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1</a>&#41;&#46; No other similar skin lesions or palpable evidence of visceromegaly were observed&#46;</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&#46; The spindle cells were arranged in cellular bands and fascicles &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#44; A and B&#41;&#46; Immunohistochemical techniques showed that the cells were positive for vimentin and smooth muscle &#945;-actin &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2C</a>&#41; and negative for S100&#44; myoglobin&#44; cytokeratins&#44; and desmin &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2D</a>&#41;&#46;</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&#44; abdominal ultrasound&#44; and computed tomography revealed no signs of bone or visceral involvement&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Solitary infantile myofibromatosis&#46;</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&#46; At the time of writing&#44; the tumor continues to regress&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Infantile myofibromatosis &#40;IM&#41; is a congenital mesenchymal disorder characterized by the presence of solitary or multiple myofibroblastic tumors which may affect the skin&#44; soft tissues&#44; bones&#44; or internal organs&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> This disorder was first described by Stout in 1954&#44; although the currently used term was introduced in 1981 by Chung and Enzinger&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Though considered a rare disease&#44; IM is nonetheless the most common fibrous tumor in infancy&#46; These tumors habitually present between birth and 2 years of age&#44; and lesions limited to the skin tend to have a good prognosis with high rates of spontaneous regression&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Although most cases of IM are sporadic&#44; a familial form has been described in monozygotic twins and in successive generations&#59; it is possible that such cases may be attributable to an autosomal dominant inheritance pattern with variable penetrance&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The etiology is unknown&#46; Yousefi et al&#46;<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&#44; although in a study of tissue samples from 4 newborns with solitary or multiple IM&#44; the authors demonstrated that tumor cells were not derived from maternal chimeric cells&#46; On the other hand&#44; the level of maternal estrogen does appear to influence the development of these tumors&#44; as spontaneous regression occurs after delivery&#44; when the exposure to estrogens has ceased&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">There are 3 patterns of clinical presentation&#58; solitary IM &#40;a single lesion affecting the skin and&#47;or muscles in the head&#44; neck&#44; or trunk&#59; this pattern is the most common one in children&#44; representing 75&#37; of all cases&#41;&#59; multicentric IM without visceral involvement &#40;multiple lesions limited to the skin and muscles&#41;&#59; and multicentric IM with visceral or systemic involvement &#40;multiple lesions not only of the skin and&#47;or muscles&#44; but also of the bones&#44; lungs&#44; heart&#44; and gastrointestinal tract&#41;&#46;<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&#58; these lesions appear as a plaque&#44; nodule&#44; or mass&#59; are solitary or multiple&#59; measure from 0&#46;5 to 5<span class="elsevierStyleHsp" style=""></span>cm&#59; are not painful&#59; have a firm consistency&#59; rarely ulcerate or bleed&#59; and may have a keloid or vascular appearance&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Biopsy of lesions that are accessible&#8212;as is usually the case with skin lesions&#8212;is required to confirm the diagnosis&#46; IM skin tumors are well-defined dermal nodules exhibiting a biphasic growth pattern&#46; A high number of spindle cells arranged in fascicles &#40;smooth muscle&#8211;like fascicles&#41; can be seen on the periphery of the tumor&#59; these cells show no nuclear abnormalities&#44; although there may be occasional mitotic figures&#44; and they express smooth muscle &#945;-actin and vimentin and are negative for S100&#46; The central area contains vascular structures with irregular lumens and a hemangiopericytic pattern&#46;<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&#44; neurofibroma&#44; leiomyoma&#44; sarcoma&#44; and neuroblastoma metastasis&#46; From a histologic point of view&#44; a distinction must be made between IM and congenital fibrosarcoma and hemangiopericytoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Prognosis for solitary and multiple lesions that do not affect internal organs is excellent&#44; 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Case for Diagnosis
Ulcerated Congenital Tumor
Tumor congénito ulcerado
N. Martí-Fajardoa,
Autor para correspondencia
nuriamarfa@hotmail.com

Corresponding Author.
, C. Ortega-Monzóa, M. Navarro-Hervasb
a Servicio de Dermatología, Hospital Universitario de La Ribera, Alzira, Valencia, Spain
b Servicio de Anatomía Patológica, Hospital Universitario de La Ribera, Alzira, Valencia, Spain
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    "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&#46; She is the first child of healthy nonconsanguineous parents and was born following a full-term normal pregnancy and a normal delivery&#46; The mother had no history of infection&#44; drug use&#44; or family history of skin disease&#46;</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&#46; The nodule was ulcerated&#44; of fibrous consistency&#44; attached to the deeper layers&#44; and measured approximately 3<span class="elsevierStyleHsp" style=""></span>cm in diameter &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46; No other similar skin lesions or palpable evidence of visceromegaly were observed&#46;</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&#46; The spindle cells were arranged in cellular bands and fascicles &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#44; A and B&#41;&#46; Immunohistochemical techniques showed that the cells were positive for vimentin and smooth muscle &#945;-actin &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2C</a>&#41; and negative for S100&#44; myoglobin&#44; cytokeratins&#44; and desmin &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2D</a>&#41;&#46;</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&#44; abdominal ultrasound&#44; and computed tomography revealed no signs of bone or visceral involvement&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">What Is Your Diagnosis&#63;</p></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0025">Diagnosis</span><p id="par0030" class="elsevierStylePara elsevierViewall">Solitary infantile myofibromatosis&#46;</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&#46; At the time of writing&#44; the tumor continues to regress&#46;</p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Comment</span><p id="par0040" class="elsevierStylePara elsevierViewall">Infantile myofibromatosis &#40;IM&#41; is a congenital mesenchymal disorder characterized by the presence of solitary or multiple myofibroblastic tumors which may affect the skin&#44; soft tissues&#44; bones&#44; or internal organs&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> This disorder was first described by Stout in 1954&#44; although the currently used term was introduced in 1981 by Chung and Enzinger&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Though considered a rare disease&#44; IM is nonetheless the most common fibrous tumor in infancy&#46; These tumors habitually present between birth and 2 years of age&#44; and lesions limited to the skin tend to have a good prognosis with high rates of spontaneous regression&#46;</p><p id="par0045" class="elsevierStylePara elsevierViewall">Although most cases of IM are sporadic&#44; a familial form has been described in monozygotic twins and in successive generations&#59; it is possible that such cases may be attributable to an autosomal dominant inheritance pattern with variable penetrance&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">The etiology is unknown&#46; Yousefi et al&#46;<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&#44; although in a study of tissue samples from 4 newborns with solitary or multiple IM&#44; the authors demonstrated that tumor cells were not derived from maternal chimeric cells&#46; On the other hand&#44; the level of maternal estrogen does appear to influence the development of these tumors&#44; as spontaneous regression occurs after delivery&#44; when the exposure to estrogens has ceased&#46;<a class="elsevierStyleCrossRefs" href="#bib0005"><span class="elsevierStyleSup">1&#44;5</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">There are 3 patterns of clinical presentation&#58; solitary IM &#40;a single lesion affecting the skin and&#47;or muscles in the head&#44; neck&#44; or trunk&#59; this pattern is the most common one in children&#44; representing 75&#37; of all cases&#41;&#59; multicentric IM without visceral involvement &#40;multiple lesions limited to the skin and muscles&#41;&#59; and multicentric IM with visceral or systemic involvement &#40;multiple lesions not only of the skin and&#47;or muscles&#44; but also of the bones&#44; lungs&#44; heart&#44; and gastrointestinal tract&#41;&#46;<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&#58; these lesions appear as a plaque&#44; nodule&#44; or mass&#59; are solitary or multiple&#59; measure from 0&#46;5 to 5<span class="elsevierStyleHsp" style=""></span>cm&#59; are not painful&#59; have a firm consistency&#59; rarely ulcerate or bleed&#59; and may have a keloid or vascular appearance&#46;</p><p id="par0065" class="elsevierStylePara elsevierViewall">Biopsy of lesions that are accessible&#8212;as is usually the case with skin lesions&#8212;is required to confirm the diagnosis&#46; IM skin tumors are well-defined dermal nodules exhibiting a biphasic growth pattern&#46; A high number of spindle cells arranged in fascicles &#40;smooth muscle&#8211;like fascicles&#41; can be seen on the periphery of the tumor&#59; these cells show no nuclear abnormalities&#44; although there may be occasional mitotic figures&#44; and they express smooth muscle &#945;-actin and vimentin and are negative for S100&#46; The central area contains vascular structures with irregular lumens and a hemangiopericytic pattern&#46;<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&#44; neurofibroma&#44; leiomyoma&#44; sarcoma&#44; and neuroblastoma metastasis&#46; From a histologic point of view&#44; a distinction must be made between IM and congenital fibrosarcoma and hemangiopericytoma&#46;<a class="elsevierStyleCrossRefs" href="#bib0015"><span class="elsevierStyleSup">3&#44;5</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Prognosis for solitary and multiple lesions that do not affect internal organs is excellent&#44; with spontaneous remission occurring in 1 to 2 years&#44; probably related to massive apoptosis&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> A conservative wait-and-see approach is appropriate for these types of IM&#46; On the other hand&#44; IM with visceral involvement is serious and associated mortality is high&#44; especially when gastrointestinal and cardiorespiratory systems are compromised&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Patients with visceral disease therefore require surgical and&#47;or medical treatment &#40;e&#46;g&#46;&#44; radiation therapy or chemotherapy with vincristine&#44; actinomycin D&#44; and cyclophosphamide&#41; in addition to palliative care&#46;</p></span></span>"
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Información del artículo
ISSN: 15782190
Idioma original: Inglés
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