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The cells were spindle-shaped and arranged horizontally to the epidermis&#44; with a focal swirling pattern and honeycomb-like fat infiltration &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Immunohistochemistry revealed CD34 expression &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Doppler ultrasound performed prior to biopsy showed a poorly defined&#44; subcutaneous echogenic area &#40;30<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>18<span class="elsevierStyleHsp" style=""></span>mm&#41; of absent flow&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Based on the histological diagnosis the patient underwent magnetic resonance imaging&#44; which revealed a subcutaneous lesion that did not reach deep planes and no locoregional lymphadenopathy&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">What Is Your Diagnosis&#63;</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Atrophic dermatofibrosarcoma protuberans &#40;DFSP&#41;&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">The tumor was excised with wide surgical margins&#44; achieving tumor-free margins&#46; The patient showed no signs of recurrence after 23 months of follow-up&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">DFSP is a cutaneous mesenchymal neoplasm of intermediate malignancy&#44; 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supporting an etiological role of trauma&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Other clinical and pathological subtypes have been described&#44; including an atrophic variant &#40;which may also correspond to an early stage of a prolonged presentation&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> This subtype can be difficult to distinguish clinically from morphea&#44; atrophoderma&#44; atrophic scar &#40;as in the present case&#41;&#44; anetoderma&#44; aplasia cutis&#44; and lipoatrophy&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;4</span></a> Histology shows a dermal tumor that is thinner than the healthy dermis and retracts the skin&#44; and consists of monomorphic spindle cells with mild atypia arranged in a storiform pattern&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;4</span></a> Immunohistochemistry is key to distinguishing this lesion from other tumors such as atrophic dermatofibroma&#44; atypical fibroxanthoma&#44; and undifferentiated pleomorphic sarcoma&#46; DFSP is strongly positive for CD34 and negative for S100&#44; EMA&#44; desmin&#44; and smooth muscle actin&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Most DFSP patients carry a translocation in chromosomes 17 and 22&#44; giving rise to the chimeric gene <span class="elsevierStyleItalic">COL1A1-PDGFB</span>&#44; detection of which by fluorescence in situ hybridization &#40;FISH&#41; or reverse transcriptase polymerase chain reaction &#40;RT-PCR&#41; is highly specific for DFSP&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;5</span></a> The main treatment for DFSP is complete eradication of the tumor through excision with wide margins&#59; Mohs surgery is considered the treatment of choice&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Imatinib treatment can be considered in cases of unresectable tumors or in advanced stages of the disease&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> After surgery&#44; follow-up every 6 to 12 months for at least 5 years is recommended&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> The prognosis for atrophic DFSP is the same as that for the classical form&#44; but confusion of this tumor with benign atrophic lesions by both patients and doctors means that diagnosis and treatment&#44; and subsequent cure&#44; are often delayed&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#44;4</span></a> The present case underscores the importance of biopsy in cases of slow-growing atrophic plaques of uncertain diagnosis&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflicts of Interest</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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        "texto" => "<p id="par0050" class="elsevierStylePara elsevierViewall">The authors thank Dr&#46; Juan Ayestaran of the Plastic Surgery Service and Dr&#46; Alexandre Nogueira of the Pathological Anatomy Service of the Hospital Universitario de Cruces for their important contributions to the diagnosis and treatment of the patient&#46;</p>"
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Case for Diagnosis
Atrophic Plaque on the Back of a 14-Year-Old Boy
Placa atrófica en la espalda de un varón de 14 años
S. Esteban Terradillosa,
Autor para correspondencia
, I. Allende Markixanaa, V. Velasco Benitob
a Servicio de Dermatología, Hospital Universitario Cruces, Barakaldo, Vizcaya, Spain
b Servicio de Anatomía Patológica, Hospital Universitario Cruces, Barakaldo, Vizcaya, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 14-year-old boy with no medical history of interest was seen for an asymptomatic lesion of stable size that had appeared 2 years earlier&#46; The patient believed that the lesion may have been associated with trauma&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed a sclerotic-looking atrophic plaque of approximately 3<span class="elsevierStyleHsp" style=""></span>cm in size with a central purple macule located in the right costolumbar area &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histopathology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Histopathology showed a hypercellular tumor with poorly defined borders located in the reticular dermis and extending into the hypodermis&#46; The cells were spindle-shaped and arranged horizontally to the epidermis&#44; with a focal swirling pattern and honeycomb-like fat infiltration &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46; Immunohistochemistry revealed CD34 expression &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Doppler ultrasound performed prior to biopsy showed a poorly defined&#44; subcutaneous echogenic area &#40;30<span class="elsevierStyleHsp" style=""></span>mm<span class="elsevierStyleHsp" style=""></span>&#215;<span class="elsevierStyleHsp" style=""></span>18<span class="elsevierStyleHsp" style=""></span>mm&#41; of absent flow&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">Based on the histological diagnosis the patient underwent magnetic resonance imaging&#44; which revealed a subcutaneous lesion that did not reach deep planes and no locoregional lymphadenopathy&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">What Is Your Diagnosis&#63;</span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Atrophic dermatofibrosarcoma protuberans &#40;DFSP&#41;&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">The tumor was excised with wide surgical margins&#44; achieving tumor-free margins&#46; The patient showed no signs of recurrence after 23 months of follow-up&#46;</p></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">DFSP is a cutaneous mesenchymal neoplasm of intermediate malignancy&#44; characterized by a marked tendency to local recurrence and a low metastatic capacity&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> It accounts for 0&#46;1&#37; of all malignant skin tumors&#46; It predominantly appears between the second and fifth decades of life&#44; although it is thought that the proportion of pediatric cases is underestimated due to late diagnosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;2</span></a> Fortunately&#44; in children this tumor appears to be less aggressive and to recur less frequently&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> It typically manifests as pinkish or brownish plaques on which exophytic nodules appear &#40;as implied by the word <span class="elsevierStyleItalic">protuberans</span>&#41;&#44; and is usually located on the trunk&#46; In children&#44; DFSP most commonly affects the lower extremities and acral areas&#44; supporting an etiological role of trauma&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Other clinical and pathological subtypes have been described&#44; including an atrophic variant &#40;which may also correspond to an early stage of a prolonged presentation&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">3</span></a> This subtype can be difficult to distinguish clinically from morphea&#44; atrophoderma&#44; atrophic scar &#40;as in the present case&#41;&#44; anetoderma&#44; aplasia cutis&#44; and lipoatrophy&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;4</span></a> Histology shows a dermal tumor that is thinner than the healthy dermis and retracts the skin&#44; and consists of monomorphic spindle cells with mild atypia arranged in a storiform pattern&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;4</span></a> Immunohistochemistry is key to distinguishing this lesion from other tumors such as atrophic dermatofibroma&#44; atypical fibroxanthoma&#44; and undifferentiated pleomorphic sarcoma&#46; DFSP is strongly positive for CD34 and negative for S100&#44; EMA&#44; desmin&#44; and smooth muscle actin&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">4</span></a> Most DFSP patients carry a translocation in chromosomes 17 and 22&#44; giving rise to the chimeric gene <span class="elsevierStyleItalic">COL1A1-PDGFB</span>&#44; detection of which by fluorescence in situ hybridization &#40;FISH&#41; or reverse transcriptase polymerase chain reaction &#40;RT-PCR&#41; is highly specific for DFSP&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">1&#44;5</span></a> The main treatment for DFSP is complete eradication of the tumor through excision with wide margins&#59; Mohs surgery is considered the treatment of choice&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> Imatinib treatment can be considered in cases of unresectable tumors or in advanced stages of the disease&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> After surgery&#44; follow-up every 6 to 12 months for at least 5 years is recommended&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">1</span></a> The prognosis for atrophic DFSP is the same as that for the classical form&#44; but confusion of this tumor with benign atrophic lesions by both patients and doctors means that diagnosis and treatment&#44; and subsequent cure&#44; are often delayed&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">3&#44;4</span></a> The present case underscores the importance of biopsy in cases of slow-growing atrophic plaques of uncertain diagnosis&#46;</p></span></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Conflicts of Interest</span><p id="par0045" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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                        "fecha" => "2018"
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                      "Revista" => array:5 [
                        "tituloSerie" => "Int J Clin Exp Pathol"
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        "texto" => "<p id="par0050" class="elsevierStylePara elsevierViewall">The authors thank Dr&#46; Juan Ayestaran of the Plastic Surgery Service and Dr&#46; Alexandre Nogueira of the Pathological Anatomy Service of the Hospital Universitario de Cruces for their important contributions to the diagnosis and treatment of the patient&#46;</p>"
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Información del artículo
ISSN: 15782190
Idioma original: Inglés
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