of less than 1 centimeter&#46; In our 2 cases&#44; the therapeutic decision was made prior to the publication of that study&#46; The notably small size of both tumors led us to discuss the most appropriate treatment in a tumor committee&#46; We decided to perform a sentinel node study in the second patient but not in the first patient&#44; and treated the tumors somewhat similarly to the way we manage cases of melanoma&#44; that is&#44; based on a pseudo-Breslow assessment of the tumors&#46; Thus&#44; the first case showed a Breslow depth of less than 1<span class="elsevierStyleHsp" style=""></span>mm&#44; whereas the second tumor&#44; which was almost in the hypodermis&#44; had a considerably greater Breslow depth&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The other reason we report these 2 cases is that the histologic appearance of the tumors led us to think about another aspect of Merkel cell carcinoma that is the subject of debate&#58; its phylogeny&#46; It has traditionally been accepted that this carcinoma originates in the Merkel cells&#44; based on the similarities in immunohistochemistry and ultrastructure&#46; However&#44; this has never been conclusively proved the hypothesis currently gaining support is that the origin of these tumors is in pluripotent cells&#46; In our first case&#44; the tumor does appear to originate in the dermoepidermal junction and the origin could therefore be in the Merkel cells&#44; which are found in the basement layer of the epidermis and sometimes in the hair follicles&#46; In the second case&#44; however&#44; the tumor was found in the subcutaneous tissue and had no contact with adnexal epithelia&#44; which makes the possibility that it originated in Merkel cells remote&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">In our first case&#44; the absence of cytokeratin 20 in the tumor as shown by immunohistochemistry&#44; is also a rare finding in Merkel carcinoma&#46; Approximately 90&#37; of cases of Merkel cell carcinomas are positive for cytokeratin 20&#44; although a negative result for this marker in no way rules out a diagnosis of Merkel cell carcinoma&#46; In fact&#44; in the differential diagnosis between Merkel cell carcinoma and skin metastasis of a neuroendocrine tumor&#44; negative staining for TTF-1 is considered to be more specific to Merkel cell carcinoma than a positive result for cytokeratin 20 because only 3&#37; of systemic neuroendocrine tumors are negative for TTF-1 but Merkel cell carcinoma is practically never positive for this marker&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">In summary&#44; we report 2 cases of very early Merkel cell carcinoma that&#44; owing to the small size of the lesions&#44; probably requires less aggressive treatment than most of these tumors&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
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        0 => array:2 [
          "identificador" => "xres97839"
          "titulo" => "Abstract"
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          "identificador" => "xpalclavsec84995"
          "titulo" => "Keywords"
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        2 => array:2 [
          "identificador" => "xres97838"
          "titulo" => "Resumen"
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          "identificador" => "xpalclavsec84994"
          "titulo" => "Palabras clave"
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        4 => array:2 [
          "identificador" => "sec0005"
          "titulo" => "Introduction"
        ]
        5 => array:2 [
          "identificador" => "sec0010"
          "titulo" => "Case 1"
        ]
        6 => array:2 [
          "identificador" => "sec0015"
          "titulo" => "Case 2"
        ]
        7 => array:2 [
          "identificador" => "sec0020"
          "titulo" => "Discussion"
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        8 => array:2 [
          "identificador" => "sec0025"
          "titulo" => "Conflicts of Interest"
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        9 => array:1 [
          "titulo" => "References"
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    "pdfFichero" => "main.pdf"
    "tienePdf" => true
    "fechaRecibido" => "2011-07-11"
    "fechaAceptado" => "2011-10-29"
    "PalabrasClave" => array:2 [
      "en" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Keywords"
          "identificador" => "xpalclavsec84995"
          "palabras" => array:5 [
            0 => "Merkel cell carcinoma"
            1 => "Early"
            2 => "Incipient"
            3 => "Treatment"
            4 => "Baby Merckel"
          ]
        ]
      ]
      "es" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
          "titulo" => "Palabras clave"
          "identificador" => "xpalclavsec84994"
          "palabras" => array:5 [
            0 => "Tumor de Merkel"
            1 => "Temprano"
            2 => "Incipiente"
            3 => "Tratamiento"
            4 => "Baby Merckel"
          ]
        ]
      ]
    ]
    "tieneResumen" => true
    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Merkel cell carcinoma is a malignant skin tumor with a poor prognosis that primarily affects photoexposed areas of elderly patients&#46; Tumor size is a very strong prognostic factor&#44; with much better outcomes associated with small lesions&#44; measuring less than 1<span class="elsevierStyleHsp" style=""></span>cm&#46; However&#44; such lesions are rarely seen in the clinic in view of the rapid growth of this tumor&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report 2 cases of incipient Merkel cell carcinoma&#46; Both cases of incipient Merkel cell carcinoma measured approximately 5<span class="elsevierStyleHsp" style=""></span>mm in diameter&#46; One tumor was confined to the epidermis and papillary dermis on the nose of a 79-year-old man and the other was located in the deep dermis&#44; almost in the hypodermis&#44; on the buttock of an 82-year-old woman&#46; In both cases&#44; the lesions had appeared weeks earlier&#46; The first tumor seemed to originate in the dermoepidermal junction whereas the second originated almost in the hypodermis&#46; Although the lesions were at a similar disease stage and had a similar size&#44; their different locations within the dermis highlight once again the controversy about which cells give rise to Merkel cell carcinoma&#46; The precursor cells could feasibly be Merkel cells in the first case but not in the second&#46;</p>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">El tumor de Merkel es una neoplasia cut&#225;nea de mal pron&#243;stico que afecta preferentemente a pacientes ancianos en &#225;reas fotoexpuestas&#46; El tama&#241;o tumoral es un factor pron&#243;stico muy importante en este tumor&#44; de modo que las lesiones peque&#241;as&#44; menores de un cent&#237;metro&#44; tienen un comportamiento mucho mejor que el resto&#46; Sin embargo&#44; el crecimiento r&#225;pido de este tumor hace dif&#237;cil encontrar casos de peque&#241;o tama&#241;o&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Describimos dos pacientes con sendos tumores de Merkel incipientes&#44; de aproximadamente 5 mil&#237;metros cada uno&#44; uno de ellos en la nariz de un var&#243;n de 79 a&#241;os confinado a la epidermis y dermis papilar&#44; y el otro en la nalga de una mujer de 82 a&#241;os&#44; localizado en la dermis profunda&#44; casi en la hipodermis&#46; En ambos casos se trataba de lesiones de semanas de evoluci&#243;n&#44; y sin embargo el primer tumor parec&#237;a proceder de la uni&#243;n dermoepid&#233;rmica&#44; mientras que el segundo se iniciaba casi en la hipodermis&#46; Estos dos casos son similares en cuanto a tiempo de evoluci&#243;n y tama&#241;o&#44; pero opuestos en lo que respecta a su localizaci&#243;n dentro de la dermis&#44; por lo que ponen de manifiesto una vez m&#225;s lo controvertido de la c&#233;lula de origen del tumor de Merkel&#44; que se hipotetiza que sea la c&#233;lula de Merkel&#44; y que ser&#237;a factible en el primer caso&#44; pero no en el segundo&#46;</p>"
      ]
    ]
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      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; Requena C&#44; et al&#46; &#171;Baby Merkel&#187;&#58; tumor de Merkel incipiente&#59; a prop&#243;sito de 2 casos&#46; Actas Dermosifiliogr&#46;2013&#59; 104&#58;71-4&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Clusters of cells in the dermoepidermal junction and papillary dermis&#59; the clusters are composed of uniform round cells with large vesicular nuclei&#44; no evident nucleoli and some signs of mitosis &#40;hematoxylin-eosin&#44; original magnification x40&#44; x100&#44; x200&#44; x400&#41;&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Immunohistochemistry study of the first case&#44; showing positive staining for pancytokeratin&#44; synaptophysin&#44; and neuron-specific enolase&#44; and negative staining for thyroid transcription factor 1&#46;</p>"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">A and B&#44; Panoramic image of the second case&#44; with perivascular lymphocytic inflammatory infiltrates&#59; the infiltrates are light in the superficial and middle dermis&#44; and dense in the deep dermis&#46; C and D&#44; Detail of the infiltrate in the deep dermis&#44; showing round cells with large nuclei with loose chromatin&#44; and abundant mitosis accompanied by normal lymphocytes &#40;hematoxylin-eosin&#44; original magnification&#58; A and B&#44; x40&#59; C and D&#44; x400&#41;&#46;</p>"
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      "titulo" => "References"
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                      "titulo" => "A case of intraepidermal Merkel Cell carcinoma within squamous cell carcinoma in situ&#58; Merkel cell carcinoma in situ&#63;"
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                          "colaboracion" => "Rockville Merkel Cell Carcinoma Group"
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                      "titulo" => "Local control of primary Merkel cell carcinoma&#58; review of 45 cases treated with Mohs micrographic surgery with and without adjuvant radiation"
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                            1 => "J&#46;A&#46; Zitelli"
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Case Report
Incipient Merkel Cell Carcinoma: A Report of 2 Cases
«Baby Merkel»: tumor de Merkel incipiente; a propósito de 2 casos
C. Requenaa,
Autor para correspondencia
celiareq@hotmail.com

Corresponding author.
, V. Travesb, B. Llombarta, C. Guilléna
a Servicio de Dermatología, Instituto Valenciano de Oncología, Valencia, Spain
b Servicio de Anatomía Patológica, Instituto Valenciano de Oncología, Valencia, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Introduction</span><p id="par0005" class="elsevierStylePara elsevierViewall">Merkel cell carcinoma is a rare malignant neuroendocrine tumor with a poor prognosis&#46; It primarily affects photoexposed areas of skin in elderly patients&#46; Incidence is higher in immunodeficient patients and a probable etiologic role has recently been attributed to polyomavirus infection&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Tumor size is an essential prognostic factor in Merkel cell carcinoma and early diagnosis and early surgical resection are therefore very important&#46; The rapid rate at which these tumors normally grow means that they are rarely removed at a size of less than 1 centimeter&#46; Published cases of intraepidermal Merkel cell carcinoma are even more rare&#44; and most of them are diagnosed coincidentally on removal of another epidermoid or basal-cell carcinoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> We report 2 cases of incipient Merkel cell carcinoma not associated with another lesion&#59; both tumors had a diameter of less than 5<span class="elsevierStyleHsp" style=""></span>mm&#46; We discuss the most appropriate treatment in these cases of small Merkel cell carcinoma and highlight the distinctive histologic aspect and unusual location&#8212;the dermoepidermal junction in one case and the junction between the dermis and the subcutaneous tissue in the other case&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case 1</span><p id="par0015" class="elsevierStylePara elsevierViewall">The first case was a 79-year-old English patient with a history of gastric ulcer and occupational exposure to asbestos&#46; The patient was being followed up in our department because of a basal cell carcinoma located on the tip of the nose&#59; the tumor had been treated 2 years earlier with Mohs surgery and the site was reconstructed with a graft&#46; A routine review detected a lesion that had gone unnoticed by the patient&#44; an erythematous papule with a diameter of approximately 2<span class="elsevierStyleHsp" style=""></span>mm&#44; located on the right nostril&#46; Owing to a clinical suspicion of basal cell carcinoma a shave biopsy of the lesion was performed&#46; Pathology identified a tumor located in the dermoepidermal junction and papillary dermis&#59; the tumor consisted of clusters of monomorphic round basophilic cells with large vesicular nuclei&#44; no visible nucleoli&#44; and scattered signs of mitosis &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Immunohistochemistry showed the cells to be positive for pancytokeratin&#44; chromogranin A&#44; synaptophysin&#44; and neuron-specific enolase&#44; and negative for cytokeratin 20&#44; thyroid transcription factor 1 &#40;TTF-1&#41;&#44; and S-100 protein &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Once the tumor had been diagnosed as Merkel cell carcinoma&#44; treatment was completed using Mohs surgery to ensure complete excision of the malignant lesion&#59; the margin was negative after a single stage&#46; The staging study&#44; which included computed tomography &#40;CT&#41; of the chest&#44; abdomen&#44; and pelvis&#44; was negative for tumors&#59; the CT scan incidentally revealed calcified pleural plaques characteristic of asbestosis&#46; The patient was free of disease after 10 months of follow-up&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case 2</span><p id="par0025" class="elsevierStylePara elsevierViewall">The second patient was an 82-year-old woman with no relevant history&#44; who visited our department with a lesion that had appeared 2 weeks earlier on the left buttock&#46; Examination revealed a very small subcutaneous nodular lesion measuring less than a centimeter&#59; the lesion was completely nonspecific&#44; covered by normal skin&#44; and palpable but not visible&#46; A punch biopsy showed a proliferation of monomorphic round cells with large vesicular nuclei&#44; no nucleoli&#44; and abundant mitosis in the deep dermis&#44; almost reaching the hypodermis &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; This proliferation of cells was accompanied by an infiltrate of nontumorous lymphocytes&#46; Immune staining was positive for cytokeratin 20 and chromogranin A&#44; and negative for TTF-1&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">In this case&#44; the staging study included a CT scan of the chest&#44; abdomen&#44; and pelvis&#44; and positron emission tomography &#40;PET&#41;&#59; the results of all tests were negative&#46; Treatment was completed by extending the margin of excision by 2<span class="elsevierStyleHsp" style=""></span>cm in diameter and down to the fascia and studying the sentinel node&#46; Both the extended margin and the sentinel node were negative for tumors&#46; The patient was still free of disease after a year and a half of follow-up&#46;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0035" class="elsevierStylePara elsevierViewall">Management of patients with Merkel cell carcinoma is often complex&#46; The aggressive nature of the tumor and its low frequency make it difficult to establish a reliable and uniformly accepted management protocol&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> In general&#44; the most appropriate treatment is considered to be surgery plus radiation therapy of the surgical site&#44;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> accompanied&#44; when applicable&#44; by a study of the sentinel nodes or selective lymph node resection&#44; sometimes with radiation therapy of the lymph node drainage area&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> However&#44; the wide surgical excision recommended in the literature is not always possible&#44; and no specific recommendations exist regarding ideal surgical margins&#46; Mohs surgery is increasingly recommended for the treatment of Merkel cell carcinoma&#44; but this modality is not available in many hospitals&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> As the tumor is known to be sensitive to radiation&#44; the use of adjuvant radiation therapy after surgery would seem to be logical&#46; However&#44; in practice&#44; we should always be reticent about treating a very small tumor on the face of a patient &#40;as in our first case&#41; with aggressive initial surgery plus radiation therapy&#46; A study by Stokes et al&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> reviewed 346 cases of Merkel cell carcinoma&#44; with known lymph node staging &#40;either because the tumor had a sentinel node or because selective lymph node resection had been performed&#41; in 213 cases&#46; Those authors correlated lymph node staging with the size of the primary Merkel cell tumor and found that tumors measuring less than 1 centimeter were found in only 2 patients &#40;4&#37;&#41; with lymph node involvement&#46; Furthermore&#44; lymph node involvement in those 2 patients was not detected by means of the sentinel node&#44; as the enlarged nodes in these patients were clinically palpable&#46; The authors therefore concluded that the sentinel node technique is probably not justified in Merkel cell tumors with a diameter of less than 1 centimeter&#46; In our 2 cases&#44; the therapeutic decision was made prior to the publication of that study&#46; The notably small size of both tumors led us to discuss the most appropriate treatment in a tumor committee&#46; We decided to perform a sentinel node study in the second patient but not in the first patient&#44; and treated the tumors somewhat similarly to the way we manage cases of melanoma&#44; that is&#44; based on a pseudo-Breslow assessment of the tumors&#46; Thus&#44; the first case showed a Breslow depth of less than 1<span class="elsevierStyleHsp" style=""></span>mm&#44; whereas the second tumor&#44; which was almost in the hypodermis&#44; had a considerably greater Breslow depth&#46;</p><p id="par0040" class="elsevierStylePara elsevierViewall">The other reason we report these 2 cases is that the histologic appearance of the tumors led us to think about another aspect of Merkel cell carcinoma that is the subject of debate&#58; its phylogeny&#46; It has traditionally been accepted that this carcinoma originates in the Merkel cells&#44; based on the similarities in immunohistochemistry and ultrastructure&#46; However&#44; this has never been conclusively proved the hypothesis currently gaining support is that the origin of these tumors is in pluripotent cells&#46; In our first case&#44; the tumor does appear to originate in the dermoepidermal junction and the origin could therefore be in the Merkel cells&#44; which are found in the basement layer of the epidermis and sometimes in the hair follicles&#46; In the second case&#44; however&#44; the tumor was found in the subcutaneous tissue and had no contact with adnexal epithelia&#44; which makes the possibility that it originated in Merkel cells remote&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">In our first case&#44; the absence of cytokeratin 20 in the tumor as shown by immunohistochemistry&#44; is also a rare finding in Merkel carcinoma&#46; Approximately 90&#37; of cases of Merkel cell carcinomas are positive for cytokeratin 20&#44; although a negative result for this marker in no way rules out a diagnosis of Merkel cell carcinoma&#46; In fact&#44; in the differential diagnosis between Merkel cell carcinoma and skin metastasis of a neuroendocrine tumor&#44; negative staining for TTF-1 is considered to be more specific to Merkel cell carcinoma than a positive result for cytokeratin 20 because only 3&#37; of systemic neuroendocrine tumors are negative for TTF-1 but Merkel cell carcinoma is practically never positive for this marker&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">In summary&#44; we report 2 cases of very early Merkel cell carcinoma that&#44; owing to the small size of the lesions&#44; probably requires less aggressive treatment than most of these tumors&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of Interest</span><p id="par0055" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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        0 => array:2 [
          "identificador" => "xres97839"
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          "titulo" => "Keywords"
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          "titulo" => "Introduction"
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          "identificador" => "sec0010"
          "titulo" => "Case 1"
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        6 => array:2 [
          "identificador" => "sec0015"
          "titulo" => "Case 2"
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        7 => array:2 [
          "identificador" => "sec0020"
          "titulo" => "Discussion"
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        8 => array:2 [
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        9 => array:1 [
          "titulo" => "References"
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    "pdfFichero" => "main.pdf"
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    "fechaRecibido" => "2011-07-11"
    "fechaAceptado" => "2011-10-29"
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        0 => array:4 [
          "clase" => "keyword"
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            0 => "Merkel cell carcinoma"
            1 => "Early"
            2 => "Incipient"
            3 => "Treatment"
            4 => "Baby Merckel"
          ]
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      ]
      "es" => array:1 [
        0 => array:4 [
          "clase" => "keyword"
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          "palabras" => array:5 [
            0 => "Tumor de Merkel"
            1 => "Temprano"
            2 => "Incipiente"
            3 => "Tratamiento"
            4 => "Baby Merckel"
          ]
        ]
      ]
    ]
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    "resumen" => array:2 [
      "en" => array:2 [
        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Merkel cell carcinoma is a malignant skin tumor with a poor prognosis that primarily affects photoexposed areas of elderly patients&#46; Tumor size is a very strong prognostic factor&#44; with much better outcomes associated with small lesions&#44; measuring less than 1<span class="elsevierStyleHsp" style=""></span>cm&#46; However&#44; such lesions are rarely seen in the clinic in view of the rapid growth of this tumor&#46;</p><p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">We report 2 cases of incipient Merkel cell carcinoma&#46; Both cases of incipient Merkel cell carcinoma measured approximately 5<span class="elsevierStyleHsp" style=""></span>mm in diameter&#46; One tumor was confined to the epidermis and papillary dermis on the nose of a 79-year-old man and the other was located in the deep dermis&#44; almost in the hypodermis&#44; on the buttock of an 82-year-old woman&#46; In both cases&#44; the lesions had appeared weeks earlier&#46; The first tumor seemed to originate in the dermoepidermal junction whereas the second originated almost in the hypodermis&#46; Although the lesions were at a similar disease stage and had a similar size&#44; their different locations within the dermis highlight once again the controversy about which cells give rise to Merkel cell carcinoma&#46; The precursor cells could feasibly be Merkel cells in the first case but not in the second&#46;</p>"
      ]
      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">El tumor de Merkel es una neoplasia cut&#225;nea de mal pron&#243;stico que afecta preferentemente a pacientes ancianos en &#225;reas fotoexpuestas&#46; El tama&#241;o tumoral es un factor pron&#243;stico muy importante en este tumor&#44; de modo que las lesiones peque&#241;as&#44; menores de un cent&#237;metro&#44; tienen un comportamiento mucho mejor que el resto&#46; Sin embargo&#44; el crecimiento r&#225;pido de este tumor hace dif&#237;cil encontrar casos de peque&#241;o tama&#241;o&#46;</p><p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Describimos dos pacientes con sendos tumores de Merkel incipientes&#44; de aproximadamente 5 mil&#237;metros cada uno&#44; uno de ellos en la nariz de un var&#243;n de 79 a&#241;os confinado a la epidermis y dermis papilar&#44; y el otro en la nalga de una mujer de 82 a&#241;os&#44; localizado en la dermis profunda&#44; casi en la hipodermis&#46; En ambos casos se trataba de lesiones de semanas de evoluci&#243;n&#44; y sin embargo el primer tumor parec&#237;a proceder de la uni&#243;n dermoepid&#233;rmica&#44; mientras que el segundo se iniciaba casi en la hipodermis&#46; Estos dos casos son similares en cuanto a tiempo de evoluci&#243;n y tama&#241;o&#44; pero opuestos en lo que respecta a su localizaci&#243;n dentro de la dermis&#44; por lo que ponen de manifiesto una vez m&#225;s lo controvertido de la c&#233;lula de origen del tumor de Merkel&#44; que se hipotetiza que sea la c&#233;lula de Merkel&#44; y que ser&#237;a factible en el primer caso&#44; pero no en el segundo&#46;</p>"
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      0 => array:2 [
        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; Requena C&#44; et al&#46; &#171;Baby Merkel&#187;&#58; tumor de Merkel incipiente&#59; a prop&#243;sito de 2 casos&#46; Actas Dermosifiliogr&#46;2013&#59; 104&#58;71-4&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">Clusters of cells in the dermoepidermal junction and papillary dermis&#59; the clusters are composed of uniform round cells with large vesicular nuclei&#44; no evident nucleoli and some signs of mitosis &#40;hematoxylin-eosin&#44; original magnification x40&#44; x100&#44; x200&#44; x400&#41;&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Immunohistochemistry study of the first case&#44; showing positive staining for pancytokeratin&#44; synaptophysin&#44; and neuron-specific enolase&#44; and negative staining for thyroid transcription factor 1&#46;</p>"
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          "en" => "<p id="spar0035" class="elsevierStyleSimplePara elsevierViewall">A and B&#44; Panoramic image of the second case&#44; with perivascular lymphocytic inflammatory infiltrates&#59; the infiltrates are light in the superficial and middle dermis&#44; and dense in the deep dermis&#46; C and D&#44; Detail of the infiltrate in the deep dermis&#44; showing round cells with large nuclei with loose chromatin&#44; and abundant mitosis accompanied by normal lymphocytes &#40;hematoxylin-eosin&#44; original magnification&#58; A and B&#44; x40&#59; C and D&#44; x400&#41;&#46;</p>"
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      "titulo" => "References"
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            0 => array:3 [
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              "etiqueta" => "1"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Clonal integration of polyomavirus in human Merkel cell carcinoma"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:4 [
                            0 => "H&#46; Feng"
                            1 => "M&#46; Shuda"
                            2 => "Y&#46; Chang"
                            3 => "P&#46;S&#46; Moore"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1126/science.1152586"
                      "Revista" => array:6 [
                        "tituloSerie" => "Science"
                        "fecha" => "2008"
                        "volumen" => "319"
                        "paginaInicial" => "1096"
                        "paginaFinal" => "1100"
                        "link" => array:1 [
                          0 => array:2 [
                            "url" => "https://www.ncbi.nlm.nih.gov/pubmed/18202256"
                            "web" => "Medline"
                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
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            1 => array:3 [
              "identificador" => "bib0010"
              "etiqueta" => "2"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Frequent detection of Merkel cell polyomavirus in human Merkel cell carcinomas and identification of a unique deletion in the VP1 gene"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
                          "autores" => array:6 [
                            0 => "A&#46; Kasemm"
                            1 => "A&#46; Sch&#246;pflin"
                            2 => "C&#46; D&#237;az"
                            3 => "W&#46; Weyers"
                            4 => "E&#46; Stickeler"
                            5 => "M&#46; Werner"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1158/0008-5472.CAN-08-0949"
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                        "fecha" => "2008"
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                          ]
                        ]
                      ]
                    ]
                  ]
                ]
              ]
            ]
            2 => array:3 [
              "identificador" => "bib0015"
              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Merkel cell carcinoma in situ"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:3 [
                            0 => "T&#46; Ferringer"
                            1 => "H&#46;C&#46; Rogers"
                            2 => "J&#46;S&#46; Metcalf"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
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            ]
            3 => array:3 [
              "identificador" => "bib0020"
              "etiqueta" => "4"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "A case of intraepidermal Merkel Cell carcinoma within squamous cell carcinoma in situ&#58; Merkel cell carcinoma in situ&#63;"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "D&#46;F&#46; Al-Ahmadie Mutasim"
                            1 => "G&#46;K&#46; Mutema"
                          ]
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                  ]
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            4 => array:3 [
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                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Merkel cell carcinoma&#58; recent progress and current priorities on etiology&#44; pathogenesis and clinical management"
                      "autores" => array:1 [
                        0 => array:2 [
                          "colaboracion" => "Rockville Merkel Cell Carcinoma Group"
                          "etal" => false
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:1 [
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                        "fecha" => "2009"
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            5 => array:3 [
              "identificador" => "bib0030"
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              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Management of Merkel tumours&#58; an evidence-based review"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => false
                          "autores" => array:2 [
                            0 => "S&#46; Henness"
                            1 => "P&#46; Verrecken"
                          ]
                        ]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1097/CCO.0b013e3282fe6ad8"
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                        "fecha" => "2008"
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            6 => array:3 [
              "identificador" => "bib0035"
              "etiqueta" => "7"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Merkel cell carcinoma&#58; critical review with guidelines for multidisciplinary management"
                      "autores" => array:1 [
                        0 => array:2 [
                          "etal" => true
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Información del artículo
ISSN: 15782190
Idioma original: Inglés
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