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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Kokubun" ] ] ] ] ] "host" => array:1 [ 0 => array:1 [ "Revista" => array:6 [ "tituloSerie" => "Ups J Med Sci" "fecha" => "2005" "volumen" => "110" "paginaInicial" => "259" "paginaFinal" => "266" "link" => array:1 [ 0 => array:2 [ "url" => "https://www.ncbi.nlm.nih.gov/pubmed/16454165" "web" => "Medline" ] ] ] ] ] ] ] ] 4 => array:3 [ "identificador" => "bib0025" "etiqueta" => "5" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Lipofibromatosis" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:1 [ 0 => "L. 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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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 12 | 12 | 24 |
| 2024 October | 55 | 33 | 88 |
| 2024 September | 66 | 18 | 84 |
| 2024 August | 102 | 43 | 145 |
| 2024 July | 57 | 27 | 84 |
| 2024 June | 85 | 51 | 136 |
| 2024 May | 53 | 26 | 79 |
| 2024 April | 60 | 19 | 79 |
| 2024 March | 51 | 25 | 76 |
| 2024 February | 56 | 33 | 89 |
| 2024 January | 39 | 30 | 69 |
| 2023 December | 34 | 18 | 52 |
| 2023 November | 39 | 21 | 60 |
| 2023 October | 45 | 17 | 62 |
| 2023 September | 49 | 23 | 72 |
| 2023 August | 33 | 20 | 53 |
| 2023 July | 36 | 27 | 63 |
| 2023 June | 49 | 19 | 68 |
| 2023 May | 50 | 17 | 67 |
| 2023 April | 47 | 18 | 65 |
| 2023 March | 41 | 32 | 73 |
| 2023 February | 50 | 20 | 70 |
| 2023 January | 38 | 28 | 66 |
| 2022 December | 45 | 38 | 83 |
| 2022 November | 48 | 21 | 69 |
| 2022 October | 59 | 26 | 85 |
| 2022 September | 100 | 39 | 139 |
| 2022 August | 179 | 18 | 197 |
| 2022 July | 104 | 36 | 140 |
| 2022 June | 174 | 30 | 204 |
| 2022 May | 418 | 37 | 455 |
| 2022 April | 445 | 40 | 485 |
| 2022 March | 359 | 42 | 401 |
| 2022 February | 391 | 20 | 411 |
| 2022 January | 443 | 33 | 476 |
| 2021 December | 292 | 29 | 321 |
| 2021 November | 240 | 36 | 276 |
| 2021 October | 275 | 38 | 313 |
| 2021 September | 268 | 39 | 307 |
| 2021 August | 341 | 18 | 359 |
| 2021 July | 252 | 30 | 282 |
| 2021 June | 201 | 26 | 227 |
| 2021 May | 111 | 22 | 133 |
| 2021 April | 160 | 38 | 198 |
| 2021 March | 130 | 17 | 147 |
| 2021 February | 111 | 25 | 136 |
| 2021 January | 56 | 9 | 65 |
| 2020 December | 33 | 7 | 40 |
| 2020 November | 27 | 8 | 35 |
| 2020 October | 19 | 12 | 31 |
| 2020 September | 24 | 10 | 34 |
| 2020 August | 23 | 20 | 43 |
| 2020 July | 28 | 11 | 39 |
| 2020 June | 34 | 10 | 44 |
| 2020 May | 21 | 19 | 40 |
| 2020 April | 29 | 12 | 41 |
| 2020 March | 29 | 13 | 42 |
| 2020 February | 1 | 6 | 7 |
| 2020 January | 4 | 6 | 10 |
| 2019 December | 8 | 1 | 9 |
| 2019 November | 4 | 2 | 6 |
| 2019 October | 0 | 1 | 1 |
| 2019 September | 4 | 2 | 6 |
| 2019 August | 4 | 8 | 12 |
| 2019 July | 4 | 7 | 11 |
| 2019 June | 6 | 6 | 12 |
| 2019 May | 6 | 29 | 35 |
| 2019 April | 4 | 20 | 24 |
| 2019 March | 4 | 10 | 14 |
| 2019 February | 0 | 5 | 5 |
| 2019 January | 4 | 1 | 5 |
| 2018 December | 0 | 5 | 5 |
| 2018 November | 3 | 0 | 3 |
| 2018 October | 4 | 0 | 4 |
| 2018 September | 1 | 0 | 1 |
| 2018 May | 0 | 2 | 2 |
| 2018 March | 12 | 1 | 13 |
| 2018 February | 118 | 2 | 120 |
| 2018 January | 55 | 6 | 61 |
| 2017 December | 42 | 8 | 50 |
| 2017 November | 33 | 2 | 35 |
| 2017 October | 24 | 6 | 30 |
| 2017 September | 32 | 3 | 35 |
| 2017 August | 36 | 3 | 39 |
| 2017 July | 21 | 4 | 25 |
| 2017 June | 32 | 10 | 42 |
| 2017 May | 30 | 5 | 35 |
| 2017 April | 20 | 5 | 25 |
| 2017 March | 12 | 2 | 14 |
| 2017 February | 21 | 7 | 28 |
| 2017 January | 18 | 7 | 25 |
| 2016 December | 31 | 15 | 46 |
| 2016 November | 34 | 9 | 43 |
| 2016 October | 32 | 15 | 47 |
| 2016 September | 37 | 11 | 48 |
| 2016 August | 29 | 5 | 34 |
| 2016 July | 20 | 3 | 23 |
| 2016 June | 6 | 3 | 9 |
| 2016 May | 9 | 3 | 12 |
| 2016 April | 3 | 0 | 3 |
| 2016 March | 5 | 0 | 5 |
| 2016 February | 11 | 4 | 15 |
| 2016 January | 21 | 8 | 29 |
| 2015 December | 18 | 1 | 19 |
| 2015 November | 11 | 9 | 20 |
| 2015 October | 17 | 8 | 25 |
| 2015 September | 14 | 5 | 19 |
| 2015 August | 14 | 0 | 14 |
| 2015 July | 33 | 5 | 38 |
| 2015 June | 18 | 2 | 20 |
| 2015 May | 26 | 3 | 29 |
| 2015 April | 13 | 11 | 24 |
| 2015 March | 12 | 7 | 19 |
| 2015 February | 13 | 5 | 18 |
| 2015 January | 13 | 5 | 18 |
| 2014 December | 11 | 3 | 14 |
| 2014 November | 7 | 1 | 8 |
| 2014 October | 13 | 2 | 15 |
| 2014 August | 0 | 1 | 1 |
Show all
