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The lesion was of stony-hard consistency and infiltrated down to deep layers&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Biopsy was performed and sections were obtained for hematoxylin-eosin staining and immunohistochemical &#40;IHC&#41; staining&#46; Infiltration of the deep dermis and the subcutaneous cell tissue by small&#44; uniform&#44; cells was observed&#46; These cells were arranged in a trabecular structure between a myxoid stroma &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46; At higher magnification&#44; a vesicular nucleus was observed in these cells with a high mitotic index&#46; Large areas of necrosis were observed with abundant apoptotic bodies&#46; IHC study was positive for cytokeratin 20 &#40;CK20&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#44; chromogranin&#44; synaptophysin&#44; and CD56&#46; Focal positivity for myogenin and desmin was also observed&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Magnetic resonance imaging was performed and involvement of the deep layers was ruled out&#46; An oncologic positron emission tomography-computed tomography scan did not detect any evidence of disease spread&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">&#191;What was the diagnosis&#63;</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Merkel cell carcinoma &#40;MCC&#41; with rhabdomyoblastic differentiation&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">The lesions were completely excised with safety margins&#46; Ipsilateral lymph node dissection was performed&#46; Radiotherapy of the tumor bed and adjuvant chemotherapy were also administered&#46; The patient is currently free of disease 1 year after diagnosis&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">MCC is an uncommon primary neuroendocrine carcinoma&#46; It usually appears in patients with a low phototype&#44; elderly patients&#44; immunodepressed patients&#44; and on sun-exposed areas&#44; although it has also been reported at sites such as the crotch or the buttocks&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2</span></a> The most typical presentation is in the form of a rapidly-growing firm nodule with a red-violaceous color&#44; but cases have also been reported with lesions in the form of panniculitis-like subcutaneous plaque&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2</span></a> Aberrant differentiation of MCC is uncommon&#46; The documented subtypes are eccrine&#44; squamous&#44; rhabdomyoblastic&#44; sarcomatous&#44; and leiomyosarcomatous&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In the pathology study&#44; MCC with rhabdomyoblastic differentiation presents as a small cell tumor that infiltrates down to the subcutaneous tissue&#46; IHC study of the tumor shows results characteristic of neuroendocrine tumors consistent with MCC &#40;QAE 1&#47;2&#44; CK20&#44; chromogranin&#44; synaptophysin&#44; and CD56 positivity&#41;&#44; but focal positivity for myogenin and desmin is also present&#44; thus showing the presence of rhabdomyoblastic differentiation&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> The immunophenotype enables differential diagnosis to be established with other types of tumor such as primary cutaneous Ewing sarcoma &#40;negative for CK20&#44; chromogranin&#44; and synaptophysin stains&#41;&#46; Although the sarcoma always shows positivity for CD99&#44; it should be remembered that up to 30&#37; of MCC are also positive&#46; Alveolar rhabdomyosarcoma &#40;negative for CK&#44; synaptophysin&#44; and chromogranin&#41;&#44; metastasis of small cell lung cancer &#40;negative for CK20 and positive for TFF-1&#41;&#44; and malignant amelanotic melanoma with rhabdomyoblastic differentiation &#40;positive for stains characteristic of melanoma&#41; should also be included in the differential diagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;5</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Currently&#44; there is discussion about the association between MCC and Merkel cell polyomavirus &#40;MCP&#41;&#46; In 2008&#44; Feng et al&#46; isolated MCP in 8 of 10 samples from patients with typical MCC&#46; However&#44; in 2013&#44; Martin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> presented a series of 12 patients with MCC with divergent differentiation in which MCP was not isolated in any samples&#44; bringing into question the association&#44; at least in MCC with divergent differentiation&#46; In our case&#44; the determination for MCP was also negative&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Treatment consists of excision with safety margins&#44; lymph node dissection&#44; and radiotherapy and chemotherapy&#46; The general prognosis for MCC is poor&#44; with 70&#37; survival at 5 years&#46; This survival rate is lower still for aberrant types&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">In conclusion&#44; we present the first case in an immunocompetent patient&#44; with no evidence of actinic damage&#44; who developed MCC with rhabdomyoblastic differentiation and who was negative for MCP&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Case for Diagnosis
Stony-Hard Subcutaneous Mass on the Arm of a Young Woman
Masa subcutánea pétrea en el brazo de una mujer joven
E. Agut-Busquet
Corresponding author
eagutbusquet@gmail.com

Corresponding author.
, M. Yébenes, J. Luelmo
Servicio de Dermatología, Hospital de Sabadell, Sabadell, Barcelona, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Clinical Characteristics</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 31-year-old woman&#44; with skin phototype <span class="elsevierStyleSmallCaps">IV</span> and no relevant personal history&#44; presented in our clinic with a painful progressively growing lesion that first appeared 1 year earlier&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Of note in the physical examination was the presence of a well-defined subcutaneous mass measuring 3<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>2&#46;5<span class="elsevierStyleHsp" style=""></span>cm &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Figure 1</a>&#41;&#46; The lesion was of stony-hard consistency and infiltrated down to deep layers&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Biopsy was performed and sections were obtained for hematoxylin-eosin staining and immunohistochemical &#40;IHC&#41; staining&#46; Infiltration of the deep dermis and the subcutaneous cell tissue by small&#44; uniform&#44; cells was observed&#46; These cells were arranged in a trabecular structure between a myxoid stroma &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Figure 2</a>&#41;&#46; At higher magnification&#44; a vesicular nucleus was observed in these cells with a high mitotic index&#46; Large areas of necrosis were observed with abundant apoptotic bodies&#46; IHC study was positive for cytokeratin 20 &#40;CK20&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Figure 3</a>&#41;&#44; chromogranin&#44; synaptophysin&#44; and CD56&#46; Focal positivity for myogenin and desmin was also observed&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Magnetic resonance imaging was performed and involvement of the deep layers was ruled out&#46; An oncologic positron emission tomography-computed tomography scan did not detect any evidence of disease spread&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">&#191;What was the diagnosis&#63;</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Merkel cell carcinoma &#40;MCC&#41; with rhabdomyoblastic differentiation&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">The lesions were completely excised with safety margins&#46; Ipsilateral lymph node dissection was performed&#46; Radiotherapy of the tumor bed and adjuvant chemotherapy were also administered&#46; The patient is currently free of disease 1 year after diagnosis&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">MCC is an uncommon primary neuroendocrine carcinoma&#46; It usually appears in patients with a low phototype&#44; elderly patients&#44; immunodepressed patients&#44; and on sun-exposed areas&#44; although it has also been reported at sites such as the crotch or the buttocks&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2</span></a> The most typical presentation is in the form of a rapidly-growing firm nodule with a red-violaceous color&#44; but cases have also been reported with lesions in the form of panniculitis-like subcutaneous plaque&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2</span></a> Aberrant differentiation of MCC is uncommon&#46; The documented subtypes are eccrine&#44; squamous&#44; rhabdomyoblastic&#44; sarcomatous&#44; and leiomyosarcomatous&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">In the pathology study&#44; MCC with rhabdomyoblastic differentiation presents as a small cell tumor that infiltrates down to the subcutaneous tissue&#46; IHC study of the tumor shows results characteristic of neuroendocrine tumors consistent with MCC &#40;QAE 1&#47;2&#44; CK20&#44; chromogranin&#44; synaptophysin&#44; and CD56 positivity&#41;&#44; but focal positivity for myogenin and desmin is also present&#44; thus showing the presence of rhabdomyoblastic differentiation&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">3</span></a> The immunophenotype enables differential diagnosis to be established with other types of tumor such as primary cutaneous Ewing sarcoma &#40;negative for CK20&#44; chromogranin&#44; and synaptophysin stains&#41;&#46; Although the sarcoma always shows positivity for CD99&#44; it should be remembered that up to 30&#37; of MCC are also positive&#46; Alveolar rhabdomyosarcoma &#40;negative for CK&#44; synaptophysin&#44; and chromogranin&#41;&#44; metastasis of small cell lung cancer &#40;negative for CK20 and positive for TFF-1&#41;&#44; and malignant amelanotic melanoma with rhabdomyoblastic differentiation &#40;positive for stains characteristic of melanoma&#41; should also be included in the differential diagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;5</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Currently&#44; there is discussion about the association between MCC and Merkel cell polyomavirus &#40;MCP&#41;&#46; In 2008&#44; Feng et al&#46; isolated MCP in 8 of 10 samples from patients with typical MCC&#46; However&#44; in 2013&#44; Martin et al&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">6</span></a> presented a series of 12 patients with MCC with divergent differentiation in which MCP was not isolated in any samples&#44; bringing into question the association&#44; at least in MCC with divergent differentiation&#46; In our case&#44; the determination for MCP was also negative&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Treatment consists of excision with safety margins&#44; lymph node dissection&#44; and radiotherapy and chemotherapy&#46; The general prognosis for MCC is poor&#44; with 70&#37; survival at 5 years&#46; This survival rate is lower still for aberrant types&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">In conclusion&#44; we present the first case in an immunocompetent patient&#44; with no evidence of actinic damage&#44; who developed MCC with rhabdomyoblastic differentiation and who was negative for MCP&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Conflicts of Interest</span><p id="par0065" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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Article information
ISSN: 15782190
Original language: English
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2017 March 30 10 40
2017 February 38 6 44
2017 January 26 4 30
2016 December 50 8 58
2016 November 56 15 71
2016 October 41 13 54
2016 May 0 2 2
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¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?