Case for Diagnosis
Ulcerated Congenital Tumor
Tumor congénito ulcerado
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Inset: Detailed image of the lesions on the thigh.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "J. Alonso-González, M.T. Rodríguez-Granados, J. Toribio" "autores" => array:3 [ 0 => array:2 [ "nombre" => "J." "apellidos" => "Alonso-González" ] 1 => array:2 [ "nombre" => "M.T." "apellidos" => "Rodríguez-Granados" ] 2 => array:2 [ "nombre" => "J." 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"apellidos" => "Navarro-Hervas" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario de La Ribera, Alzira, Valencia, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario de La Ribera, Alzira, Valencia, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding Author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Tumor congénito ulcerado" ] ] "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" => 631 "Ancho" => 900 "Tamanyo" => 163583 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, hematoxylin-eosin, original magnification ×10. B, hematoxylin-eosin, original magnification ×20. C, smooth muscle actin, original magnification ×10. D. Desmin, original magnification ×10.</p>" ] ] ] "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 newborn girl was evaluated in our dermatology department for a congenital tumor in the right frontal region. She is the first child of healthy nonconsanguineous parents and was born following a full-term normal pregnancy and a normal delivery. The mother had no history of infection, drug use, or family history of skin disease.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">The patient presented an erythematous nodule with a vascular appearance. The nodule was ulcerated, of fibrous consistency, attached to the deeper layers, and measured approximately 3<span class="elsevierStyleHsp" style=""></span>cm in diameter (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). No other similar skin lesions or palpable evidence of visceromegaly were observed.</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Histology</span><p id="par0015" class="elsevierStylePara elsevierViewall">Histology revealed superficial and deep dermal proliferation of spindle cells with no nuclear atypia. The spindle cells were arranged in cellular bands and fascicles (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>, A and B). Immunohistochemical techniques showed that the cells were positive for vimentin and smooth muscle α-actin (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2C</a>) and negative for S100, myoglobin, cytokeratins, and desmin (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2D</a>).</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0020">Additional Tests</span><p id="par0020" class="elsevierStylePara elsevierViewall">Bone series, abdominal ultrasound, and computed tomography revealed no signs of bone or visceral involvement.</p><p id="par0025" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis?</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Solitary infantile myofibromatosis.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">A decision was made to adopt a wait-and-see approach and perform periodic examinations. At the time of writing, the tumor continues to regress.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Infantile myofibromatosis (IM) is a congenital mesenchymal disorder characterized by the presence of solitary or multiple myofibroblastic tumors which may affect the skin, soft tissues, bones, or internal organs.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> This disorder was first described by Stout in 1954, although the currently used term was introduced in 1981 by Chung and Enzinger.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Though considered a rare disease, IM is nonetheless the most common fibrous tumor in infancy. These tumors habitually present between birth and 2 years of age, and lesions limited to the skin tend to have a good prognosis with high rates of spontaneous regression.</p><p id="par0045" class="elsevierStylePara elsevierViewall">Although most cases of IM are sporadic, a familial form has been described in monozygotic twins and in successive generations; it is possible that such cases may be attributable to an autosomal dominant inheritance pattern with variable penetrance.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The etiology is unknown. Yousefi et al.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> hypothesized that mesenchymal stem cells transferred during pregnancy may participate in tissue remodeling in the fetus, although in a study of tissue samples from 4 newborns with solitary or multiple IM, the authors demonstrated that tumor cells were not derived from maternal chimeric cells. On the other hand, the level of maternal estrogen does appear to influence the development of these tumors, as spontaneous regression occurs after delivery, when the exposure to estrogens has ceased.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">There are 3 patterns of clinical presentation: solitary IM (a single lesion affecting the skin and/or muscles in the head, neck, or trunk; this pattern is the most common one in children, representing 75% of all cases); multicentric IM without visceral involvement (multiple lesions limited to the skin and muscles); and multicentric IM with visceral or systemic involvement (multiple lesions not only of the skin and/or muscles, but also of the bones, lungs, heart, and gastrointestinal tract).<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">The clinical presentation of cutaneous tumors in IM is heterogeneous: these lesions appear as a plaque, nodule, or mass; are solitary or multiple; measure from 0.5 to 5<span class="elsevierStyleHsp" style=""></span>cm; are not painful; have a firm consistency; rarely ulcerate or bleed; and may have a keloid or vascular appearance.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Biopsy of lesions that are accessible—as is usually the case with skin lesions—is required to confirm the diagnosis. IM skin tumors are well-defined dermal nodules exhibiting a biphasic growth pattern. A high number of spindle cells arranged in fascicles (smooth muscle–like fascicles) can be seen on the periphery of the tumor; these cells show no nuclear abnormalities, although there may be occasional mitotic figures, and they express smooth muscle α-actin and vimentin and are negative for S100. The central area contains vascular structures with irregular lumens and a hemangiopericytic pattern.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">Differential diagnosis in cases of isolated lesions includes deep hemangiomas, neurofibroma, leiomyoma, sarcoma, and neuroblastoma metastasis. From a histologic point of view, a distinction must be made between IM and congenital fibrosarcoma and hemangiopericytoma.<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3,5</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Prognosis for solitary and multiple lesions that do not affect internal organs is excellent, with spontaneous remission occurring in 1 to 2 years, probably related to massive apoptosis.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> A conservative wait-and-see approach is appropriate for these types of IM. On the other hand, IM with visceral involvement is serious and associated mortality is high, especially when gastrointestinal and cardiorespiratory systems are compromised.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Patients with visceral disease therefore require surgical and/or medical treatment (e.g., radiation therapy or chemotherapy with vincristine, actinomycin D, and cyclophosphamide) in addition to palliative care.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:8 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Medical History" ] 1 => array:2 [ "identificador" => "sec0010" "titulo" => "Physical Examination" ] 2 => array:2 [ "identificador" => "sec0015" "titulo" => "Histology" ] 3 => array:2 [ "identificador" => "sec0020" "titulo" => "Additional Tests" ] 4 => array:2 [ "identificador" => "sec0025" "titulo" => "Diagnosis" ] 5 => array:2 [ "identificador" => "sec0030" "titulo" => "Clinical Course and Treatment" ] 6 => array:2 [ "identificador" => "sec0035" "titulo" => "Comment" ] 7 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Martí-Fajardo N, et al. Tumor congénito ulcerado. Actas Dermosifiliogr. 2013;104:525–6.</p>" ] ] "multimedia" => array:2 [ 0 => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 475 "Ancho" => 501 "Tamanyo" => 35985 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 631 "Ancho" => 900 "Tamanyo" => 163583 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, hematoxylin-eosin, original magnification ×10. B, hematoxylin-eosin, original magnification ×20. C, smooth muscle actin, original magnification ×10. D. Desmin, original magnification ×10.</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:6 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Infantile myofibromatosis: report of nine patients" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "M. Larralde" 1 => "M.V. Hoffner" 2 => "P. Boggio" 3 => "M.E. Abad" 4 => "P.C. Luna" 5 => "N. Correa" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1111/j.1525-1470.2009.01073.x" "Revista" => array:6 [ "tituloSerie" => "Pediatr Dermatol" "fecha" => "2010" "volumen" => "27" "paginaInicial" => "29" "paginaFinal" => "33" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/20199406" "web" => "Medline" ] ] ] ] ] ] ] ] 1 => array:3 [ "identificador" => "bib0010" "etiqueta" => "2" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "A case of congenital multiple myofibromatosis developing in an infant" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "B.J. Schrodt" 1 => "J.P. Callen" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1542/peds.104.1.113" "Revista" => array:6 [ "tituloSerie" => "Pediatrics" "fecha" => "1999" "volumen" => "104" "paginaInicial" => "113" "paginaFinal" => "115" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/10390272" "web" => "Medline" ] ] ] ] ] ] ] ] 2 => array:3 [ "identificador" => "bib0015" "etiqueta" => "3" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Dermatological presentations of infantile myofibromatosis: a review of 27 cases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:2 [ 0 => "D. Stanford" 1 => "M. Rogers" ] ] ] ] ] "host" => array:1 [ 0 => array:2 [ "doi" => "10.1046/j.1440-0960.2000.00420.x" "Revista" => array:7 [ "tituloSerie" => "Australas J Dermatol" "fecha" => "2000" "volumen" => "41" "paginaInicial" => "156" "paginaFinal" => "161" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/10954986" "web" => "Medline" ] ] "itemHostRev" => array:3 [ "pii" => "S1542356508011087" "estado" => "S300" "issn" => "15423565" ] ] ] ] ] ] ] 3 => array:3 [ "identificador" => "bib0020" "etiqueta" => "4" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Neonatal cases of infantile myofibromatosis do not derive from maternal cells transferred during pregnancy" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:6 [ 0 => "P. Yousefi" 1 => "K. Khosrotehrani" 2 => "M. Oster" 3 => "Y. De Prost" 4 => "S. Fraitag" 5 => "S. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 14 | 13 | 27 |
| 2024 October | 73 | 44 | 117 |
| 2024 September | 88 | 30 | 118 |
| 2024 August | 100 | 74 | 174 |
| 2024 July | 76 | 40 | 116 |
| 2024 June | 83 | 37 | 120 |
| 2024 May | 60 | 37 | 97 |
| 2024 April | 81 | 30 | 111 |
| 2024 March | 69 | 31 | 100 |
| 2024 February | 68 | 33 | 101 |
| 2024 January | 64 | 33 | 97 |
| 2023 December | 60 | 17 | 77 |
| 2023 November | 68 | 24 | 92 |
| 2023 October | 75 | 20 | 95 |
| 2023 September | 56 | 29 | 85 |
| 2023 August | 49 | 27 | 76 |
| 2023 July | 82 | 39 | 121 |
| 2023 June | 48 | 20 | 68 |
| 2023 May | 85 | 31 | 116 |
| 2023 April | 64 | 21 | 85 |
| 2023 March | 60 | 29 | 89 |
| 2023 February | 54 | 17 | 71 |
| 2023 January | 35 | 21 | 56 |
| 2022 December | 44 | 30 | 74 |
| 2022 November | 44 | 22 | 66 |
| 2022 October | 35 | 16 | 51 |
| 2022 September | 27 | 38 | 65 |
| 2022 August | 17 | 26 | 43 |
| 2022 July | 31 | 31 | 62 |
| 2022 June | 29 | 21 | 50 |
| 2022 May | 47 | 41 | 88 |
| 2022 April | 43 | 30 | 73 |
| 2022 March | 53 | 56 | 109 |
| 2022 February | 40 | 26 | 66 |
| 2022 January | 35 | 39 | 74 |
| 2021 December | 35 | 34 | 69 |
| 2021 November | 44 | 39 | 83 |
| 2021 October | 38 | 64 | 102 |
| 2021 September | 31 | 38 | 69 |
| 2021 August | 42 | 29 | 71 |
| 2021 July | 27 | 34 | 61 |
| 2021 June | 34 | 37 | 71 |
| 2021 May | 34 | 40 | 74 |
| 2021 April | 73 | 44 | 117 |
| 2021 March | 68 | 51 | 119 |
| 2021 February | 58 | 36 | 94 |
| 2021 January | 36 | 29 | 65 |
| 2020 December | 34 | 31 | 65 |
| 2020 November | 31 | 23 | 54 |
| 2020 October | 38 | 18 | 56 |
| 2020 September | 36 | 20 | 56 |
| 2020 August | 28 | 23 | 51 |
| 2020 July | 30 | 16 | 46 |
| 2020 June | 22 | 26 | 48 |
| 2020 May | 31 | 16 | 47 |
| 2020 April | 28 | 22 | 50 |
| 2020 March | 28 | 19 | 47 |
| 2020 February | 2 | 5 | 7 |
| 2020 January | 4 | 1 | 5 |
| 2019 December | 8 | 1 | 9 |
| 2019 November | 4 | 0 | 4 |
| 2019 September | 12 | 0 | 12 |
| 2019 August | 4 | 6 | 10 |
| 2019 July | 4 | 5 | 9 |
| 2019 June | 3 | 4 | 7 |
| 2019 May | 4 | 6 | 10 |
| 2019 April | 2 | 7 | 9 |
| 2019 March | 2 | 8 | 10 |
| 2019 February | 2 | 2 | 4 |
| 2019 January | 1 | 0 | 1 |
| 2018 December | 0 | 8 | 8 |
| 2018 November | 1 | 0 | 1 |
| 2018 October | 2 | 0 | 2 |
| 2018 September | 3 | 0 | 3 |
| 2018 March | 1 | 0 | 1 |
| 2018 February | 39 | 3 | 42 |
| 2018 January | 56 | 4 | 60 |
| 2017 December | 51 | 4 | 55 |
| 2017 November | 32 | 2 | 34 |
| 2017 October | 41 | 7 | 48 |
| 2017 September | 34 | 4 | 38 |
| 2017 August | 32 | 8 | 40 |
| 2017 July | 48 | 7 | 55 |
| 2017 June | 46 | 5 | 51 |
| 2017 May | 42 | 3 | 45 |
| 2017 April | 37 | 7 | 44 |
| 2017 March | 50 | 5 | 55 |
| 2017 February | 146 | 4 | 150 |
| 2017 January | 36 | 5 | 41 |
| 2016 December | 59 | 6 | 65 |
| 2016 November | 77 | 6 | 83 |
| 2016 October | 102 | 12 | 114 |
| 2016 September | 180 | 6 | 186 |
| 2016 August | 102 | 7 | 109 |
| 2016 July | 44 | 7 | 51 |
| 2016 June | 8 | 7 | 15 |
| 2016 May | 5 | 0 | 5 |
| 2016 April | 4 | 1 | 5 |
| 2016 March | 4 | 0 | 4 |
| 2016 February | 4 | 1 | 5 |
| 2016 January | 10 | 1 | 11 |
| 2015 December | 3 | 0 | 3 |
| 2015 November | 19 | 1 | 20 |
| 2015 October | 20 | 3 | 23 |
| 2015 September | 8 | 1 | 9 |
| 2015 August | 13 | 2 | 15 |
| 2015 July | 67 | 11 | 78 |
| 2015 June | 33 | 3 | 36 |
| 2015 May | 41 | 3 | 44 |
| 2015 April | 36 | 3 | 39 |
| 2015 March | 25 | 4 | 29 |
| 2015 February | 48 | 3 | 51 |
| 2015 January | 33 | 1 | 34 |
| 2014 December | 30 | 3 | 33 |
| 2014 November | 25 | 6 | 31 |
| 2014 October | 31 | 3 | 34 |
| 2014 September | 32 | 2 | 34 |
| 2014 August | 26 | 4 | 30 |
| 2014 July | 27 | 5 | 32 |
| 2014 June | 42 | 2 | 44 |
| 2014 May | 41 | 7 | 48 |
| 2014 April | 40 | 7 | 47 |
| 2014 March | 41 | 7 | 48 |
| 2014 February | 29 | 7 | 36 |
| 2014 January | 30 | 11 | 41 |
| 2013 December | 19 | 5 | 24 |
| 2013 November | 29 | 2 | 31 |
| 2013 October | 8 | 2 | 10 |
| 2013 September | 3 | 4 | 7 |
| 2013 August | 2 | 4 | 6 |
| 2013 July | 5 | 2 | 7 |
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