What is your diagnosis?
Case for diagnosis. Stewart-Treves syndrome after mastectomy
Roberta Akeme de Oliveira Sato
, Clovis Antônio Lopes Pinto, Celia Antonia Xavier de Moraes Alves, Juliana Arêas de Souza Lima Beltrame Ferreira
Corresponding author
Department of Dermatology, Faculdade de Medicina de Jundiaí, Jundiaí, SP, Brazil
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Stewart-Treves syndrome after mastectomy" "tieneTextoCompleto" => true "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "651" "paginaFinal" => "653" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "Roberta Akeme de Oliveira Sato, Clovis Antônio Lopes Pinto, Celia Antonia Xavier de Moraes Alves, Juliana Arêas de Souza Lima Beltrame Ferreira" "autores" => array:4 [ 0 => array:4 [ "nombre" => "Roberta Akeme de Oliveira" "apellidos" => "Sato" "email" => array:1 [ 0 => "robertaakeme@hotmail.com" ] "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">*</span>" "identificador" => "cor0005" ] ] ] 1 => array:2 [ "nombre" => "Clovis Antônio Lopes" "apellidos" => "Pinto" ] 2 => array:2 [ "nombre" => "Celia Antonia Xavier de Moraes" "apellidos" => "Alves" ] 3 => array:2 [ "nombre" => "Juliana Arêas de Souza Lima Beltrame" "apellidos" => "Ferreira" ] ] "afiliaciones" => array:1 [ 0 => array:2 [ "entidad" => "Department of Dermatology, Faculdade de Medicina de Jundiaí, Jundiaí, SP, Brazil" "identificador" => "aff0005" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 845 "Ancho" => 1500 "Tamanyo" => 295437 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Proliferation of epithelioid and spindle cells, permeating malformed vascular channels and spaces, (Hematoxylin & eosin, ×100).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Case report</span><p id="par0005" class="elsevierStylePara elsevierViewall">A 56-year-old female patient reported the appearance of a nodule on the posterior region of the right arm six months before. During this period, she reported that the lesion showed progressive growth, pain, and friability, with frequent local bleeding. This patient had been submitted to a radical mastectomy and right axillary dissection, with adjuvant chemotherapy and radiotherapy 11 years before, and since then, she had chronic lymphedema in the right upper limb.</p><p id="par0010" class="elsevierStylePara elsevierViewall">Dermatological examination revealed a hyperchromic tumor, measuring approximately 5.0 × 4.0 cm, with central areas of ulceration and slight local bleeding on the posterior region of the right arm. Lymphedema was also observed in the right upper limb and violaceous-brown satellite nodules, measuring up to 0.5 cm (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">An incisional biopsy of the tumor and satellite nodules was performed. The patient returned for consultation after one month with a histopathological result that showed a neoplasm characterized by vascular formations permeated by atypical epithelioid cells infiltrating the superficial and deep dermis, with perineural permeation (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">At this consultation the tumor was slightly larger and there was an increase in the number of satellite lesions (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>).</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia></span><span id="sec1005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect1005">What is your diagnosis?</span><p id="par0025" class="elsevierStylePara elsevierViewall"><ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">a)</span><p id="par0030" class="elsevierStylePara elsevierViewall">Stewart-Treves syndrome</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">b)</span><p id="par0035" class="elsevierStylePara elsevierViewall">Squamous cell carcinoma</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">c)</span><p id="par0040" class="elsevierStylePara elsevierViewall">Melanoma</p></li><li class="elsevierStyleListItem" id="lsti0020"><span class="elsevierStyleLabel">d)</span><p id="par0045" class="elsevierStylePara elsevierViewall">Merkel carcinoma</p></li></ul></p><p id="par0050" class="elsevierStylePara elsevierViewall">Immunohistochemistry examination was requested, which showed CD31 positivity, confirming the hypothesis of Stewart-Treves syndrome (<a class="elsevierStyleCrossRef" href="#fig0020">Fig. 4</a>).</p><elsevierMultimedia ident="fig0020"></elsevierMultimedia></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Discussion</span><p id="par0055" class="elsevierStylePara elsevierViewall">Stewart-Treves syndrome (STS) is a rare entity, with a poor prognosis, consisting of the appearance of cutaneous angiosarcoma in areas of chronic lymphedema and accounts for approximately 5% of angiosarcomas.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1–4</span></a> Its occurrence is more commonly observed after a radical mastectomy with axillary dissection, with a latency period that varies from five to 11 years.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,4</span></a> However, it can also appear in areas of chronic lymphedema due to venous stasis, morbid obesity, post-surgical procedures, lymphatic malformations, and chronic infections, among others.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,5</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The etiology and pathophysiology of the syndrome remain unknown. It is debated whether the lymphedema would favor oncogenesis due to the lymphatic drainage failure and interstitial fluid accumulation and stasis, in addition to the possible occurrence of neoplastic transformation during angiogenesis.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> They initially present as macules and papules, developing into nodules and tumors that can reach large volumes.<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6,7</span></a> Satellite lesions, pain, and local bleeding are common.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">The diagnosis is based on the clinical picture, along with the biopsy. Histopathology may be suggestive, with irregular vascular spaces lined by mitotic hyperchromatic pleomorphic tumor endothelial cells.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Immunohistochemistry helps in the diagnostic confirmation, with both CD31 and CD34 markers being positive.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The treatment is based on surgical resection with wide margins, and chemotherapy and radiotherapy can also be used.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,3,6,7</span></a> In the present case, the oncology service chose to use chemotherapy with docetaxel, and the oncology surgery team, so far has not indicated surgical treatment. The occurrence of distant metastases is not uncommon,<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> with the lungs being the most commonly affected organs; the patient in the present case was submitted to CT scans of the chest, abdomen, and pelvis, which did not disclose the presence of metastases.</p><p id="par0075" class="elsevierStylePara elsevierViewall">Although it is rare, knowledge of STS is necessary because, due to its aggressiveness, only its early diagnosis can help to increase patient survival.</p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Financial support</span><p id="par0080" class="elsevierStylePara elsevierViewall">None declared.</p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Authors' contributions</span><p id="par0085" class="elsevierStylePara elsevierViewall">Roberta Akeme de Oliveira Sato: Design and planning of the study; drafting and editing of the manuscript; critical review of the literature.</p><p id="par0090" class="elsevierStylePara elsevierViewall">Clovis Antônio Lopes Pinto: Intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; effective participation in research orientation.</p><p id="par0095" class="elsevierStylePara elsevierViewall">Celia Antonia Xavier de Moraes Alves: Effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases.</p><p id="par0100" class="elsevierStylePara elsevierViewall">Juliana Arêas de Souza Lima Beltrame Ferreira: Effective participation in research orientation; intellectual participation in the propaedeutic and/or therapeutic conduct of the studied cases; critical review of the manuscript; approval of the final version of the manuscript.</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Conflicts of interest</span><p id="par0105" class="elsevierStylePara elsevierViewall">None declared.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:7 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Case report" ] 1 => array:2 [ "identificador" => "sec1005" "titulo" => "What is your diagnosis?" ] 2 => array:2 [ "identificador" => "sec0010" "titulo" => "Discussion" ] 3 => array:2 [ "identificador" => "sec0015" "titulo" => "Financial support" ] 4 => array:2 [ "identificador" => "sec0020" "titulo" => "Authors' contributions" ] 5 => array:2 [ "identificador" => "sec0025" "titulo" => "Conflicts of interest" ] 6 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "fechaRecibido" => "2021-10-14" "fechaAceptado" => "2021-12-14" "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Study conducted at the Department of Dermatology, Faculdade de Medicina de Jundiaí, Jundiaí, SP, Brazil.</p>" ] ] "multimedia" => array:4 [ 0 => array:8 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1093 "Ancho" => 1500 "Tamanyo" => 143959 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0005" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">(A), Lymphedema in the right upper limb. (B), Tumor with ulcerated areas in the posterior region of the right arm, accompanied by satellite nodules.</p>" ] ] 1 => array:8 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 845 "Ancho" => 1500 "Tamanyo" => 295437 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0010" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Proliferation of epithelioid and spindle cells, permeating malformed vascular channels and spaces, (Hematoxylin & eosin, ×100).</p>" ] ] 2 => array:8 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 842 "Ancho" => 1005 "Tamanyo" => 79201 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0015" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Significant increase in the number of satellite lesions and in the ulcerated area of the central tumor.</p>" ] ] 3 => array:8 [ "identificador" => "fig0020" "etiqueta" => "Figure 4" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr4.jpeg" "Alto" => 1112 "Ancho" => 1500 "Tamanyo" => 462030 ] ] "detalles" => array:1 [ 0 => array:3 [ "identificador" => "at0020" "detalle" => "Figure " "rol" => "short" ] ] "descripcion" => array:1 [ "en" => "<p id="spar0020" class="elsevierStyleSimplePara elsevierViewall">Immunohistochemistry showing positivity for CD31.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:7 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Clinicopathologic features of Stewart-Treves syndrome" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "L.L. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 12 | 8 | 20 |
| 2024 October | 117 | 74 | 191 |
| 2024 September | 133 | 107 | 240 |
| 2024 August | 160 | 113 | 273 |
| 2024 July | 131 | 108 | 239 |
| 2024 June | 93 | 72 | 165 |
| 2024 May | 81 | 62 | 143 |
| 2024 April | 93 | 98 | 191 |
| 2024 March | 93 | 69 | 162 |
| 2024 February | 127 | 84 | 211 |
| 2024 January | 71 | 59 | 130 |
| 2023 December | 57 | 56 | 113 |
| 2023 November | 69 | 75 | 144 |
| 2023 October | 54 | 69 | 123 |
| 2023 September | 72 | 75 | 147 |
| 2023 August | 61 | 32 | 93 |
| 2023 July | 54 | 41 | 95 |
| 2023 June | 57 | 57 | 114 |
| 2023 May | 42 | 25 | 67 |
| 2023 April | 34 | 19 | 53 |
| 2023 March | 51 | 51 | 102 |
| 2023 February | 38 | 29 | 67 |
| 2023 January | 39 | 48 | 87 |
| 2022 December | 63 | 42 | 105 |
| 2022 November | 78 | 57 | 135 |
| 2022 October | 85 | 73 | 158 |
| 2022 September | 134 | 99 | 233 |
| 2022 August | 75 | 52 | 127 |
| 2022 July | 29 | 45 | 74 |
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