Case for Diagnosis
Infant with Subcutaneous Tumor in the Genital Area
Lactante con tumoración subcutánea en el área genital
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Bernat García, A. Fuertes Prosper, E. Marti Ibor" "autores" => array:3 [ 0 => array:4 [ "nombre" => "J." "apellidos" => "Bernat García" "email" => array:1 [ 0 => "josefabernat@hotmail.com" ] "referencia" => array:2 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] 1 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">¿</span>" "identificador" => "cor0005" ] ] ] 1 => array:3 [ "nombre" => "A." "apellidos" => "Fuertes Prosper" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] 2 => array:3 [ "nombre" => "E." "apellidos" => "Marti Ibor" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio Dermatología, Hospital Universitario Doctor Peset, Valencia, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Anatomía Patológica, Hospital Universitario Doctor Peset, 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" => "Lactante con tumoración subcutánea en el área genital" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:6 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 1034 "Ancho" => 775 "Tamanyo" => 83760 ] ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Medical History</span><p id="par0005" class="elsevierStylePara elsevierViewall">We present the case of a 10-month old girl with a painless subcutaneous tumor on the mons veneris and labia majora. The lesion had not been present at birth and had been noticed by the girl's parents several weeks before consultation.</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Physical Examination</span><p id="par0010" class="elsevierStylePara elsevierViewall">Clinically, the lesion was a firm, poorly circumscribed subcutaneous nodular tumor localized in the genital region. It was more palpable than visible (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>). No other lesions, similar or otherwise, were detected elsewhere on the body. The girl was in good health and her development was normal for her age.</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">Histologic examination of the lesion showed a proliferation of fibrous tissue in the deep dermis and subcutaneous tissue interspersed with lobules of mature adipose tissue. Examination under higher magnification showed how the fibrous tissue was organized into bands of fibrosis with a moderate number of spindle cells that resembled immature fibroblasts (<a class="elsevierStyleCrossRef" href="#fig0010">Figs. 2</a>A and B).</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">Magnetic resonance imaging of the pelvis showed a tumor on the anterior portion of both labia majora and on the mons veneris, predominantly affecting the subcutaneous tissue; there was no enhancement with the administration of contrast material. The staging study (bone scan, chest radiograph, echocardiogram, and abdominal ultrasound) showed no other alterations.</p><p id="par0025" class="elsevierStylePara elsevierViewall"><span class="elsevierStyleBold">What Is Your Diagnosis?</span></p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Lipofibromatosis.</p></span><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Clinical Course and Treatment</span><p id="par0035" class="elsevierStylePara elsevierViewall">Because of the size and location of the lesion, it was decided to adopt a watch and wait approach. A year after diagnosis, the lesion was less noticeable and showed signs of spontaneous regression, although a slightly hardened area was still palpable in the area of the mons veneris.</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Lipofibromatosis is an uncommon benign lesion that was first described by Fetsch et al.<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a>in 2000 and recognized as an independent fibrofatty tumor of childhood in the World Health Organization Classification of Tumours in 2002.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">The tumor is exclusive to children and is sometimes congenital. It presents as a poorly circumscribed subcutaneous mass measuring a few centimeters that grows slowly and painlessly. The hands and feet are the most common sites affected.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,3</span></a> Tumors in the genital region, such as that described in our patient, are very rare. Histologically, the lesion consists of a poorly circumscribed mass with alternating streaks of adipose tissue consisting of mature adipocytes, with bands of fibrosis formed by abundant spindle cells resembling immature fibroblasts. These fibrous bands and spindle cells replace the connective tissue septa of the hypodermis. Adipose tissue can account for over 70% of the tumor.<a class="elsevierStyleCrossRefs" href="#bib0010"><span class="elsevierStyleSup">2,4</span></a> The tumor is similar to fibrous hamartoma of infancy but it lacks the islands of immature oval cells immersed in a myxoid stroma. Mitotic figures are scarce and cellular atypia is not observed. Intermediate cells containing lipid vacuoles in their cytoplasm may be observed in the interface between the fascicles of spindle cells and mature adipocytes.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,5</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Spindle cells are focally and variably immunoreactive to CD34, bcl-2, S-100 protein, smooth muscle actin, and epithelial membrane actin. CD99 positivity may be occasionally observed. Immunostains for desmin and cytokeratin are negative.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,2,5,6</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The differential diagnosis of lipofibromatosis includes fibrous hamartoma of infancy, calcifying aponeurotic fibroma, lipoblastoma, and fibrolipoma.</p><p id="par0060" class="elsevierStylePara elsevierViewall">The tumor is poorly circumscribed and recurrence is common due to the difficulty of achieving complete surgical resection. However, there have been no reports of metastasis or malignant transformation.</p><p id="par0065" class="elsevierStylePara elsevierViewall">Complete excision is the treatment of choice, particularly if the operation can be performed with minimal morbidity. However, patients with a risk of considerable functional impairment following complete excision should be managed on a case-by-case basis, as there have been reports of patients with incompletely excised tumors not experiencing recurrence during long-term follow-up. Recurrence appears to be more common in boys, congenital tumors, tumors on the hands or feet, incompletely excised tumors, and tumors with mitotic figures in the spindle cell component.<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1,5</span></a></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" => "Histopathology" ] 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: Bernat García J, Fuertes Prosper A, Marti Ibor E. Lactante con tumoración subcutánea en el área genital. Actas Dermosifiliogr. 2014;105:619–620.</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" => 1034 "Ancho" => 775 "Tamanyo" => 83760 ] ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 515 "Ancho" => 982 "Tamanyo" => 244718 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">A, Hematoxylin-eosin, original magnification ×40. B, Hematoxylin-eosin, original magnification ×100.</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" => "A clinicopathologic study of 45 pediatric soft tissue tumors with an admixture of adipose tissue and fibroblastic elements, and a proposal for classification as lipofibromatosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "J.F. Fetsch" 1 => "M. Miettinen" 2 => "W.B. Laskin" 3 => "M. Michal" 4 => "F.M. 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Requena" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "LibroEditado" => array:5 [ "titulo" => "Tumores cutáneos de partes blandas" "paginaInicial" => "933" "paginaFinal" => "934" "edicion" => "1.<span class="elsevierStyleSup">a</span> ed" "serieFecha" => "2012" ] ] ] ] ] ] 5 => array:3 [ "identificador" => "bib0030" "etiqueta" => "6" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Inmunohistoquímica en dermatopatología: revisión de los anticuerpos utilizados con mayor frecuencia (parte <span class="elsevierStyleSmallCaps">i</span>)" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:4 [ 0 => "L. Fuertes" 1 => "C. Santonja" 2 => "H. Kutzner" 3 => "L. 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| año/Mes | Html | Total | |
|---|---|---|---|
| 2024 Noviembre | 13 | 12 | 25 |
| 2024 Octubre | 55 | 33 | 88 |
| 2024 Septiembre | 66 | 18 | 84 |
| 2024 Agosto | 102 | 43 | 145 |
| 2024 Julio | 57 | 27 | 84 |
| 2024 Junio | 85 | 51 | 136 |
| 2024 Mayo | 53 | 26 | 79 |
| 2024 Abril | 60 | 19 | 79 |
| 2024 Marzo | 51 | 25 | 76 |
| 2024 Febrero | 56 | 33 | 89 |
| 2024 Enero | 39 | 30 | 69 |
| 2023 Diciembre | 34 | 18 | 52 |
| 2023 Noviembre | 39 | 21 | 60 |
| 2023 Octubre | 45 | 17 | 62 |
| 2023 Septiembre | 49 | 23 | 72 |
| 2023 Agosto | 33 | 20 | 53 |
| 2023 Julio | 36 | 27 | 63 |
| 2023 Junio | 49 | 19 | 68 |
| 2023 Mayo | 50 | 17 | 67 |
| 2023 Abril | 47 | 18 | 65 |
| 2023 Marzo | 41 | 32 | 73 |
| 2023 Febrero | 50 | 20 | 70 |
| 2023 Enero | 38 | 28 | 66 |
| 2022 Diciembre | 45 | 38 | 83 |
| 2022 Noviembre | 48 | 21 | 69 |
| 2022 Octubre | 59 | 26 | 85 |
| 2022 Septiembre | 100 | 39 | 139 |
| 2022 Agosto | 179 | 18 | 197 |
| 2022 Julio | 104 | 36 | 140 |
| 2022 Junio | 174 | 30 | 204 |
| 2022 Mayo | 418 | 37 | 455 |
| 2022 Abril | 445 | 40 | 485 |
| 2022 Marzo | 359 | 42 | 401 |
| 2022 Febrero | 391 | 20 | 411 |
| 2022 Enero | 443 | 33 | 476 |
| 2021 Diciembre | 292 | 29 | 321 |
| 2021 Noviembre | 240 | 36 | 276 |
| 2021 Octubre | 275 | 38 | 313 |
| 2021 Septiembre | 268 | 39 | 307 |
| 2021 Agosto | 341 | 18 | 359 |
| 2021 Julio | 252 | 30 | 282 |
| 2021 Junio | 201 | 26 | 227 |
| 2021 Mayo | 111 | 22 | 133 |
| 2021 Abril | 160 | 38 | 198 |
| 2021 Marzo | 130 | 17 | 147 |
| 2021 Febrero | 111 | 25 | 136 |
| 2021 Enero | 56 | 9 | 65 |
| 2020 Diciembre | 33 | 7 | 40 |
| 2020 Noviembre | 27 | 8 | 35 |
| 2020 Octubre | 19 | 12 | 31 |
| 2020 Septiembre | 24 | 10 | 34 |
| 2020 Agosto | 23 | 20 | 43 |
| 2020 Julio | 28 | 11 | 39 |
| 2020 Junio | 34 | 10 | 44 |
| 2020 Mayo | 21 | 19 | 40 |
| 2020 Abril | 29 | 12 | 41 |
| 2020 Marzo | 29 | 13 | 42 |
| 2020 Febrero | 1 | 6 | 7 |
| 2020 Enero | 4 | 6 | 10 |
| 2019 Diciembre | 8 | 1 | 9 |
| 2019 Noviembre | 4 | 2 | 6 |
| 2019 Octubre | 0 | 1 | 1 |
| 2019 Septiembre | 4 | 2 | 6 |
| 2019 Agosto | 4 | 8 | 12 |
| 2019 Julio | 4 | 7 | 11 |
| 2019 Junio | 6 | 6 | 12 |
| 2019 Mayo | 6 | 29 | 35 |
| 2019 Abril | 4 | 20 | 24 |
| 2019 Marzo | 4 | 10 | 14 |
| 2019 Febrero | 0 | 5 | 5 |
| 2019 Enero | 4 | 1 | 5 |
| 2018 Diciembre | 0 | 5 | 5 |
| 2018 Noviembre | 3 | 0 | 3 |
| 2018 Octubre | 4 | 0 | 4 |
| 2018 Septiembre | 1 | 0 | 1 |
| 2018 Mayo | 0 | 2 | 2 |
| 2018 Marzo | 12 | 1 | 13 |
| 2018 Febrero | 118 | 2 | 120 |
| 2018 Enero | 55 | 6 | 61 |
| 2017 Diciembre | 42 | 8 | 50 |
| 2017 Noviembre | 33 | 2 | 35 |
| 2017 Octubre | 24 | 6 | 30 |
| 2017 Septiembre | 32 | 3 | 35 |
| 2017 Agosto | 36 | 3 | 39 |
| 2017 Julio | 21 | 4 | 25 |
| 2017 Junio | 32 | 10 | 42 |
| 2017 Mayo | 30 | 5 | 35 |
| 2017 Abril | 20 | 5 | 25 |
| 2017 Marzo | 12 | 2 | 14 |
| 2017 Febrero | 21 | 7 | 28 |
| 2017 Enero | 18 | 7 | 25 |
| 2016 Diciembre | 31 | 15 | 46 |
| 2016 Noviembre | 34 | 9 | 43 |
| 2016 Octubre | 32 | 15 | 47 |
| 2016 Septiembre | 37 | 11 | 48 |
| 2016 Agosto | 29 | 5 | 34 |
| 2016 Julio | 20 | 3 | 23 |
| 2016 Junio | 6 | 3 | 9 |
| 2016 Mayo | 9 | 3 | 12 |
| 2016 Abril | 3 | 0 | 3 |
| 2016 Marzo | 5 | 0 | 5 |
| 2016 Febrero | 11 | 4 | 15 |
| 2016 Enero | 21 | 8 | 29 |
| 2015 Diciembre | 18 | 1 | 19 |
| 2015 Noviembre | 11 | 9 | 20 |
| 2015 Octubre | 17 | 8 | 25 |
| 2015 Septiembre | 14 | 5 | 19 |
| 2015 Agosto | 14 | 0 | 14 |
| 2015 Julio | 33 | 5 | 38 |
| 2015 Junio | 18 | 2 | 20 |
| 2015 Mayo | 26 | 3 | 29 |
| 2015 Abril | 13 | 11 | 24 |
| 2015 Marzo | 12 | 7 | 19 |
| 2015 Febrero | 13 | 5 | 18 |
| 2015 Enero | 13 | 5 | 18 |
| 2014 Diciembre | 11 | 3 | 14 |
| 2014 Noviembre | 7 | 1 | 8 |
| 2014 Octubre | 13 | 2 | 15 |
| 2014 Agosto | 0 | 1 | 1 |
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