Case Report
Myxoid Dermatofibrosarcoma Protuberans in Childhood
Dermatofibrosarcoma protuberans mixoide infantil
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Campos, C. Zarco, F. Acquadro, E. Riveiro-Falkenbach, J.L. Rodríguez-Peralto" "autores" => array:5 [ 0 => array:3 [ "nombre" => "M." "apellidos" => "Campos" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 1 => array:3 [ "nombre" => "C." "apellidos" => "Zarco" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "F." "apellidos" => "Acquadro" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] ] 3 => array:3 [ "nombre" => "E." "apellidos" => "Riveiro-Falkenbach" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 4 => array:4 [ "nombre" => "J.L." "apellidos" => "Rodríguez-Peralto" "email" => array:1 [ 0 => "jrodriguezp.hdoc@salud.madrid.org" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] ] "afiliaciones" => array:3 [ 0 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario 12 de Octubre, Madrid, Spain" "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario 12 de Octubre, Madrid, Spain" "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] 2 => array:3 [ "entidad" => "Grupo de Citogenética Molecular, CNIO, Madrid, Spain" "etiqueta" => "<span class="elsevierStyleSup">c</span>" "identificador" => "aff0015" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Dermatofibrosarcoma protuberans mixoide infantil" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1129 "Ancho" => 850 "Tamanyo" => 461567 ] ] "descripcion" => array:1 [ "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 (hematoxylin-eosin, original magnification ×40).</p>" ] ] ] "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.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> Incidence peaks in individuals in their 30s.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> The tumor grows slowly, is locally aggressive and of intermediate malignancy, and recurs in more than a third of cases. Metastasis is rare but spread is usually hematogenous when it does occur. Between 10% and 15% of recurrences are found in areas of fibrosarcoma, neurofibrosarcoma, or myxofibrosarcoma.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3–5</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">Different types of dermatofibrosarcoma protuberans have been described, including the classic, pigmented (Bernard tumor), fibrosarcomatous, granular cell, flat atrophic, and myxoid variants, as well as variants with myogenic differentiation.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2</span></a> The fibrosarcomatous subtype is associated with a worse prognosis, a risk of metastasis of 10% to 15%, and a rate of tumor-related death of 5.8%.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6,7</span></a> One of the least common types is the myxoid variant, which presents on the limbs, head, neck, and trunk in adults. It is very rare in children and only 3 cases have been reported in the literature.<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.</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 2.5<span class="elsevierStyleHsp" style=""></span>cm across at the widest point. The tumor was on the dorsal aspect of the middle phalanx of the middle finger of the left hand (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). According to the patient, the lesion had been present for 10 years. It had been growing slowly and progressively but had become painful in the last few months prior to the visit. It was impossible to ascertain whether the lesion had been present from birth. Physical examination showed that the lesion had a variable consistency and extended to the deep layers. It did not move with the tendons and did not hinder movement of the patient's finger.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">An initial incisional biopsy was taken and, after histopathologic diagnosis, it was decided to fully excise the lesion. Simple surgical excision was performed with primary wound closure. The excision extended down to the tendon and margins exceeded 1<span class="elsevierStyleHsp" style=""></span>cm. During the operation, the tumor appeared to be of variable consistency, with soft areas of myxoid appearance and no invasion of the underlying tendons. Six months after excision, there had been no recurrence.</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% buffered formalin and embedded in their entirety in paraffin. Sections 3<span class="elsevierStyleHsp" style=""></span>μm thick were taken and stained with hematoxylin-eosin. Subsequently, immunohistochemical staining was performed with the following antibodies using the avidin-biotin complex method and with positive and negative controls: CD34 (DAKO, 1:50), protein S-100 (DAKO, 1:5000), factor XIIIa (Behring, 1:1000), desmin (DAKO, 1:100), actin 1A4 (DAKO, 1:50), Ki-67 (DAKO, 1:300), epithelial membrane antigen (EMA) (DAKO, 1:100), and B-cell lymphoma (bcl-2) (DAKO, 1:80). A fluorescence in situ hybridization (FISH) analysis was performed on the paraffin-embedded tumor tissue to detect the translocation t(17;22)(q22;q13).</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. The tumor engulfed the skin appendages but neither invaded nor destroyed them (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>). The proliferative cells had an eosinophilic cytoplasm with blurred edges and elongated oval nuclei showing fine chromatin and no nucleoli. Only 2 nonatypical mitotic figures were identified per 10 high-magnification fields. Cellularity was less marked in the deep part of the lesion, which had stromal areas with a myxoid appearance.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The excised tissue consisted of an ellipse of skin with a nodule measuring 2.4<span class="elsevierStyleHsp" style=""></span>cm along the longest axis. Histologically, it comprised a mesenchymal neoformation with a diffuse growth pattern and infiltrative borders. The tumor occupied the entire reticular dermis and extended to the subcutaneous cell tissue. It displayed a honeycomb pattern, in which individual adipocytes were isolated (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>). However, 90% of the neoformation comprised hypocellular areas with a myxoid appearance. The cells that were present were fusiform and stellate, without atypia or mitosis. Thin-walled capillaries were seen in abundance and inflammatory cells were few. Focally, a dense fibrous stroma was present. At the edge of the lesion, both in the superficial and deep layers, there were focal areas with the same characteristics as those seen in the incisional biopsy material. The surgical margins at the sides and bottom were extensively involved.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Staining with colloidal iron was observed (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>). On immunohistochemical staining, cytoplasmic expression of CD34 was strong in the areas of the lesion with a classic pattern but weak in the myxoid areas. The other markers used—protein S-100, factor XIIIa, actin 1A4, EMA, and bcl-2—were negative. The Ki-67 cell proliferation index was 5% in areas with the classic pattern and 20% in myxoid areas. Diagnosis of dermatofibrosarcoma protuberans was confirmed on demonstrating translocation t(17;22)(q22;q13) by FISH.</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.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> In one of the largest published series of dermatofibrosarcoma protuberans, only 4.3% of the lesions were of this type.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Myxoid lesions differ from the classic variant by presenting mainly on the limbs; the next most frequent sites are the head and neck, followed by the trunk and the anogenital region.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Myxoid dermatofibrosarcoma protuberans is very rare in children. Only 3 childhood cases have been reported; these occurred on the eyelid, scalp, and elbow, and all were in boys.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,4</span></a> We report the first case of myxoid dermatofibrosarcoma protuberans on the finger of a girl.</p><p id="par0050" class="elsevierStylePara elsevierViewall">Clinically, the lesion presented as a nodular dermal or subcutaneous, firm, raised mass that was slow-growing and progressive.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> The most common potential diagnoses for such a lesion are epidermal, mucinous, or synovial cyst; lipoma; pyogenic granuloma; and lymphoma.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> In view of the site, our case was initially considered to be a giant-cell tumor of the tendon sheath.</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, although others disagree.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a> No metastases were reported in a recent series of 23 cases of myxoid dermatofibrosarcoma protuberans. In that same series, 2 patients had local recurrence, one after incomplete resection and the other after leaving margins less than 2<span class="elsevierStyleHsp" style=""></span>mm.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> Metastases have not been reported in children.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> In our patient, the lesion had grown slowly and progressively for more than 10 years. There was no recurrence or metastasis 6 months after excision.</p><p id="par0060" class="elsevierStylePara elsevierViewall">Microscopically, the tumor is located in the reticular dermis and subcutaneous cell tissue and shows a Grenz zone, although it occasionally affects the epidermis and forms an ulcer.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> The areas with myxoid, basophilic, or pale stroma predominate, due to the high hyaluronan content, as indicated by staining with colloidal iron. It is likely that the myxoid transformation is due to an increased synthesis of glycosaminoglycans, which inhibit polymerization of the collagen.<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, particularly in recurrence, although the presence of such areas does not necessarily point to a myxoid variant. The myxoid subtype is diagnosed when more than 50% of the stroma of the lesion is myxoid.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,8</span></a> In the present case, 70% of the tumor was myxoid. A myxoid stroma has few nonatypical cells. These cells have a fusiform and stellate morphology, with mildly eosinophilic or pale cytoplasm, undefined borders, oval nuclei with fine chromatin and no nucleoli, and occasional or no mitotic figures.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,5</span></a> In addition, the tumor contains randomly distributed fine-walled capillaries, which rarely contain mast cells. As in a Bernard tumor, pigmented dendritic cells can also be identified.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,5,9</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; these features are spindle cells arranged in interlaced strands with scant collagen between them and wagon-wheel areas. The lesion engulfs skin appendages without invading them, and on invading subcutaneous adipose tissue, a characteristic honeycomb pattern is formed when individual adipocytes are isolated.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3,5,8,9</span></a> In our case, 30% 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.</p><p id="par0065" class="elsevierStylePara elsevierViewall">The most specific immunohistochemical marker is CD34, for which tumors show strong diffuse staining in 50% to 100% of cases; the sensitivity of CD34 positivity is 84% to 100%. In myxoid and fibrosarcomatous areas, staining is weak and focal, or negative.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,5,6</span></a> In our case, the lesion showed diffuse positive staining which was stronger in areas of low cellularity. In addition, these tumors are positive for vimentin and CD99. Some are focally positive for factor XIIIa.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> EMA expression might occur in myxoid areas, possibly indicating perineural differentiation,<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> but S-100, actin, and desmin are not expressed.<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 (19.8%) and fibrosarcomatous areas (11.8%) than in areas with classic histology (2.2%).<a class="elsevierStyleCrossRef" href="#bib0055"><span class="elsevierStyleSup">11</span></a> In fact, in our case, the Ki-67 proliferative index in myxoid areas was 20% and as low as 5% in the areas of highest cellularity.</p><p id="par0070" class="elsevierStylePara elsevierViewall">Dermatofibrosarcoma protuberans is associated with reciprocal translocation t(17;22)(q22;q13) or a supernumerary ring chromosome r(17;22). Both give rise to a chimeric gene <span class="elsevierStyleItalic">COL1A1-PDGFB</span> through fusion of the collagen type 1α gene on chromosome 17 and the platelet growth factor-β chain gene on chromosome 22. This leads to continual stimulation of cell growth.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,6</span></a> Translocation is found most frequently in children, whereas the ring chromosome predominates in adults, possibly indicating that this change is a late event in the pathogenesis of dermatofibrosarcoma protuberans.<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.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Currently, treatment of dermatofibrosarcoma protuberans with selective tyrosine kinase inhibitors is reserved for unresectable disease, recurrences, or metastatic disease.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> A positive immunoreaction with the dermatofibrosarcoma protuberans receptor (PDGFR) B antibody indicates constitutional activation of PDGFR, thereby providing an alternative indirect method for confirming the presence of the dysregulated <span class="elsevierStyleItalic">PDGF</span> gene involved in this translocation.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Faced with a cutaneous/subcutaneous lesion with predominantly myxoid areas and staining for CD34, the pathologist should consider differential diagnosis with benign and malignant mesenchymal lesions with different biological behaviors and different treatment approaches,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> especially when only superficial or small biopsy samples are available. The presence of classic areas of dermatofibrosarcoma protuberans, even if focal, is usually of great help in the diagnosis.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> In the present case, incisional biopsy showed classic areas of dermatofibrosarcoma protuberans, which helped the initial diagnosis. However, this diagnosis had to be reconsidered when the excised specimen was found to be predominantly of myxoid type.</p><p id="par0080" class="elsevierStylePara elsevierViewall">The main differential diagnosis in this case is superficial acral fibromyxoma, 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 0.6 to 5 <span class="elsevierStyleHsp" style=""></span>cm across. Acral fibromyxoma has a predilection for fingers and toes and tends to involve the nails. Recurrences are frequent after partial resections.<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, have a fascicular growth pattern which can either be random or form a wagon-wheel pattern in a myxoid or collagenous matrix. Both tumors have moderate vascularization, slight atypia, and limited mytosis. Both also express CD34, EMA, and CD99, and are negative for S-100, actin, desmin, keratin, and HMB-45.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,12</span></a> Clinically, our case appears to correspond more to a superficial acral fibromyxoma. However, subcutaneous tissue infiltration with a honeycomb pattern, the presence of translocation t(17;22)(q22;q13) in the FISH analysis, and the absence of giant multinucleated cells that are present in superficial acral fibromyxoma ruled out this possibility.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,12</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, and a predominantly myxoid histology has not been reported.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> Cellular digital fibromas are positive for CD34 and factor XIIIa, whereas our patient's tumor was negative for these antibodies. According to some, a cellular digital fibroma can be thought of as a cellular variant of a superficial acral fibromyxoma.<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, although it does not metastasize. It grows slowly and is located in subcutaneous tissue, although it can extend to the dermis. It follows a lobulated growth pattern, however, without the diffuse honeycomb infiltation<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> that was present in our case. A superficial angiomyxoma is composed of spindle and stellate cells in an abundant and highly vascularized basophilic stroma. Neutrophils surround thin-walled vascular structures and epithelial elements (epithelial cords or keratin cysts) are observed in up to half the cases; none of these features were observed in our case.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a> These tumors are also positive for CD34, vimentin, and actin, and negative for desmin, S-100, and factor XIIIa.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Our lesion was negative for actin.</p><p id="par0095" class="elsevierStylePara elsevierViewall">Giant cell fibroblastoma is a tumor that affects children, with a predilection for boys. Its morphologic spectrum overlaps that of dermatofibrosarcoma protuberans, and the 2 tumors share a histologic, immunohistochemical, and genetic profile. Areas of giant cell fibroblastoma are found in almost 15% of cases of dermatofibrosarcoma protuberans. These mixed tumors affect the dermis and subcutaneous cellular tissue, engulfing the appendages.<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4,6</span></a> The lesions are hypocellular, with loose bundles of wavy spindle cells in an abundant highly-vascularized collagenous or myxoid stroma. They have a characteristic cell population of pleomorphic cells with a single nucleus or giant multinucleated cells (absent in our case), are positive for CD34, and have translocation t(17;22)(q22;q13). They can recur as dermatofibrosarcoma protuberans and vice versa.<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, but these tumors have well defined borders (whereas the border in our case was infiltrative) and are composed of spindle cells arranged in a wagon wheel pattern, sheets, or bundles. Half the cases are positive for CD34, but they are also positive for perineural markers such as Glut-1 and EMA.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The lesion we describe was negative for these markers. Finally, we note that although solitary fibrous tumors are also positive for CD34 and CD99 and can have a prominent myxoid stroma, they are well delimited, express bcl-2, and have a characteristic hemangiopericytoma-like vascular pattern, unlike the lesion in our case.<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.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:8 [ 0 => array:2 [ "identificador" => "xres95948" "titulo" => "Abstract" ] 1 => array:2 [ "identificador" => "xpalclavsec83108" "titulo" => "Keywords" ] 2 => array:2 [ "identificador" => "xres95949" "titulo" => "Resumen" ] 3 => array:2 [ "identificador" => "xpalclavsec83107" "titulo" => "Palabras clave" ] 4 => array:3 [ "identificador" => "sec0005" "titulo" => "Introduction" "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0010" "titulo" => "Case Description" ] 1 => array:2 [ "identificador" => "sec0015" "titulo" => "Histopathologic Study" ] ] ] 5 => array:2 [ "identificador" => "sec0020" "titulo" => "Discussion" ] 6 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflicts of Interest" ] 7 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "PalabrasClave" => array:2 [ "en" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Keywords" "identificador" => "xpalclavsec83108" "palabras" => array:3 [ 0 => "Dermatofibrosarcoma protuberans" 1 => "Myxoid tumor" 2 => "Childhood" ] ] ] "es" => array:1 [ 0 => array:4 [ "clase" => "keyword" "titulo" => "Palabras clave" "identificador" => "xpalclavsec83107" "palabras" => array:3 [ 0 => "Dermatofibrosarcoma protuberans" 1 => "Mixoide" 2 => "Infantil" ] ] ] ] "tieneResumen" => true "resumen" => array:2 [ "en" => array:2 [ "titulo" => "Abstract" "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Dermatofibrosarcoma protuberans is a locally aggressive skin tumor that affects young and middle-aged adults. A number of histological variants have been described, the myxoid type being one of the least common. Microscopically it is formed of a neoplastic growth that is located in the dermis and hypodermis and has a predominant myxoid component. Peripherally there are infiltrating bundles of spindle-shaped cells that are diffusely positive for the CD34 immunohistochemical marker. We report a case of myxoid dermatofibrosarcoma protuberans on a finger of the left hand of a 14-year-old girl. The tumor had been present for at least 10 years. This is the first pediatric case of myxoid dermatofibrosarcoma protuberans at this site. This histological subtype has mainly been described on the extremities in adults and is very rare in children. We discuss the differential diagnosis with other CD34<span class="elsevierStyleSup">+</span> myxoid mesenchymal tumors.</p>" ] "es" => array:2 [ "titulo" => "Resumen" "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">El dermatofibrosarcoma protuberans (DFSP) es un tumor cutáneo, localmente agresivo, que afecta a adultos jóvenes o de edad media. Se han descrito diferentes formas histológicas, siendo la mixoide una de las más infrecuentes. Microscópicamente está constituido por una neoformación que ocupa la dermis e hipodermis, de predominio mixoide, con áreas periféricas conformadas por haces de células fusiformes, de crecimiento infiltrativo, que expresan el marcador inmunohistoquímico CD34 de forma difusa. Presentamos por primera vez un DFSP mixoide en un dedo de la mano izquierda de una niña de 14 años, de más de 10 años de evolución. Se trata del primer caso infantil de DFSP mixoide en dicha localización. Este subtipo histológico se ha descrito fundamentalmente en las extremidades de adultos, siendo excepcional en niños. Se comenta el diagnóstico diferencial con otros tumores mesenquimales mixoides CD34 positivos.</p>" ] ] "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara">Please cite this article as: Campos M, et al. Dermatofibrosarcoma protuberans mixoide infantile. Actas Dermosifiliogr.2012;103:422-6.</p>" ] ] "multimedia" => array:4 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 591 "Ancho" => 700 "Tamanyo" => 45373 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Clinical appearance of the lesion: a skin-colored nodule measuring 2.5<span class="elsevierStyleHsp" style=""></span>cm on the middle phalanx of the middle finger of the left hand.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 1129 "Ancho" => 850 "Tamanyo" => 461567 ] ] "descripcion" => array:1 [ "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 (hematoxylin-eosin, original magnification ×40).</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 1100 "Ancho" => 828 "Tamanyo" => 413872 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0025" class="elsevierStyleSimplePara elsevierViewall">The lesion has extensive myxoid areas, as well as a subcutaneous cellular infiltrate in a honeycomb pattern (hematoxylin-eosin, original magnification ×100).</p>" ] ] 3 => array:7 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 715 "Ancho" => 950 "Tamanyo" => 299685 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0030" class="elsevierStyleSimplePara elsevierViewall">Strong positive staining with colloidal iron in myxoid areas (colloidal iron, original magnification ×100).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:13 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Myxoid Dermatofibrosarcoma Protuberans: Clinicopathologic Immunohistochemical and Molecular Analysis of Eight Cases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "T. 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Hotta" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1136/jcp.2004.024430" "Revista" => array:6 [ "tituloSerie" => "J Clin Pathol" "fecha" => "2005" "volumen" => "58" "paginaInicial" => "984" "paginaFinal" => "986" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16126884" "web" => "Medline" ] ] ] ] ] ] ] ] 11 => array:3 [ "identificador" => "bib0060" "etiqueta" => "12" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Superficial acral fibromyxoma: a clinipathologic and immunohistochemical analysis of 37 cases of a distinctive soft tissue tumor with a predilection for the fingers and toes" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:3 [ 0 => "J.F. Fetsch" 1 => "W.B. Laskin" 2 => "M. Miettinen" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1053/hupa.2001.25903" "Revista" => array:6 [ "tituloSerie" => "Hum Pathol" "fecha" => "2001" "volumen" => "32" "paginaInicial" => "704" "paginaFinal" => "714" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/11486169" "web" => "Medline" ] ] ] ] ] ] ] ] 12 => array:3 [ "identificador" => "bib0065" "etiqueta" => "13" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Cellular digital fibromas: distinctive CD34-positive lesions that may mimic dermatofibrosarcoma protuberans" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "J. McNiff" 1 => "A. Subtil" 2 => "S.E. Cowper" 3 => "R. Lazova" 4 => "E.J. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 9 | 14 | 23 |
| 2024 Octubre | 117 | 39 | 156 |
| 2024 Septiembre | 93 | 22 | 115 |
| 2024 Agosto | 140 | 59 | 199 |
| 2024 Julio | 102 | 39 | 141 |
| 2024 Junio | 101 | 36 | 137 |
| 2024 Mayo | 112 | 40 | 152 |
| 2024 Abril | 79 | 26 | 105 |
| 2024 Marzo | 118 | 29 | 147 |
| 2024 Febrero | 82 | 26 | 108 |
| 2024 Enero | 69 | 31 | 100 |
| 2023 Diciembre | 73 | 22 | 95 |
| 2023 Noviembre | 91 | 30 | 121 |
| 2023 Octubre | 93 | 32 | 125 |
| 2023 Septiembre | 87 | 30 | 117 |
| 2023 Agosto | 72 | 16 | 88 |
| 2023 Julio | 100 | 42 | 142 |
| 2023 Junio | 75 | 19 | 94 |
| 2023 Mayo | 92 | 27 | 119 |
| 2023 Abril | 67 | 12 | 79 |
| 2023 Marzo | 68 | 26 | 94 |
| 2023 Febrero | 86 | 19 | 105 |
| 2023 Enero | 55 | 47 | 102 |
| 2022 Diciembre | 82 | 39 | 121 |
| 2022 Noviembre | 53 | 28 | 81 |
| 2022 Octubre | 31 | 22 | 53 |
| 2022 Septiembre | 29 | 35 | 64 |
| 2022 Agosto | 21 | 36 | 57 |
| 2022 Julio | 28 | 32 | 60 |
| 2022 Junio | 32 | 20 | 52 |
| 2022 Mayo | 83 | 38 | 121 |
| 2022 Abril | 87 | 43 | 130 |
| 2022 Marzo | 64 | 54 | 118 |
| 2022 Febrero | 71 | 35 | 106 |
| 2022 Enero | 82 | 54 | 136 |
| 2021 Diciembre | 69 | 33 | 102 |
| 2021 Noviembre | 60 | 41 | 101 |
| 2021 Octubre | 64 | 46 | 110 |
| 2021 Septiembre | 93 | 48 | 141 |
| 2021 Agosto | 105 | 37 | 142 |
| 2021 Julio | 99 | 34 | 133 |
| 2021 Junio | 60 | 34 | 94 |
| 2021 Mayo | 85 | 39 | 124 |
| 2021 Abril | 201 | 48 | 249 |
| 2021 Marzo | 109 | 23 | 132 |
| 2021 Febrero | 124 | 43 | 167 |
| 2021 Enero | 74 | 25 | 99 |
| 2020 Diciembre | 61 | 16 | 77 |
| 2020 Noviembre | 46 | 19 | 65 |
| 2020 Octubre | 44 | 15 | 59 |
| 2020 Septiembre | 71 | 18 | 89 |
| 2020 Agosto | 36 | 20 | 56 |
| 2020 Julio | 54 | 18 | 72 |
| 2020 Junio | 55 | 25 | 80 |
| 2020 Mayo | 31 | 13 | 44 |
| 2020 Abril | 33 | 22 | 55 |
| 2020 Marzo | 38 | 26 | 64 |
| 2020 Febrero | 6 | 9 | 15 |
| 2020 Enero | 6 | 10 | 16 |
| 2019 Diciembre | 8 | 1 | 9 |
| 2019 Noviembre | 4 | 1 | 5 |
| 2019 Septiembre | 8 | 2 | 10 |
| 2019 Agosto | 4 | 3 | 7 |
| 2019 Julio | 4 | 5 | 9 |
| 2019 Junio | 6 | 15 | 21 |
| 2019 Mayo | 4 | 21 | 25 |
| 2019 Abril | 2 | 6 | 8 |
| 2019 Marzo | 2 | 6 | 8 |
| 2019 Febrero | 2 | 4 | 6 |
| 2019 Enero | 1 | 0 | 1 |
| 2018 Noviembre | 1 | 0 | 1 |
| 2018 Octubre | 3 | 0 | 3 |
| 2018 Septiembre | 3 | 0 | 3 |
| 2018 Febrero | 54 | 7 | 61 |
| 2018 Enero | 157 | 7 | 164 |
| 2017 Diciembre | 170 | 7 | 177 |
| 2017 Noviembre | 89 | 5 | 94 |
| 2017 Octubre | 47 | 4 | 51 |
| 2017 Septiembre | 40 | 12 | 52 |
| 2017 Agosto | 60 | 8 | 68 |
| 2017 Julio | 28 | 3 | 31 |
| 2017 Junio | 59 | 10 | 69 |
| 2017 Mayo | 42 | 9 | 51 |
| 2017 Abril | 43 | 10 | 53 |
| 2017 Marzo | 37 | 4 | 41 |
| 2017 Febrero | 75 | 11 | 86 |
| 2017 Enero | 32 | 4 | 36 |
| 2016 Diciembre | 45 | 4 | 49 |
| 2016 Noviembre | 77 | 24 | 101 |
| 2016 Octubre | 89 | 26 | 115 |
| 2016 Septiembre | 179 | 14 | 193 |
| 2016 Agosto | 105 | 14 | 119 |
| 2016 Julio | 53 | 10 | 63 |
| 2016 Junio | 16 | 17 | 33 |
| 2016 Mayo | 3 | 10 | 13 |
| 2016 Abril | 8 | 4 | 12 |
| 2016 Marzo | 7 | 8 | 15 |
| 2016 Febrero | 8 | 8 | 16 |
| 2016 Enero | 11 | 1 | 12 |
| 2015 Diciembre | 13 | 10 | 23 |
| 2015 Noviembre | 17 | 11 | 28 |
| 2015 Octubre | 17 | 12 | 29 |
| 2015 Septiembre | 12 | 5 | 17 |
| 2015 Agosto | 16 | 3 | 19 |
| 2015 Julio | 84 | 8 | 92 |
| 2015 Junio | 60 | 4 | 64 |
| 2015 Mayo | 96 | 9 | 105 |
| 2015 Abril | 81 | 10 | 91 |
| 2015 Marzo | 77 | 5 | 82 |
| 2015 Febrero | 74 | 10 | 84 |
| 2015 Enero | 74 | 18 | 92 |
| 2014 Diciembre | 109 | 12 | 121 |
| 2014 Noviembre | 71 | 12 | 83 |
| 2014 Octubre | 128 | 15 | 143 |
| 2014 Septiembre | 64 | 11 | 75 |
| 2014 Agosto | 88 | 13 | 101 |
| 2014 Julio | 95 | 20 | 115 |
| 2014 Junio | 104 | 18 | 122 |
| 2014 Mayo | 52 | 10 | 62 |
| 2014 Abril | 50 | 5 | 55 |
| 2014 Marzo | 60 | 6 | 66 |
| 2014 Febrero | 56 | 13 | 69 |
| 2014 Enero | 42 | 7 | 49 |
| 2013 Diciembre | 34 | 12 | 46 |
| 2013 Noviembre | 51 | 16 | 67 |
| 2013 Octubre | 44 | 8 | 52 |
| 2013 Septiembre | 34 | 10 | 44 |
| 2013 Agosto | 45 | 19 | 64 |
| 2013 Julio | 21 | 17 | 38 |
| 2013 Junio | 25 | 38 | 63 |
| 2013 Mayo | 15 | 23 | 38 |
| 2013 Abril | 16 | 27 | 43 |
| 2013 Marzo | 21 | 10 | 31 |
| 2013 Febrero | 36 | 4 | 40 |
| 2013 Enero | 42 | 4 | 46 |
| 2012 Diciembre | 37 | 7 | 44 |
| 2012 Octubre | 0 | 2 | 2 |
| 2012 Agosto | 2 | 2 | 4 |
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