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During the operation&#44; the tumor appeared to be of variable consistency&#44; with soft areas of myxoid appearance and no invasion of the underlying tendons&#46; Six months after excision&#44; there had been no recurrence&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathologic Study</span><p id="par0025" class="elsevierStylePara elsevierViewall">The material from the incisional biopsy and the surgical excision were both fixed in 10&#37; buffered formalin and embedded in their entirety in paraffin&#46; Sections 3<span class="elsevierStyleHsp" style=""></span>&#956;m thick were taken and stained with hematoxylin-eosin&#46; Subsequently&#44; immunohistochemical staining was performed with the following antibodies using the avidin-biotin complex method and with positive and negative controls&#58; CD34 &#40;DAKO&#44; 1&#58;50&#41;&#44; protein S-100 &#40;DAKO&#44; 1&#58;5000&#41;&#44; factor XIIIa &#40;Behring&#44; 1&#58;1000&#41;&#44; desmin &#40;DAKO&#44; 1&#58;100&#41;&#44; actin 1A4 &#40;DAKO&#44; 1&#58;50&#41;&#44; Ki-67 &#40;DAKO&#44; 1&#58;300&#41;&#44; epithelial membrane antigen &#40;EMA&#41; &#40;DAKO&#44; 1&#58;100&#41;&#44; and B-cell lymphoma &#40;bcl-2&#41; &#40;DAKO&#44; 1&#58;80&#41;&#46; A fluorescence in situ hybridization &#40;FISH&#41; analysis was performed on the paraffin-embedded tumor tissue to detect the translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41;&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">The incisional biopsy showed diffuse infiltration of the reticular dermis by a neoformation consisting of short bundles of spindle cells interlaced in different directions and an evident Grenz zone&#46; The tumor engulfed the skin appendages but neither invaded nor destroyed them &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The proliferative cells had an eosinophilic cytoplasm with blurred edges and elongated oval nuclei showing fine chromatin and no nucleoli&#46; Only 2 nonatypical mitotic figures were identified per 10 high-magnification fields&#46; Cellularity was less marked in the deep part of the lesion&#44; which had stromal areas with a myxoid appearance&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The excised tissue consisted of an ellipse of skin with a nodule measuring 2&#46;4<span class="elsevierStyleHsp" style=""></span>cm along the longest axis&#46; Histologically&#44; it comprised a mesenchymal neoformation with a diffuse growth pattern and infiltrative borders&#46; The tumor occupied the entire reticular dermis and extended to the subcutaneous cell tissue&#46; It displayed a honeycomb pattern&#44; in which individual adipocytes were isolated &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; However&#44; 90&#37; of the neoformation comprised hypocellular areas with a myxoid appearance&#46; The cells that were present were fusiform and stellate&#44; without atypia or mitosis&#46; Thin-walled capillaries were seen in abundance and inflammatory cells were few&#46; Focally&#44; a dense fibrous stroma was present&#46; At the edge of the lesion&#44; both in the superficial and deep layers&#44; there were focal areas with the same characteristics as those seen in the incisional biopsy material&#46; The surgical margins at the sides and bottom were extensively involved&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Staining with colloidal iron was observed &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; On immunohistochemical staining&#44; cytoplasmic expression of CD34 was strong in the areas of the lesion with a classic pattern but weak in the myxoid areas&#46; The other markers used&#8212;protein S-100&#44; factor XIIIa&#44; actin 1A4&#44; EMA&#44; and bcl-2&#8212;were negative&#46; The Ki-67 cell proliferation index was 5&#37; in areas with the classic pattern and 20&#37; in myxoid areas&#46; Diagnosis of dermatofibrosarcoma protuberans was confirmed on demonstrating translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41; by FISH&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">The myxoid subtype is a very rare dermatofibrosarcoma protuberans variant first described in 1983 by Frierson et al&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> In one of the largest published series of dermatofibrosarcoma protuberans&#44; only 4&#46;3&#37; of the lesions were of this type&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Myxoid lesions differ from the classic variant by presenting mainly on the limbs&#59; the next most frequent sites are the head and neck&#44; followed by the trunk and the anogenital region&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Myxoid dermatofibrosarcoma protuberans is very rare in children&#46; Only 3 childhood cases have been reported&#59; these occurred on the eyelid&#44; scalp&#44; and elbow&#44; and all were in boys&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> We report the first case of myxoid dermatofibrosarcoma protuberans on the finger of a girl&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Clinically&#44; the lesion presented as a nodular dermal or subcutaneous&#44; firm&#44; raised mass that was slow-growing and progressive&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> The most common potential diagnoses for such a lesion are epidermal&#44; mucinous&#44; or synovial cyst&#59; lipoma&#59; pyogenic granuloma&#59; and lymphoma&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> In view of the site&#44; our case was initially considered to be a giant-cell tumor of the tendon sheath&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Some authors suggest that the myxoid variant has a slightly better prognosis than the classic dermatofibrosarcoma protuberans variant&#44; although others disagree&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> No metastases were reported in a recent series of 23 cases of myxoid dermatofibrosarcoma protuberans&#46; In that same series&#44; 2 patients had local recurrence&#44; one after incomplete resection and the other after leaving margins less than 2<span class="elsevierStyleHsp" style=""></span>mm&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Metastases have not been reported in children&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> In our patient&#44; the lesion had grown slowly and progressively for more than 10 years&#46; There was no recurrence or metastasis 6 months after excision&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">Microscopically&#44; the tumor is located in the reticular dermis and subcutaneous cell tissue and shows a Grenz zone&#44; although it occasionally affects the epidermis and forms an ulcer&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The areas with myxoid&#44; basophilic&#44; or pale stroma predominate&#44; due to the high hyaluronan content&#44; as indicated by staining with colloidal iron&#46; It is likely that the myxoid transformation is due to an increased synthesis of glycosaminoglycans&#44; which inhibit polymerization of the collagen&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> It is not uncommon for traditional dermatofibrosarcoma protuberans to have focal areas with a myxoid appearance&#44; particularly in recurrence&#44; although the presence of such areas does not necessarily point to a myxoid variant&#46; The myxoid subtype is diagnosed when more than 50&#37; of the stroma of the lesion is myxoid&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;8</span></a> In the present case&#44; 70&#37; of the tumor was myxoid&#46; A myxoid stroma has few nonatypical cells&#46; These cells have a fusiform and stellate morphology&#44; with mildly eosinophilic or pale cytoplasm&#44; undefined borders&#44; oval nuclei with fine chromatin and no nucleoli&#44; and occasional or no mitotic figures&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5</span></a> In addition&#44; the tumor contains randomly distributed fine-walled capillaries&#44; which rarely contain mast cells&#46; As in a Bernard tumor&#44; pigmented dendritic cells can also be identified&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5&#44;9</span></a> One of the keys to diagnosis is the presence of areas with the characteristic features of classic dermatofibrosarcoma protuberans at the edge of the lesions&#59; these features are spindle cells arranged in interlaced strands with scant collagen between them and wagon-wheel areas&#46; The lesion engulfs skin appendages without invading them&#44; and on invading subcutaneous adipose tissue&#44; a characteristic honeycomb pattern is formed when individual adipocytes are isolated&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3&#44;5&#44;8&#44;9</span></a> In our case&#44; 30&#37; of the tumor had the features of the classic variant and there were areas at the edge of the lesion in which invasion of the underlying fatty tissue had occurred&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">The most specific immunohistochemical marker is CD34&#44; for which tumors show strong diffuse staining in 50&#37; to 100&#37; of cases&#59; the sensitivity of CD34 positivity is 84&#37; to 100&#37;&#46; In myxoid and fibrosarcomatous areas&#44; staining is weak and focal&#44; or negative&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5&#44;6</span></a> In our case&#44; the lesion showed diffuse positive staining which was stronger in areas of low cellularity&#46; In addition&#44; these tumors are positive for vimentin and CD99&#46; Some are focally positive for factor XIIIa&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> EMA expression might occur in myxoid areas&#44; possibly indicating perineural differentiation&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> but S-100&#44; actin&#44; and desmin are not expressed&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Ki-67 cell proliferation indices have been reported to be greater in myxoid areas &#40;19&#46;8&#37;&#41; and fibrosarcomatous areas &#40;11&#46;8&#37;&#41; than in areas with classic histology &#40;2&#46;2&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> In fact&#44; in our case&#44; the Ki-67 proliferative index in myxoid areas was 20&#37; and as low as 5&#37; in the areas of highest cellularity&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">Dermatofibrosarcoma protuberans is associated with reciprocal translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41; or a supernumerary ring chromosome r&#40;17&#59;22&#41;&#46; Both give rise to a chimeric gene <span class="elsevierStyleItalic">COL1A1-PDGFB</span> through fusion of the collagen type 1&#945; gene on chromosome 17 and the platelet growth factor-&#946; chain gene on chromosome 22&#46; This leads to continual stimulation of cell growth&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;6</span></a> Translocation is found most frequently in children&#44; whereas the ring chromosome predominates in adults&#44; possibly indicating that this change is a late event in the pathogenesis of dermatofibrosarcoma protuberans&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> This genetic abnormality has also been found in the myxoid variant and in giant-cell fibroblastoma&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Currently&#44; treatment of dermatofibrosarcoma protuberans with selective tyrosine kinase inhibitors is reserved for unresectable disease&#44; recurrences&#44; or metastatic disease&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> A positive immunoreaction with the dermatofibrosarcoma protuberans receptor &#40;PDGFR&#41; B antibody indicates constitutional activation of PDGFR&#44; thereby providing an alternative indirect method for confirming the presence of the dysregulated <span class="elsevierStyleItalic">PDGF</span> gene involved in this translocation&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Faced with a cutaneous&#47;subcutaneous lesion with predominantly myxoid areas and staining for CD34&#44; the pathologist should consider differential diagnosis with benign and malignant mesenchymal lesions with different biological behaviors and different treatment approaches&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> especially when only superficial or small biopsy samples are available&#46; The presence of classic areas of dermatofibrosarcoma protuberans&#44; even if focal&#44; is usually of great help in the diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> In the present case&#44; incisional biopsy showed classic areas of dermatofibrosarcoma protuberans&#44; which helped the initial diagnosis&#46; However&#44; this diagnosis had to be reconsidered when the excised specimen was found to be predominantly of myxoid type&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">The main differential diagnosis in this case is superficial acral fibromyxoma&#44; which usually affects patients between 14 and 72 years of age and grows for between 3 months and 30 years as a solitary mass that reaches a size of 0&#46;6 to 5 <span class="elsevierStyleHsp" style=""></span>cm across&#46; Acral fibromyxoma has a predilection for fingers and toes and tends to involve the nails&#46; Recurrences are frequent after partial resections&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> Both tumors are located in the dermis and subcutaneous tissue and are composed of stellate and spindle cells&#44; have a fascicular growth pattern which can either be random or form a wagon-wheel pattern in a myxoid or collagenous matrix&#46; Both tumors have moderate vascularization&#44; slight atypia&#44; and limited mytosis&#46; Both also express CD34&#44; EMA&#44; and CD99&#44; and are negative for S-100&#44; actin&#44; desmin&#44; keratin&#44; and HMB-45&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;12</span></a> Clinically&#44; our case appears to correspond more to a superficial acral fibromyxoma&#46; However&#44; subcutaneous tissue infiltration with a honeycomb pattern&#44; the presence of translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41; in the FISH analysis&#44; and the absence of giant multinucleated cells that are present in superficial acral fibromyxoma ruled out this possibility&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;12</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Other tumors such as cellular digital fibroma can be readily ruled out because clinically they present as a papule and not as a lobulated mass and are rare in children&#44; and a predominantly myxoid histology has not been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> Cellular digital fibromas are positive for CD34 and factor XIIIa&#44; whereas our patient&#39;s tumor was negative for these antibodies&#46; According to some&#44; a cellular digital fibroma can be thought of as a cellular variant of a superficial acral fibromyxoma&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Superficial angiomyxoma is another tumor that often recurs locally&#44; although it does not metastasize&#46; It grows slowly and is located in subcutaneous tissue&#44; although it can extend to the dermis&#46; It follows a lobulated growth pattern&#44; however&#44; without the diffuse honeycomb infiltation<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> that was present in our case&#46; A superficial angiomyxoma is composed of spindle and stellate cells in an abundant and highly vascularized basophilic stroma&#46; Neutrophils surround thin-walled vascular structures and epithelial elements &#40;epithelial cords or keratin cysts&#41; are observed in up to half the cases&#59; none of these features were observed in our case&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> These tumors are also positive for CD34&#44; vimentin&#44; and actin&#44; and negative for desmin&#44; S-100&#44; and factor XIIIa&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Our lesion was negative for actin&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Giant cell fibroblastoma is a tumor that affects children&#44; with a predilection for boys&#46; Its morphologic spectrum overlaps that of dermatofibrosarcoma protuberans&#44; and the 2 tumors share a histologic&#44; immunohistochemical&#44; and genetic profile&#46; Areas of giant cell fibroblastoma are found in almost 15&#37; of cases of dermatofibrosarcoma protuberans&#46; These mixed tumors affect the dermis and subcutaneous cellular tissue&#44; engulfing the appendages&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;6</span></a> The lesions are hypocellular&#44; with loose bundles of wavy spindle cells in an abundant highly-vascularized collagenous or myxoid stroma&#46; They have a characteristic cell population of pleomorphic cells with a single nucleus or giant multinucleated cells &#40;absent in our case&#41;&#44; are positive for CD34&#44; and have translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41;&#46; They can recur as dermatofibrosarcoma protuberans and vice versa&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Extraneural spindle cell perineuriomas may have a prominent myxoid stroma&#44; but these tumors have well defined borders &#40;whereas the border in our case was infiltrative&#41; and are composed of spindle cells arranged in a wagon wheel pattern&#44; sheets&#44; or bundles&#46; Half the cases are positive for CD34&#44; but they are also positive for perineural markers such as Glut-1 and EMA&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The lesion we describe was negative for these markers&#46; Finally&#44; we note that although solitary fibrous tumors are also positive for CD34 and CD99 and can have a prominent myxoid stroma&#44; they are well delimited&#44; express bcl-2&#44; and have a characteristic hemangiopericytoma-like vascular pattern&#44; unlike the lesion in our case&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of Interest</span><p id="par0105" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Dermatofibrosarcoma protuberans is a locally aggressive skin tumor that affects young and middle-aged adults&#46; A number of histological variants have been described&#44; the myxoid type being one of the least common&#46; Microscopically it is formed of a neoplastic growth that is located in the dermis and hypodermis and has a predominant myxoid component&#46; Peripherally there are infiltrating bundles of spindle-shaped cells that are diffusely positive for the CD34 immunohistochemical marker&#46; We report a case of myxoid dermatofibrosarcoma protuberans on a finger of the left hand of a 14-year-old girl&#46; The tumor had been present for at least 10 years&#46; This is the first pediatric case of myxoid dermatofibrosarcoma protuberans at this site&#46; This histological subtype has mainly been described on the extremities in adults and is very rare in children&#46; We discuss the differential diagnosis with other CD34<span class="elsevierStyleSup">&#43;</span> myxoid mesenchymal tumors&#46;</p>"
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        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El dermatofibrosarcoma protuberans &#40;DFSP&#41; es un tumor cut&#225;neo&#44; localmente agresivo&#44; que afecta a adultos j&#243;venes o de edad media&#46; Se han descrito diferentes formas histol&#243;gicas&#44; siendo la mixoide una de las m&#225;s infrecuentes&#46; Microsc&#243;picamente est&#225; constituido por una neoformaci&#243;n que ocupa la dermis e hipodermis&#44; de predominio mixoide&#44; con &#225;reas perif&#233;ricas conformadas por haces de c&#233;lulas fusiformes&#44; de crecimiento infiltrativo&#44; que expresan el marcador inmunohistoqu&#237;mico CD34 de forma difusa&#46; Presentamos por primera vez un DFSP mixoide en un dedo de la mano izquierda de una ni&#241;a de 14 a&#241;os&#44; de m&#225;s de 10 a&#241;os de evoluci&#243;n&#46; Se trata del primer caso infantil de DFSP mixoide en dicha localizaci&#243;n&#46; Este subtipo histol&#243;gico se ha descrito fundamentalmente en las extremidades de adultos&#44; siendo excepcional en ni&#241;os&#46; Se comenta el diagn&#243;stico diferencial con otros tumores mesenquimales mixoides CD34 positivos&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; Campos M&#44; et al&#46; Dermatofibrosarcoma protuberans mixoide infantile&#46; Actas Dermosifiliogr&#46;2012&#59;103&#58;422-6&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Clinical appearance of the lesion&#58; a skin-colored nodule measuring 2&#46;5<span class="elsevierStyleHsp" style=""></span>cm on the middle phalanx of the middle finger of the left hand&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Low-magnification image showing a spindle-cell neoformation which has diffusely infiltrated the reticular dermis and part of the subcutaneous tissue &#40;hematoxylin-eosin&#44; original magnification &#215;40&#41;&#46;</p>"
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The lesion has extensive myxoid areas&#44; as well as a subcutaneous cellular infiltrate in a honeycomb pattern &#40;hematoxylin-eosin&#44; original magnification &#215;100&#41;&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Strong positive staining with colloidal iron in myxoid areas &#40;colloidal iron&#44; original magnification &#215;100&#41;&#46;</p>"
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Case Report
Myxoid Dermatofibrosarcoma Protuberans in Childhood
Dermatofibrosarcoma protuberans mixoide infantil
M. Camposa, C. Zarcob, F. Acquadroc, E. Riveiro-Falkenbacha, J.L. Rodríguez-Peraltoa,
Corresponding author
a Servicio de Anatomía Patológica, Hospital Universitario 12 de Octubre, Madrid, Spain
b Servicio de Dermatología, Hospital Universitario 12 de Octubre, Madrid, Spain
c Grupo de Citogenética Molecular, CNIO, Madrid, 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">Dermatofibrosarcoma protuberans is a mesenchymal tumor with fibroblast and myofibroblast differentiation that occurs in the dermis and subcutaneous tissue of young or middle-aged adults&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> Incidence peaks in individuals in their 30s&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> The tumor grows slowly&#44; is locally aggressive and of intermediate malignancy&#44; and recurs in more than a third of cases&#46; Metastasis is rare but spread is usually hematogenous when it does occur&#46; Between 10&#37; and 15&#37; of recurrences are found in areas of fibrosarcoma&#44; neurofibrosarcoma&#44; or myxofibrosarcoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#8211;5</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Different types of dermatofibrosarcoma protuberans have been described&#44; including the classic&#44; pigmented &#40;Bernard tumor&#41;&#44; fibrosarcomatous&#44; granular cell&#44; flat atrophic&#44; and myxoid variants&#44; as well as variants with myogenic differentiation&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;2</span></a> The fibrosarcomatous subtype is associated with a worse prognosis&#44; a risk of metastasis of 10&#37; to 15&#37;&#44; and a rate of tumor-related death of 5&#46;8&#37;&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;7</span></a> One of the least common types is the myxoid variant&#44; which presents on the limbs&#44; head&#44; neck&#44; and trunk in adults&#46; It is very rare in children and only 3 cases have been reported in the literature&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> We present the first report of myxoid dermatofibrosarcoma protuberans on the finger of 14-year-old girl&#46;</p><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Case Description</span><p id="par0015" class="elsevierStylePara elsevierViewall">The 14-year-old patient attended the dermatology department of our hospital with a lobulated mass measuring 2&#46;5<span class="elsevierStyleHsp" style=""></span>cm across at the widest point&#46; The tumor was on the dorsal aspect of the middle phalanx of the middle finger of the left hand &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; According to the patient&#44; the lesion had been present for 10 years&#46; It had been growing slowly and progressively but had become painful in the last few months prior to the visit&#46; It was impossible to ascertain whether the lesion had been present from birth&#46; Physical examination showed that the lesion had a variable consistency and extended to the deep layers&#46; It did not move with the tendons and did not hinder movement of the patient&#39;s finger&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">An initial incisional biopsy was taken and&#44; after histopathologic diagnosis&#44; it was decided to fully excise the lesion&#46; Simple surgical excision was performed with primary wound closure&#46; The excision extended down to the tendon and margins exceeded 1<span class="elsevierStyleHsp" style=""></span>cm&#46; During the operation&#44; the tumor appeared to be of variable consistency&#44; with soft areas of myxoid appearance and no invasion of the underlying tendons&#46; Six months after excision&#44; there had been no recurrence&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Histopathologic Study</span><p id="par0025" class="elsevierStylePara elsevierViewall">The material from the incisional biopsy and the surgical excision were both fixed in 10&#37; buffered formalin and embedded in their entirety in paraffin&#46; Sections 3<span class="elsevierStyleHsp" style=""></span>&#956;m thick were taken and stained with hematoxylin-eosin&#46; Subsequently&#44; immunohistochemical staining was performed with the following antibodies using the avidin-biotin complex method and with positive and negative controls&#58; CD34 &#40;DAKO&#44; 1&#58;50&#41;&#44; protein S-100 &#40;DAKO&#44; 1&#58;5000&#41;&#44; factor XIIIa &#40;Behring&#44; 1&#58;1000&#41;&#44; desmin &#40;DAKO&#44; 1&#58;100&#41;&#44; actin 1A4 &#40;DAKO&#44; 1&#58;50&#41;&#44; Ki-67 &#40;DAKO&#44; 1&#58;300&#41;&#44; epithelial membrane antigen &#40;EMA&#41; &#40;DAKO&#44; 1&#58;100&#41;&#44; and B-cell lymphoma &#40;bcl-2&#41; &#40;DAKO&#44; 1&#58;80&#41;&#46; A fluorescence in situ hybridization &#40;FISH&#41; analysis was performed on the paraffin-embedded tumor tissue to detect the translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41;&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">The incisional biopsy showed diffuse infiltration of the reticular dermis by a neoformation consisting of short bundles of spindle cells interlaced in different directions and an evident Grenz zone&#46; The tumor engulfed the skin appendages but neither invaded nor destroyed them &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; The proliferative cells had an eosinophilic cytoplasm with blurred edges and elongated oval nuclei showing fine chromatin and no nucleoli&#46; Only 2 nonatypical mitotic figures were identified per 10 high-magnification fields&#46; Cellularity was less marked in the deep part of the lesion&#44; which had stromal areas with a myxoid appearance&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The excised tissue consisted of an ellipse of skin with a nodule measuring 2&#46;4<span class="elsevierStyleHsp" style=""></span>cm along the longest axis&#46; Histologically&#44; it comprised a mesenchymal neoformation with a diffuse growth pattern and infiltrative borders&#46; The tumor occupied the entire reticular dermis and extended to the subcutaneous cell tissue&#46; It displayed a honeycomb pattern&#44; in which individual adipocytes were isolated &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46; However&#44; 90&#37; of the neoformation comprised hypocellular areas with a myxoid appearance&#46; The cells that were present were fusiform and stellate&#44; without atypia or mitosis&#46; Thin-walled capillaries were seen in abundance and inflammatory cells were few&#46; Focally&#44; a dense fibrous stroma was present&#46; At the edge of the lesion&#44; both in the superficial and deep layers&#44; there were focal areas with the same characteristics as those seen in the incisional biopsy material&#46; The surgical margins at the sides and bottom were extensively involved&#46;</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Staining with colloidal iron was observed &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; On immunohistochemical staining&#44; cytoplasmic expression of CD34 was strong in the areas of the lesion with a classic pattern but weak in the myxoid areas&#46; The other markers used&#8212;protein S-100&#44; factor XIIIa&#44; actin 1A4&#44; EMA&#44; and bcl-2&#8212;were negative&#46; The Ki-67 cell proliferation index was 5&#37; in areas with the classic pattern and 20&#37; in myxoid areas&#46; Diagnosis of dermatofibrosarcoma protuberans was confirmed on demonstrating translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41; by FISH&#46;</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">The myxoid subtype is a very rare dermatofibrosarcoma protuberans variant first described in 1983 by Frierson et al&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> In one of the largest published series of dermatofibrosarcoma protuberans&#44; only 4&#46;3&#37; of the lesions were of this type&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Myxoid lesions differ from the classic variant by presenting mainly on the limbs&#59; the next most frequent sites are the head and neck&#44; followed by the trunk and the anogenital region&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Myxoid dermatofibrosarcoma protuberans is very rare in children&#46; Only 3 childhood cases have been reported&#59; these occurred on the eyelid&#44; scalp&#44; and elbow&#44; and all were in boys&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;4</span></a> We report the first case of myxoid dermatofibrosarcoma protuberans on the finger of a girl&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Clinically&#44; the lesion presented as a nodular dermal or subcutaneous&#44; firm&#44; raised mass that was slow-growing and progressive&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> The most common potential diagnoses for such a lesion are epidermal&#44; mucinous&#44; or synovial cyst&#59; lipoma&#59; pyogenic granuloma&#59; and lymphoma&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> In view of the site&#44; our case was initially considered to be a giant-cell tumor of the tendon sheath&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">Some authors suggest that the myxoid variant has a slightly better prognosis than the classic dermatofibrosarcoma protuberans variant&#44; although others disagree&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> No metastases were reported in a recent series of 23 cases of myxoid dermatofibrosarcoma protuberans&#46; In that same series&#44; 2 patients had local recurrence&#44; one after incomplete resection and the other after leaving margins less than 2<span class="elsevierStyleHsp" style=""></span>mm&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Metastases have not been reported in children&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> In our patient&#44; the lesion had grown slowly and progressively for more than 10 years&#46; There was no recurrence or metastasis 6 months after excision&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">Microscopically&#44; the tumor is located in the reticular dermis and subcutaneous cell tissue and shows a Grenz zone&#44; although it occasionally affects the epidermis and forms an ulcer&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The areas with myxoid&#44; basophilic&#44; or pale stroma predominate&#44; due to the high hyaluronan content&#44; as indicated by staining with colloidal iron&#46; It is likely that the myxoid transformation is due to an increased synthesis of glycosaminoglycans&#44; which inhibit polymerization of the collagen&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> It is not uncommon for traditional dermatofibrosarcoma protuberans to have focal areas with a myxoid appearance&#44; particularly in recurrence&#44; although the presence of such areas does not necessarily point to a myxoid variant&#46; The myxoid subtype is diagnosed when more than 50&#37; of the stroma of the lesion is myxoid&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;8</span></a> In the present case&#44; 70&#37; of the tumor was myxoid&#46; A myxoid stroma has few nonatypical cells&#46; These cells have a fusiform and stellate morphology&#44; with mildly eosinophilic or pale cytoplasm&#44; undefined borders&#44; oval nuclei with fine chromatin and no nucleoli&#44; and occasional or no mitotic figures&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5</span></a> In addition&#44; the tumor contains randomly distributed fine-walled capillaries&#44; which rarely contain mast cells&#46; As in a Bernard tumor&#44; pigmented dendritic cells can also be identified&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5&#44;9</span></a> One of the keys to diagnosis is the presence of areas with the characteristic features of classic dermatofibrosarcoma protuberans at the edge of the lesions&#59; these features are spindle cells arranged in interlaced strands with scant collagen between them and wagon-wheel areas&#46; The lesion engulfs skin appendages without invading them&#44; and on invading subcutaneous adipose tissue&#44; a characteristic honeycomb pattern is formed when individual adipocytes are isolated&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3&#44;5&#44;8&#44;9</span></a> In our case&#44; 30&#37; of the tumor had the features of the classic variant and there were areas at the edge of the lesion in which invasion of the underlying fatty tissue had occurred&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">The most specific immunohistochemical marker is CD34&#44; for which tumors show strong diffuse staining in 50&#37; to 100&#37; of cases&#59; the sensitivity of CD34 positivity is 84&#37; to 100&#37;&#46; In myxoid and fibrosarcomatous areas&#44; staining is weak and focal&#44; or negative&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5&#44;6</span></a> In our case&#44; the lesion showed diffuse positive staining which was stronger in areas of low cellularity&#46; In addition&#44; these tumors are positive for vimentin and CD99&#46; Some are focally positive for factor XIIIa&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> EMA expression might occur in myxoid areas&#44; possibly indicating perineural differentiation&#44;<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> but S-100&#44; actin&#44; and desmin are not expressed&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Ki-67 cell proliferation indices have been reported to be greater in myxoid areas &#40;19&#46;8&#37;&#41; and fibrosarcomatous areas &#40;11&#46;8&#37;&#41; than in areas with classic histology &#40;2&#46;2&#37;&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> In fact&#44; in our case&#44; the Ki-67 proliferative index in myxoid areas was 20&#37; and as low as 5&#37; in the areas of highest cellularity&#46;</p><p id="par0070" class="elsevierStylePara elsevierViewall">Dermatofibrosarcoma protuberans is associated with reciprocal translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41; or a supernumerary ring chromosome r&#40;17&#59;22&#41;&#46; Both give rise to a chimeric gene <span class="elsevierStyleItalic">COL1A1-PDGFB</span> through fusion of the collagen type 1&#945; gene on chromosome 17 and the platelet growth factor-&#946; chain gene on chromosome 22&#46; This leads to continual stimulation of cell growth&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;6</span></a> Translocation is found most frequently in children&#44; whereas the ring chromosome predominates in adults&#44; possibly indicating that this change is a late event in the pathogenesis of dermatofibrosarcoma protuberans&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> This genetic abnormality has also been found in the myxoid variant and in giant-cell fibroblastoma&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Currently&#44; treatment of dermatofibrosarcoma protuberans with selective tyrosine kinase inhibitors is reserved for unresectable disease&#44; recurrences&#44; or metastatic disease&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> A positive immunoreaction with the dermatofibrosarcoma protuberans receptor &#40;PDGFR&#41; B antibody indicates constitutional activation of PDGFR&#44; thereby providing an alternative indirect method for confirming the presence of the dysregulated <span class="elsevierStyleItalic">PDGF</span> gene involved in this translocation&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Faced with a cutaneous&#47;subcutaneous lesion with predominantly myxoid areas and staining for CD34&#44; the pathologist should consider differential diagnosis with benign and malignant mesenchymal lesions with different biological behaviors and different treatment approaches&#44;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> especially when only superficial or small biopsy samples are available&#46; The presence of classic areas of dermatofibrosarcoma protuberans&#44; even if focal&#44; is usually of great help in the diagnosis&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> In the present case&#44; incisional biopsy showed classic areas of dermatofibrosarcoma protuberans&#44; which helped the initial diagnosis&#46; However&#44; this diagnosis had to be reconsidered when the excised specimen was found to be predominantly of myxoid type&#46;</p><p id="par0080" class="elsevierStylePara elsevierViewall">The main differential diagnosis in this case is superficial acral fibromyxoma&#44; which usually affects patients between 14 and 72 years of age and grows for between 3 months and 30 years as a solitary mass that reaches a size of 0&#46;6 to 5 <span class="elsevierStyleHsp" style=""></span>cm across&#46; Acral fibromyxoma has a predilection for fingers and toes and tends to involve the nails&#46; Recurrences are frequent after partial resections&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> Both tumors are located in the dermis and subcutaneous tissue and are composed of stellate and spindle cells&#44; have a fascicular growth pattern which can either be random or form a wagon-wheel pattern in a myxoid or collagenous matrix&#46; Both tumors have moderate vascularization&#44; slight atypia&#44; and limited mytosis&#46; Both also express CD34&#44; EMA&#44; and CD99&#44; and are negative for S-100&#44; actin&#44; desmin&#44; keratin&#44; and HMB-45&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;12</span></a> Clinically&#44; our case appears to correspond more to a superficial acral fibromyxoma&#46; However&#44; subcutaneous tissue infiltration with a honeycomb pattern&#44; the presence of translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41; in the FISH analysis&#44; and the absence of giant multinucleated cells that are present in superficial acral fibromyxoma ruled out this possibility&#46;<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5&#44;12</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Other tumors such as cellular digital fibroma can be readily ruled out because clinically they present as a papule and not as a lobulated mass and are rare in children&#44; and a predominantly myxoid histology has not been reported&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> Cellular digital fibromas are positive for CD34 and factor XIIIa&#44; whereas our patient&#39;s tumor was negative for these antibodies&#46; According to some&#44; a cellular digital fibroma can be thought of as a cellular variant of a superficial acral fibromyxoma&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">Superficial angiomyxoma is another tumor that often recurs locally&#44; although it does not metastasize&#46; It grows slowly and is located in subcutaneous tissue&#44; although it can extend to the dermis&#46; It follows a lobulated growth pattern&#44; however&#44; without the diffuse honeycomb infiltation<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> that was present in our case&#46; A superficial angiomyxoma is composed of spindle and stellate cells in an abundant and highly vascularized basophilic stroma&#46; Neutrophils surround thin-walled vascular structures and epithelial elements &#40;epithelial cords or keratin cysts&#41; are observed in up to half the cases&#59; none of these features were observed in our case&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> These tumors are also positive for CD34&#44; vimentin&#44; and actin&#44; and negative for desmin&#44; S-100&#44; and factor XIIIa&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Our lesion was negative for actin&#46;</p><p id="par0095" class="elsevierStylePara elsevierViewall">Giant cell fibroblastoma is a tumor that affects children&#44; with a predilection for boys&#46; Its morphologic spectrum overlaps that of dermatofibrosarcoma protuberans&#44; and the 2 tumors share a histologic&#44; immunohistochemical&#44; and genetic profile&#46; Areas of giant cell fibroblastoma are found in almost 15&#37; of cases of dermatofibrosarcoma protuberans&#46; These mixed tumors affect the dermis and subcutaneous cellular tissue&#44; engulfing the appendages&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#44;6</span></a> The lesions are hypocellular&#44; with loose bundles of wavy spindle cells in an abundant highly-vascularized collagenous or myxoid stroma&#46; They have a characteristic cell population of pleomorphic cells with a single nucleus or giant multinucleated cells &#40;absent in our case&#41;&#44; are positive for CD34&#44; and have translocation t&#40;17&#59;22&#41;&#40;q22&#59;q13&#41;&#46; They can recur as dermatofibrosarcoma protuberans and vice versa&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Extraneural spindle cell perineuriomas may have a prominent myxoid stroma&#44; but these tumors have well defined borders &#40;whereas the border in our case was infiltrative&#41; and are composed of spindle cells arranged in a wagon wheel pattern&#44; sheets&#44; or bundles&#46; Half the cases are positive for CD34&#44; but they are also positive for perineural markers such as Glut-1 and EMA&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The lesion we describe was negative for these markers&#46; Finally&#44; we note that although solitary fibrous tumors are also positive for CD34 and CD99 and can have a prominent myxoid stroma&#44; they are well delimited&#44; express bcl-2&#44; and have a characteristic hemangiopericytoma-like vascular pattern&#44; unlike the lesion in our case&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle">Conflicts of Interest</span><p id="par0105" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
    "textoCompletoSecciones" => array:1 [
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          "identificador" => "xres95948"
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            0 => "Dermatofibrosarcoma protuberans"
            1 => "Myxoid tumor"
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            0 => "Dermatofibrosarcoma protuberans"
            1 => "Mixoide"
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        "titulo" => "Abstract"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Dermatofibrosarcoma protuberans is a locally aggressive skin tumor that affects young and middle-aged adults&#46; A number of histological variants have been described&#44; the myxoid type being one of the least common&#46; Microscopically it is formed of a neoplastic growth that is located in the dermis and hypodermis and has a predominant myxoid component&#46; Peripherally there are infiltrating bundles of spindle-shaped cells that are diffusely positive for the CD34 immunohistochemical marker&#46; We report a case of myxoid dermatofibrosarcoma protuberans on a finger of the left hand of a 14-year-old girl&#46; The tumor had been present for at least 10 years&#46; This is the first pediatric case of myxoid dermatofibrosarcoma protuberans at this site&#46; This histological subtype has mainly been described on the extremities in adults and is very rare in children&#46; We discuss the differential diagnosis with other CD34<span class="elsevierStyleSup">&#43;</span> myxoid mesenchymal tumors&#46;</p>"
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      "es" => array:2 [
        "titulo" => "Resumen"
        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El dermatofibrosarcoma protuberans &#40;DFSP&#41; es un tumor cut&#225;neo&#44; localmente agresivo&#44; que afecta a adultos j&#243;venes o de edad media&#46; Se han descrito diferentes formas histol&#243;gicas&#44; siendo la mixoide una de las m&#225;s infrecuentes&#46; Microsc&#243;picamente est&#225; constituido por una neoformaci&#243;n que ocupa la dermis e hipodermis&#44; de predominio mixoide&#44; con &#225;reas perif&#233;ricas conformadas por haces de c&#233;lulas fusiformes&#44; de crecimiento infiltrativo&#44; que expresan el marcador inmunohistoqu&#237;mico CD34 de forma difusa&#46; Presentamos por primera vez un DFSP mixoide en un dedo de la mano izquierda de una ni&#241;a de 14 a&#241;os&#44; de m&#225;s de 10 a&#241;os de evoluci&#243;n&#46; Se trata del primer caso infantil de DFSP mixoide en dicha localizaci&#243;n&#46; Este subtipo histol&#243;gico se ha descrito fundamentalmente en las extremidades de adultos&#44; siendo excepcional en ni&#241;os&#46; Se comenta el diagn&#243;stico diferencial con otros tumores mesenquimales mixoides CD34 positivos&#46;</p>"
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        "etiqueta" => "&#9734;"
        "nota" => "<p class="elsevierStyleNotepara">Please cite this article as&#58; Campos M&#44; et al&#46; Dermatofibrosarcoma protuberans mixoide infantile&#46; Actas Dermosifiliogr&#46;2012&#59;103&#58;422-6&#46;</p>"
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          "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Clinical appearance of the lesion&#58; a skin-colored nodule measuring 2&#46;5<span class="elsevierStyleHsp" style=""></span>cm on the middle phalanx of the middle finger of the left hand&#46;</p>"
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          "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Low-magnification image showing a spindle-cell neoformation which has diffusely infiltrated the reticular dermis and part of the subcutaneous tissue &#40;hematoxylin-eosin&#44; original magnification &#215;40&#41;&#46;</p>"
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      2 => array:7 [
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          "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The lesion has extensive myxoid areas&#44; as well as a subcutaneous cellular infiltrate in a honeycomb pattern &#40;hematoxylin-eosin&#44; original magnification &#215;100&#41;&#46;</p>"
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          "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Strong positive staining with colloidal iron in myxoid areas &#40;colloidal iron&#44; original magnification &#215;100&#41;&#46;</p>"
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      "titulo" => "References"
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                            0 => "T&#46; Mentzel"
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                          ]
                        ]
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                  ]
                  "host" => array:1 [
                    0 => array:2 [
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                          "etal" => false
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                      "titulo" => "Dermatofibrosarcoma protuberans&#44; giant cell fibroblastoma and hybrid lesions in children&#58; clinicopathologic comparative analysis of 28 cases with molecular data&#8212;a study from the French Federation of Cancer Centers Sarcoma Group"
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                            1 => "L&#46; Guillou"
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ISSN: 15782190
Original language: English
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