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Upon examination&#44; a 5-cm&#44; rounded&#44; erythematous-violaceous&#44; pre-auricular tumor was observed&#44; with friable and necrotic areas associated with a 1-cm satellite lesion with similar characteristics and a post-SCC excision skin graft scar &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; No regional lymph node enlargement was detected&#46; The hypotheses were SCC&#44; SCC metastasis&#44; and angiosarcoma&#46; Histopathological examination &#40;<a class="elsevierStyleCrossRefs" href="#fig0010">Figs&#46; 2 and 3</a>&#41; showed a dermal neoplasm with polygonal clear cells&#44; evident nuclear pleomorphism&#44; cell debris&#44; and frequent mitoses&#46; Immunohistochemistry disclosed positivity for epithelial markers AE1&#47;AE3 and epithelial membrane antigen &#40;EMA&#41; which&#44; associated with histopathological findings&#44; allowed the diagnosis of sebaceous carcinoma &#40;SC&#41;&#44; and thus&#44; the patient was referred to the Head and Neck Surgery Division of the institution&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">In a review of 1349 SC cases&#44; a predominance of males &#40;54&#37;&#41; was observed&#44; as well as mean age of 73 years&#44; 86&#37; whites&#44; 38&#46;7&#37; on the palpebra&#44; with a survival rate of 91&#46;9&#37;&#44; and 79&#46;2&#37; in 5 and 10 years respectively&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The most frequent metastases were found in the lymph nodes&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> Most cases occur <span class="elsevierStyleItalic">de novo</span>&#44; although it may originate from benign sebaceous lesions and&#44; when located in the upper or lower eyelid&#44; it is associated with the Meibomian and Zeis glands&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> Clinical presentation is variable&#59; it is usually painless and slow-growing&#44; but it can be rapid-growing and aggressive&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">It is the third or fourth most frequent malignant neoplasm of the eyelids&#44; 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especially due to the rapid growth and aggressiveness of the tumor&#44; which when diagnosed had a specific satellite lesion and tumorous clinical aspect&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0035" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors&#8217; contributions</span><p id="par0040" class="elsevierStylePara elsevierViewall">Luana Moraes Campos&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; critical review of the literature&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Joana Alexandria Ferreira Dias&#58; Approval of the final version of the manuscript&#59; critical review of the manuscript&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Paula Basso Lima&#58; 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Case Letter
Extraocular sebaceous carcinoma: tumor presentation of rapid evolution
Luana Moraes Campos, Joana Alexandria Ferreira Dias, Paula Basso Lima, Sílvio Alencar Marques
Corresponding author
silvio.marques@unesp.br

Corresponding author.
Faculdade de Medicina, Universidade Estadual Paulista, Botucatu, SP, Brazil
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    "titulo" => "Extraocular sebaceous carcinoma&#58; tumor presentation of rapid evolution"
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    "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Sebaceous carcinoma &#40;SC&#41; is a rare malignant neoplasm derived from the adnexal epithelium of the sebaceous glands&#44; with a higher incidence in the ocular region&#44; particularly in the eyelid region&#44; and has a potentially aggressive behavior&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> Older age&#44; previous radiotherapy&#44; and association with Muir-Torre syndrome are predisposing conditions&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a></p><p id="par0010" class="elsevierStylePara elsevierViewall">This is the case report of a 75-year-old white male patient with a history of squamous cell carcinoma &#40;SCC&#41;&#44; referred for treatment of facial lesion noted three months before&#44; with rapid growth and bleeding episodes associated with trauma&#46; Upon examination&#44; a 5-cm&#44; rounded&#44; erythematous-violaceous&#44; pre-auricular tumor was observed&#44; with friable and necrotic areas associated with a 1-cm satellite lesion with similar characteristics and a post-SCC excision skin graft scar &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; No regional lymph node enlargement was detected&#46; The hypotheses were SCC&#44; SCC metastasis&#44; and angiosarcoma&#46; Histopathological examination &#40;<a class="elsevierStyleCrossRefs" href="#fig0010">Figs&#46; 2 and 3</a>&#41; showed a dermal neoplasm with polygonal clear cells&#44; evident nuclear pleomorphism&#44; cell debris&#44; and frequent mitoses&#46; Immunohistochemistry disclosed positivity for epithelial markers AE1&#47;AE3 and epithelial membrane antigen &#40;EMA&#41; which&#44; associated with histopathological findings&#44; allowed the diagnosis of sebaceous carcinoma &#40;SC&#41;&#44; and thus&#44; the patient was referred to the Head and Neck Surgery Division of the institution&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><elsevierMultimedia ident="fig0010"></elsevierMultimedia><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">In a review of 1349 SC cases&#44; a predominance of males &#40;54&#37;&#41; was observed&#44; as well as mean age of 73 years&#44; 86&#37; whites&#44; 38&#46;7&#37; on the palpebra&#44; with a survival rate of 91&#46;9&#37;&#44; and 79&#46;2&#37; in 5 and 10 years respectively&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> The most frequent metastases were found in the lymph nodes&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> Most cases occur <span class="elsevierStyleItalic">de novo</span>&#44; although it may originate from benign sebaceous lesions and&#44; when located in the upper or lower eyelid&#44; it is associated with the Meibomian and Zeis glands&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> Clinical presentation is variable&#59; it is usually painless and slow-growing&#44; but it can be rapid-growing and aggressive&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0020" class="elsevierStylePara elsevierViewall">It is the third or fourth most frequent malignant neoplasm of the eyelids&#44; depending on the reference&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a> The most frequent extraocular location is the cephalic segment&#44; especially the face&#46; The diagnosis of SC should be a warning sign&#44; as it is a possible marker of Muir-Torre syndrome&#44; a genodermatosis characterized by the presence of skin tumors of sebaceous origin associated with systemic malignancies&#44; particularly of the gastrointestinal tract&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> A subcutaneous nodule is usually observed in SC&#44; which is normochromic&#59; however&#44; it may disclose different morphologies&#44; colors and behavior&#44; depending on its place of origin&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;3</span></a></p><p id="par0025" class="elsevierStylePara elsevierViewall">The differential diagnosis of extraocular SC includes basal cell carcinoma &#40;BCC&#41;&#44; SCC&#44; amelanotic melanoma&#44; Merkel cell carcinoma&#44; and cutaneous lymphoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;3</span></a> The immunohistochemical use of markers for BerEP4&#44; EMA &#40;negative in BCC&#41;&#44; AE1 and AE3 &#40;negative in melanoma&#44; lymphomas&#41;&#44; adipophilin &#40;negative in SCC&#44; Merkel&#41;&#44; p53 and Ki-67&#44; will aid in the diagnosis and prognosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#8211;4</span></a> The treatment comprises surgical resection with a 1-cm margin or the use of the micrographic surgical technique&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> A therapeutic option in cases of metastatic SC to the lungs and CNS is immunotherapy with pembrolizumab&#44; which belongs to the class of inhibitors of anti-PD1 immunological checkpoints &#40;programmed death-1&#41;&#44; and is also used in metastatic melanoma and Merkel cell carcinoma&#46;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0030" class="elsevierStylePara elsevierViewall">This case report exemplifies a case of an atypical presentation of extraocular SC&#44; especially due to the rapid growth and aggressiveness of the tumor&#44; which when diagnosed had a specific satellite lesion and tumorous clinical aspect&#46;</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Financial support</span><p id="par0035" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Authors&#8217; contributions</span><p id="par0040" class="elsevierStylePara elsevierViewall">Luana Moraes Campos&#58; Approval of the final version of the manuscript&#59; design and planning of the study&#59; critical review of the literature&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Joana Alexandria Ferreira Dias&#58; Approval of the final version of the manuscript&#59; critical review of the manuscript&#46;</p><p id="par0050" class="elsevierStylePara elsevierViewall">Paula Basso Lima&#58; Approval of the final version of the manuscript&#59; collection&#44; analysis&#44; and interpretation of data&#59; critical review of the manuscript&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">S&#237;lvio Alencar Marques&#58; Approval of the final version of the manuscript&#59; drafting and editing of the manuscript&#59; critical review of the literature&#59; critical review of the manuscript&#46;</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflicts of interest</span><p id="par0060" class="elsevierStylePara elsevierViewall">None declared&#46;</p></span></span>"
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