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There were no palpable locoregional lymph nodes&#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">Hematoxylin-eosin staining of a biopsy specimen revealed an orthokeratotic epidermis and a dermal infiltrate of elongated pleomorphic cells&#44; intermixed with other cells with rounded and irregular nuclei as well as abundant aberrant mitotic figures &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a> and <a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>A&#41;&#46; This infiltrate formed a storiform pattern over a collagenized fibrous stroma with striking vascular structures &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>B&#41;&#46; Immunohistochemical staining was positive for CD68 and CD31 and showed focal positivity for S100&#44; isolated positivity for vimentin&#44; and a Ki-67 proliferation index of nearly 70&#37;&#46; Other stains&#44; including for smooth muscle actin&#44; desmin&#44; cytokeratin AE1&#47;AE3&#44; CD34&#44; CD10&#44; common leukocyte antigen&#44; HMB-45&#44; and Melan-A&#44; were negative&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">Pleomorphic dermal sarcoma&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">The lesion was excised but the surgical margins were not clear&#46; Therefore&#44; slow Mohs surgery was performed and complete excision was achieved with a single stage of surgery&#46; The defect was closed with a full-thickness skin graft&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">A staging study revealed swelling of a left retroauricular lymph node&#44; which was shown by biopsy to be diseased&#46; The case was evaluated by the tumor committee and complementary treatments were ruled out&#46; After 18 months of follow-up&#44; there had been no local recurrence and no changes in the course of the disease&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Sarcomas are neoplasms of mesenchymal origin that are more common in adults&#46; Classification and prognosis are determined by cell lineage&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Recent advances in immunohistochemistry and molecular genetics have brought about a gradual decrease in the incidence of the tumor previously known as malignant fibrous histiocytoma and an increase in the incidence of more specific entities&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2</span></a> In the latest classification of bone and soft tissue tumors&#44; 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this tumor presents pleomorphic epithelioid cells and atypical spindle cells with numerous mitotic figures and extensive areas of ulceration and necrosis&#44; as well as involvement of deep tissues and vascular and perineural invasion&#44; which distinguishes it from atypical fibroxanthoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a> Immunohistochemistry is essential to rule out other entities such as melanocytic tumors&#44; malignant nerve sheath tumors&#44; poorly differentiated squamous cell carcinomas&#44; angiosarcomas&#44; leiomyosarcomas&#44; and Merkel cell carcinoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;6</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Metastatic spread occurs in an estimated 10&#37; of cases and local recurrence in 30&#37; of cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a> Data from large case series are not available due to the multiple nomenclature changes&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The first-line treatment is conventional surgery with tumor-free margins or Mohs surgery&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;6</span></a> As in other fibrohistiocytic tumors&#44; 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Case and Research Letter
Exophytic Tumor on the Left Temple
Tumoración excrecente en sien izquierda
E. Varas-Meisa,
Autor para correspondencia
edu.varas.meis@gmail.com

Corresponding author.
, M.M. Otero-Rivasb, M.Á. Rodriguez-Prietoa
a Servicio de Dermatología, Complejo Asistencial Universitario, León, Spain
b Servicio de Dermatología, Hospital Universitario Lucus Augusti, Lugo, 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">An 82-year-old man with hypertension&#44; a hiatal hernia&#44; and osteoporosis was seen in outpatients for a painful&#44; localized&#44; nonbleeding&#44; nonulcerated lesion on the left temple that had first appeared 4 months earlier&#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 2-cm erythematous-violaceous exophytic tumor on the left temple with well-defined borders&#46; The lesion extended to the inferior lateral edge of the eyebrow &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; Palpation revealed that the lesion was adherent to the deeper tissue layers&#46; There were no palpable locoregional lymph nodes&#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">Hematoxylin-eosin staining of a biopsy specimen revealed an orthokeratotic epidermis and a dermal infiltrate of elongated pleomorphic cells&#44; intermixed with other cells with rounded and irregular nuclei as well as abundant aberrant mitotic figures &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a> and <a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>A&#41;&#46; This infiltrate formed a storiform pattern over a collagenized fibrous stroma with striking vascular structures &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>B&#41;&#46; Immunohistochemical staining was positive for CD68 and CD31 and showed focal positivity for S100&#44; isolated positivity for vimentin&#44; and a Ki-67 proliferation index of nearly 70&#37;&#46; Other stains&#44; including for smooth muscle actin&#44; desmin&#44; cytokeratin AE1&#47;AE3&#44; CD34&#44; CD10&#44; common leukocyte antigen&#44; HMB-45&#44; and Melan-A&#44; were negative&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Diagnosis</span><p id="par0025" class="elsevierStylePara elsevierViewall">Pleomorphic dermal sarcoma&#46;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Clinical Course and Treatment</span><p id="par0030" class="elsevierStylePara elsevierViewall">The lesion was excised but the surgical margins were not clear&#46; Therefore&#44; slow Mohs surgery was performed and complete excision was achieved with a single stage of surgery&#46; The defect was closed with a full-thickness skin graft&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">A staging study revealed swelling of a left retroauricular lymph node&#44; which was shown by biopsy to be diseased&#46; The case was evaluated by the tumor committee and complementary treatments were ruled out&#46; After 18 months of follow-up&#44; there had been no local recurrence and no changes in the course of the disease&#46;</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Sarcomas are neoplasms of mesenchymal origin that are more common in adults&#46; Classification and prognosis are determined by cell lineage&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Recent advances in immunohistochemistry and molecular genetics have brought about a gradual decrease in the incidence of the tumor previously known as malignant fibrous histiocytoma and an increase in the incidence of more specific entities&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;2</span></a> In the latest classification of bone and soft tissue tumors&#44; published by the World Health Organization &#40;WHO&#41; in 2013&#44; the term malignant fibrous histiocytoma was replaced by undifferentiated pleomorphic sarcoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">1&#44;3</span></a> This is a diagnosis of exclusion for fibrohistiocytic neoplasms that cannot be clearly differentiated using the diagnostic techniques currently available&#46; It is important to avoid confusion between the tumor now known as undifferentiated pleomorphic sarcoma and the similarly named pleomorphic dermal sarcoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Pleomorphic dermal sarcoma typically appears in men in the seventh or eighth decade of life as a rapidly growing dermal nodule with necrotic and hemorrhagic areas&#44; usually on sun-exposed sites such as the head and neck&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;5</span></a> Characteristically&#44; this tumor presents pleomorphic epithelioid cells and atypical spindle cells with numerous mitotic figures and extensive areas of ulceration and necrosis&#44; as well as involvement of deep tissues and vascular and perineural invasion&#44; which distinguishes it from atypical fibroxanthoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a> Immunohistochemistry is essential to rule out other entities such as melanocytic tumors&#44; malignant nerve sheath tumors&#44; poorly differentiated squamous cell carcinomas&#44; angiosarcomas&#44; leiomyosarcomas&#44; and Merkel cell carcinoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">2&#44;6</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Metastatic spread occurs in an estimated 10&#37; of cases and local recurrence in 30&#37; of cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#8211;6</span></a> Data from large case series are not available due to the multiple nomenclature changes&#46;</p><p id="par0060" class="elsevierStylePara elsevierViewall">The first-line treatment is conventional surgery with tumor-free margins or Mohs surgery&#46;<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">4&#44;6</span></a> As in other fibrohistiocytic tumors&#44; there are no consensus recommendations on the treatment of metastatic disease or follow-up&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">This case underscores the need to be familiar with and apply the new WHO classification&#44; which will make it possible to standardize the diagnosis of soft tissue tumors&#44; thereby improving clinical management of these neoplasms&#46; The case also highlights the importance of immunohistochemistry in the diagnosis of these tumors and the need to perform staging studies to assess the possibility of distant metastasis&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Conflicts of Interest</span><p id="par0070" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest&#46;</p></span></span>"
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