Case and Research Letter
Infantile Digital Fibromatosis: Primum Non Nocere
Fibromatosis digital infantil: primum non nocere
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A) Proliferación fusocelular que ocupa el espesor completo de la dermis (hematoxilina-eosina, ×4). B) Detalle a mayor aumento de las células fusiformes (hematoxilina-eosina, ×40). C) Inclusiones intracelulares realzadas mediante tinción tricrómica de Masson, ×40.</p>" ] ] ] "autores" => array:1 [ 0 => array:2 [ "autoresLista" => "E. Rodríguez-Lomba, B. Lozano-Masdemont, M. Campos-Domínguez" "autores" => array:3 [ 0 => array:2 [ "nombre" => "E." "apellidos" => "Rodríguez-Lomba" ] 1 => array:2 [ "nombre" => "B." "apellidos" => "Lozano-Masdemont" ] 2 => array:2 [ "nombre" => "M." 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"apellidos" => "Campos-Domínguez" ] ] ] ] ] "idiomaDefecto" => "es" "EPUB" => "https://multimedia.elsevier.es/PublicationsMultimediaV1/item/epub/S0001731021003677?idApp=UINPBA000044" "url" => "/00017310/0000011300000004/v1_202205250546/S0001731021003677/v1_202205250546/es/main.assets" ] ] "en" => array:15 [ "idiomaDefecto" => true "cabecera" => "<span class="elsevierStyleTextfn">Case and Research Letter</span>" "titulo" => " Infantile Digital Fibromatosis: <span class="elsevierStyleItalic">Primum Non Nocere</span>" "tieneTextoCompleto" => true "saludo" => "To the Editor," "paginas" => array:1 [ 0 => array:2 [ "paginaInicial" => "T432" "paginaFinal" => "T434" ] ] "autores" => array:1 [ 0 => array:4 [ "autoresLista" => "E. Rodríguez-Lomba, B. Lozano-Masdemont, M. Campos-Domínguez" "autores" => array:3 [ 0 => array:4 [ "nombre" => "E." "apellidos" => "Rodríguez-Lomba" "email" => array:1 [ 0 => "enriquerlomba@outlook.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" => "B." "apellidos" => "Lozano-Masdemont" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">b</span>" "identificador" => "aff0010" ] ] ] 2 => array:3 [ "nombre" => "M." "apellidos" => "Campos-Domínguez" "referencia" => array:1 [ 0 => array:2 [ "etiqueta" => "<span class="elsevierStyleSup">a</span>" "identificador" => "aff0005" ] ] ] ] "afiliaciones" => array:2 [ 0 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital General Universitario Gregorio Marañón, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Servicio de Dermatología, Hospital Universitario de Móstoles, Móstoles, Madrid, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Fibromatosis digital infantil: <span class="elsevierStyleItalic">primum non nocere</span>" ] ] "resumenGrafico" => array:2 [ "original" => 0 "multimedia" => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 678 "Ancho" => 1305 "Tamanyo" => 294909 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Histological findings. A, Proliferation of spindle cells occupying the entire thickness of the dermis (hematoxylin–eosin, original magnification ×4). B, Higher-magnification image of spindle cells (hematoxylin–eosin, original magnification ×40). C, Intracellular inclusions visualized using Masson trichrome stain (original magnification ×40).</p>" ] ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">Infantile digital fibromatosis is a mesenchymal tumor of myofibroblastic origin that usually occurs on the fingers and toes of children. Clinically, it presents as one or more generally asymptomatic, firm nodules. Its predominantly acral location and rapid growth can lead to a decrease or loss of motor function.</p><p id="par0010" class="elsevierStylePara elsevierViewall">A 4-year-old girl presented with an asymptomatic soft tissue tumor on the third finger of the right hand that was clinically and histologically compatible with infantile digital fibromatosis, for which she underwent plastic surgery (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>A). One month after the intervention, she presented with a new rapidly growing lesion on the fourth finger of the right hand (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a>B). She was treated with intralesional steroid injections, to which she responded poorly. The lesions resolved partially after a follow-up period of 1 year (<a class="elsevierStyleCrossRef" href="#fig0005">Fig. 1</a><span class="elsevierStyleSmallCaps">C</span>).</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0015" class="elsevierStylePara elsevierViewall">The second patient was a healthy 1-year-old boy who presented with a congenital soft-tissue tumor that was located on the third toe of the left foot and had grown progressively (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>A). Despite undergoing 2 surgeries in another center, including complete amputation of the third finger, the lesion had recurred after a few months (<a class="elsevierStyleCrossRef" href="#fig0010">Fig. 2</a>B). The patient was followed up at his reference hospital, and therefore we have no further data on his progression.</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">Infantile digital fibromatosis is considered a rare benign mesenchymal tumor that accounts for around 2.5% of all cases of fibromatosis.<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">1</span></a> Onset can occur at birth or in early childhood (86% of cases are described in the first year of life).</p><p id="par0025" class="elsevierStylePara elsevierViewall">Histology reveals circumscribed, nonencapsulated nodules located in the deep dermis and extending to the subcutaneous cellular tissue. Spindle cells grouped into fascicles intersect in an anarchic manner. The presence of intracellular and eosinophilic inclusion bodies that become more evident with immunohistochemical staining (e.g. Masson trichrome and Mallory phosphotungstic-acid hematoxylin) is pathognomonic<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">2</span></a> (<a class="elsevierStyleCrossRef" href="#fig0015">Fig. 3</a>A–C). However, the absence of these inclusion bodies does not rule out this diagnosis if other clinical and histological findings are compatible.</p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0030" class="elsevierStylePara elsevierViewall">These tumors generally stabilize and subsequently involute after a period of rapid growth lasting 10–14 months. There are multiple documented cases of spontaneous involution after stabilization, in some cases leading to complete lesion resolution in 6 months to 5 years.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">3,4</span></a> To date, there are no documented reports of distant metastasis.</p><p id="par0035" class="elsevierStylePara elsevierViewall">The differential diagnosis includes other causes of soft tissue tumors such as juvenile aponeurotic fibroma, pachydermodactyly, hypertrophic scars and keloids, and terminal osseous dysplasia. Unlike infantile digital fibromatosis, juvenile aponeurotic fibroma is an invasive tumor characterized by calcification, and usually occurs on the palms and soles, but not the fingers. Pachydermodactyly most commonly affects young adults and is usually associated with microtrauma. Terminal osseous dysplasia is an X-linked dominantly inherited syndrome with multifocal involvement and is not limited to the fingers.</p><p id="par0040" class="elsevierStylePara elsevierViewall">There is currently no clear consensus on the treatment of infantile digital fibromatosis. Surgical intervention with wide margins has traditionally been considered the first-line treatment. However, the recurrence rate is very high (61–74%) and the associated postoperative morbidity, as in the second case described here, may be prohibitive. Given the high number of documented cases of spontaneous involution, an initial conservative attitude is recommended.<a class="elsevierStyleCrossRefs" href="#bib0055"><span class="elsevierStyleSup">3,4</span></a> In symptomatic patients or those with an associated functional deficit, Mohs micrographic surgery may offer better results in terms of efficacy and postsurgical sequelae than conventional surgery with wide margins.<a class="elsevierStyleCrossRefs" href="#bib0065"><span class="elsevierStyleSup">5,6</span></a> Nonsurgical approaches include cryotherapy,<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">7</span></a> regular steroid infiltrations<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">8</span></a> and 5-fluorouracil, for which variable responses have been reported, as in the first of our 2 cases.</p><p id="par0045" class="elsevierStylePara elsevierViewall">In conclusion, infantile digital fibromatosis is a soft tissue tumor, treatment of which can be complex and should be preceded by a thorough assessment of the risks and benefits. In asymptomatic patients without significant functional deficits, we propose that clinical observation and nonsurgical treatment are likely the most appropriate therapeutic options.</p><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Conflicts of Interest</span><p id="par0050" class="elsevierStylePara elsevierViewall">The authors declare that they have no conflicts of interest.</p></span></span>" "textoCompletoSecciones" => array:1 [ "secciones" => array:2 [ 0 => array:2 [ "identificador" => "sec0005" "titulo" => "Conflicts of Interest" ] 1 => array:1 [ "titulo" => "References" ] ] ] "pdfFichero" => "main.pdf" "tienePdf" => true "multimedia" => array:3 [ 0 => array:7 [ "identificador" => "fig0005" "etiqueta" => "Figure 1" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr1.jpeg" "Alto" => 735 "Ancho" => 1305 "Tamanyo" => 156931 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">Patient 1. A, Indurated plaques on the third finger of the right hand. B, Recurrence on the fourth finger after intervention. C, Partial response after 1 year of steroid infiltration and clinical observation.</p>" ] ] 1 => array:7 [ "identificador" => "fig0010" "etiqueta" => "Figure 2" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr2.jpeg" "Alto" => 639 "Ancho" => 1305 "Tamanyo" => 96798 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Patient 2. A, Indurated pink nodule between the second and third toes of the left foot. B, Recurrence several months after complete excision and digital amputation.</p>" ] ] 2 => array:7 [ "identificador" => "fig0015" "etiqueta" => "Figure 3" "tipo" => "MULTIMEDIAFIGURA" "mostrarFloat" => true "mostrarDisplay" => false "figura" => array:1 [ 0 => array:4 [ "imagen" => "gr3.jpeg" "Alto" => 678 "Ancho" => 1305 "Tamanyo" => 294909 ] ] "descripcion" => array:1 [ "en" => "<p id="spar0015" class="elsevierStyleSimplePara elsevierViewall">Histological findings. A, Proliferation of spindle cells occupying the entire thickness of the dermis (hematoxylin–eosin, original magnification ×4). B, Higher-magnification image of spindle cells (hematoxylin–eosin, original magnification ×40). C, Intracellular inclusions visualized using Masson trichrome stain (original magnification ×40).</p>" ] ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0015" "bibliografiaReferencia" => array:8 [ 0 => array:3 [ "identificador" => "bib0045" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Infantile digital fibromatosis: a rare tumour of infancy. Report of five cases" "autores" => array:1 [ 0 => array:2 [ "etal" => false "autores" => array:5 [ 0 => "V. Girgenti" 1 => "L. Restano" 2 => "F. Arcangeli" 3 => "S. Cambiaghi" 4 => "C. 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| Year/Month | Html | Total | |
|---|---|---|---|
| 2024 November | 12 | 12 | 24 |
| 2024 October | 143 | 58 | 201 |
| 2024 September | 150 | 49 | 199 |
| 2024 August | 147 | 73 | 220 |
| 2024 July | 108 | 44 | 152 |
| 2024 June | 112 | 34 | 146 |
| 2024 May | 89 | 51 | 140 |
| 2024 April | 97 | 33 | 130 |
| 2024 March | 92 | 37 | 129 |
| 2024 February | 89 | 42 | 131 |
| 2024 January | 83 | 28 | 111 |
| 2023 December | 69 | 23 | 92 |
| 2023 November | 109 | 40 | 149 |
| 2023 October | 106 | 35 | 141 |
| 2023 September | 100 | 23 | 123 |
| 2023 August | 74 | 30 | 104 |
| 2023 July | 118 | 38 | 156 |
| 2023 June | 83 | 23 | 106 |
| 2023 May | 75 | 33 | 108 |
| 2023 April | 74 | 27 | 101 |
| 2023 March | 67 | 33 | 100 |
| 2023 February | 76 | 38 | 114 |
| 2023 January | 75 | 65 | 140 |
| 2022 December | 60 | 47 | 107 |
| 2022 November | 72 | 54 | 126 |
| 2022 October | 79 | 51 | 130 |
| 2022 September | 93 | 59 | 152 |
| 2022 August | 107 | 68 | 175 |
| 2022 July | 100 | 53 | 153 |
| 2022 June | 125 | 57 | 182 |
| 2022 May | 160 | 52 | 212 |
| 2022 April | 64 | 43 | 107 |
| 2022 March | 51 | 77 | 128 |
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