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2.
Braz J Otorhinolaryngol ; 89(4): 101270, 2023.
Artículo en Inglés | MEDLINE | ID: mdl-37331237

RESUMEN

OBJECTIVE: This study aims to perform a meta-analysis to figure out the efficacy of OK-432 sclerotherapy between Macrocystic (MAC) lymphangiomas and Microcystic (MIC) lymphangiomas. METHODS: We conducted a systematic review and meta-analysis to clarify the relationship between OK-432 and lymphangiomas. PubMed and ISI Web of Science were searched from inception to May 2022. Joanna Briggs Institute (JBI) manual was used to evaluate the risk of bias. We calculated pooled Relative Risks (RR) and 95% Confidence Interval (95% CI) using random effects model to evaluate the relations between OK-432 and lymphangiomas. RESULTS: A total of 11 studies (including 352 cases) about OK-432 sclerotherapy for lymphangioma were included in the current meta-analyses. The results suggested that the efficacy of OK-432 was significantly in MAC lesions than in MIC (RR=1.51, 95% CI 1.298-1.764), with significant moderate degrees of heterogeneity among 11 studies (I2=51.2%, p=0.025). Subgroup analyses suggested that there was significant association in both retrospective studies (RR=1.26, 95% CI 1.03-1.53) and classification (by 1 cm) (RR=1.37, 95% CI 1.04-1.80) were associated with the efficacy of OK-432. CONCLUSION: To our knowledge, our study represents the first meta-analysis examining the efficacy of OK-432 in the treatment of different types of LMs. However, the regional differences and the age differences of the subjects are the main limitations of this study and should be avoided in further research. Our results suggested that OK-432 sclerotherapy for macrocystic lymphangiomas was more effective.


Asunto(s)
Quistes , Linfangioma , Humanos , Picibanil/uso terapéutico , Escleroterapia/métodos , Estudios Retrospectivos , Linfangioma/terapia , Resultado del Tratamiento
3.
Rev. otorrinolaringol. cir. cabeza cuello ; 83(1): 37-40, mar. 2023. ilus
Artículo en Español | LILACS | ID: biblio-1431951

RESUMEN

Los linfangiomas son malformaciones linfáticas benignas infrecuentes; se manifiestan principalmente en la infancia. Pueden desarrollarse en cualquier zona del cuerpo, más frecuentemente cabeza y cuello, siendo su compromiso faríngeo extremadamente poco común. Dada su clínica inespecífica se requiere un análisis anatomopatológico preciso para concretar un correcto diagnóstico. Presentamos el caso de una mujer de 40 años con historia de disfagia y odinofagia de un año de evolución cuyo estudio imagenológico y con nasofibroscopía evidenció un tumor redondeado alojado en seno piriforme derecho, la biopsia excisional confirmó el diagnóstico de linfangioma. Se debe considerar el diagnóstico de linfangioma dentro de los diagnósticos diferenciales de tumores en faringe y concientizar su presencia en pacientes adulto, para ofrecer el manejo óptimo basado, ya sea en escisión quirúrgica completa, para evitar la recurrencia asociado, o al uso de sustancias esclerosantes.


Lymphangiomas are infrequent benign lymphatic malformations, with clinical manifestations mainly at birth or in childhood. They can develop in any area of the body, most frequently the head and neck, and their pharyngeal involvement is extremely rare. Given its nonspecific symptoms, it requires a precise pathological analysis to make a correct diagnosis. We present the case of a 40-year-old woman with a 1-year history of dysphagia and odynophagia whose imaging study and nasofibroscopy revealed a rounded tumor lodged in the right pyriform sinus. The excisional biopsy confirmed the diagnosis of lymphangioma. The diagnosis of lymphangioma should be considered within the differentials of tumors in the pharynx and awareness of its presence in adults to offer optimal management based on complete surgical excision to avoid recurrence or management with use of sclerosing substances.


Asunto(s)
Humanos , Femenino , Adulto , Neoplasias Hipofaríngeas/diagnóstico por imagen , Linfangioma/diagnóstico por imagen , Biopsia , Neoplasias Hipofaríngeas/patología , Tomografía Computarizada por Rayos X/métodos , Linfangioma/patología
4.
Med. lab ; 27(1): 33-38, 2023. ilus
Artículo en Español | LILACS | ID: biblio-1412750

RESUMEN

El hemangiolinfangioma es un tipo muy raro de malformación del sistema vascular, caracterizado histológicamente por la presencia de vasos venosos y linfáticos dilatados quísticamente, cuyas células endoteliales de revestimiento son positivas para marcadores de inmunohistoquímica como CD31, CD34 y D2-40. El compromiso extenso retroperitoneal y del tracto gastrointestinal es infrecuente. Se presenta el caso de una paciente femenina de 24 años con antecedente de dolor pélvico crónico, con exacerbación de síntomas. El diagnóstico imagenológico mostró una masa retroperitoneal multiquística. Se hizo hemicolectomía derecha y resección de la masa, encontrándose que dicha lesión estaba íntimamente adherida al mesenterio con compromiso extenso del tracto gastrointestinal, y cuyo estudio histopatológico reveló un hemangiolinfangioma, con mejoría clínica posterior a la resección quirúrgica. Aportamos a la literatura mundial, la caracterización de los hallazgos clínicos, imagenológicos e histopatológicos de este tipo de malformaciones


Hemangiolymphangioma is a very rare type of malformation of the vascular system, characterized histologically by the presence of cystically dilated venous and lymphatic vessels, whose lining endothelial cells are positive for immunohistochemical markers such as CD31, CD34 and D2-40. Extensive retroperitoneal and gastrointestinal tract involvement is uncommon. We present the case of a 24-yearold female patient with a history of chronic pelvic pain with exacerbation of symptoms. The imaging diagnosis revealed a multicystic retroperitoneal mass. A right hemicolectomy and resection of the mass was performed, finding that the lesion was intimately adherent to the mesentery with extensive involvement of the gastrointestinal tract, and whose histopathological study revealed a hemangiolymphangioma, with clinical improvement after surgical resection. We contribute to the world literature with the characterization of the clinical, imaging and histopathological findings of this type of malformations


Asunto(s)
Humanos , Femenino , Adulto Joven , Neoplasias Peritoneales/diagnóstico , Hemangioma/diagnóstico , Linfangioma/diagnóstico , Mesenterio/patología , Neoplasias Peritoneales/cirugía , Neoplasias Peritoneales/patología , Células Endoteliales/patología , Malformaciones Vasculares/diagnóstico , Malformaciones Vasculares/patología , Hemangioma/cirugía , Hemangioma/patología , Linfangioma/cirugía , Linfangioma/patología
5.
Rev. cuba. oftalmol ; 35(4)dic. 2022.
Artículo en Español | LILACS, CUMED | ID: biblio-1441765

RESUMEN

Los linfangiomas orbitarios son malformaciones vasculares benignas, de crecimiento lento, abortivas y no funcionales, que se presentan principalmente en la primera década de la vida. Las opciones terapéuticas en estos casos presentan una resolutividad limitada, algunos tratamientos suelen ser agresivos y provocar daños del aparato visual. Se presenta un caso de una paciente femenina de 6 años de edad atendida por proptosis del ojo izquierdo a la que se le realizó el diagnóstico clínico-imagenológico de linfangioma de la órbita, con el objetivo de mostrar el resultado alcanzado en el manejo de la misma mediante el uso del sildenafilo por vía oral, modalidad terapéutica en estudio a nivel mundial en el tratamiento de estas afecciones. El tratamiento con sildenafilo en el linfangioma orbitario demostró ser eficaz en la mejoría del cuadro clínico y por imágenes. Durante el tratamiento no se reportaron reacciones adversas(AU)


Orbital lymphatic malformations are benign, slow-growing, abortive, nonfunctional vascular malformations that occur mainly in the first decade of life. Therapeutic options in these cases present limited resolution, some treatments are usually aggressive and cause damage to the visual apparatus. We present a case of a 6-year-old female patient treated for proptosis of the left eye. The clinical-imaging diagnosis of lymphangioma of the orbit was made to show the results achieved in its treatment through the use of oral sildenafil, a therapeutic modality under study worldwide in the treatment of these conditions. The treatment with sildenafil in orbital lymphangioma proved to be effective in the improvement of the clinical and imaging picture. No adverse reactions were reported during treatment(AU)


Asunto(s)
Humanos , Femenino , Niño , Malformaciones Vasculares/terapia , Linfangioma/etiología
7.
Oral Maxillofac Surg ; 26(1): 171-176, 2022 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-34089420

RESUMEN

We present a challenging case of HIV-related lymphangioma-like Kaposi sarcoma (LLKS) affecting the oral cavity. A 54-year-old Brazilian male patient was referred to our center complaining of bleeding lesions affecting the oral cavity for 2 months. Interestingly, these oral lesions were the first clinical manifestation of HIV infection. Clinically, multiple erythematous nodular and patch lesions were observed. An incisional biopsy was performed, revealing numerous microscopic angled and irregular vascular channels lined with flattened endothelial cells. More cellularized and solid areas consisting of more fusiform cells with little pleomorphism and with slit-like vascular channels were noted. Based on immunoreactivity for CD31, CD34, D2-40, and HHV-8, the final diagnosis was oral LLKS. Highly active antiretroviral therapy (HAART) was initiated with dolutegravir, tenofovir, and lamivudine. During follow-up, the patient showed KS metastases to other sites and a chemotherapeutic protocol was initiated. Regression of the oral lesion was clearly noted by the clinicians 1 year after the KS diagnosis. Dentists should be able to recognize systemic diseases that affect the oral cavity such as KS in order to make an early diagnosis of its oral manifestations and to implement effective therapeutic measures to ensure a better prognosis.


Asunto(s)
Infecciones por VIH , Linfangioma , Sarcoma de Kaposi , Terapia Antirretroviral Altamente Activa , Células Endoteliales , Infecciones por VIH/diagnóstico , Infecciones por VIH/tratamiento farmacológico , Humanos , Masculino , Persona de Mediana Edad , Sarcoma de Kaposi/tratamiento farmacológico
8.
J Ultrasound Med ; 41(4): 1019-1026, 2022 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-34288011

RESUMEN

Fetal lymphangioma is an uncommon congenital malformation that is mainly comprised of the subcutaneous tissue of the neck. This malformation can develop in other areas like the thoracic and axillary regions, though rarely. We report 6 consecutive cases of lymphatic malformation in a fetal center in Dominican Republic. In our case series fetal chest lymphangiomas were present in 2 fetuses. In addition, 2 cases of axillary lymphangiomas also involved the thoracic region. Adequate management by a multidiciplinary team is necessary to provide a better approach to delivery.


Asunto(s)
Linfangioma , Anomalías Linfáticas , Femenino , Feto , Humanos , Linfangioma/diagnóstico por imagen , Embarazo , Diagnóstico Prenatal , Ultrasonografía Prenatal
10.
Rev. colomb. cir ; 37(2): 245-250, 20220316. tab, fig
Artículo en Español | LILACS | ID: biblio-1362958

RESUMEN

Introducción. Los linfangiomas son anormalidades benignas del sistema linfático, que corresponden a dilataciones quísticas de estos vasos y se localizan especialmente en el cuello. Solo el 10 % de todas estas malformaciones se encuentran en el abdomen y presentan síntomas variables de acuerdo al tamaño y su ubicación especifica, siendo el dolor abdominal el principal síntoma. Métodos. Se presentan cinco pacientes pediátricos con malformaciones linfáticas abdominales. Se describen su cuadro clínico, localización, tratamiento y la experiencia en el manejo de dicha patología en un hospital de referencia. Resultados. Los métodos más apropiados para hacer una aproximación diagnóstica son la ecografía, la tomografía computarizada y la resonancia nuclear magnética. Dentro de las opciones descritas para el tratamiento están la farmacológica, la escleroterapia y la resección quirúrgica, tanto por vía abierta como por laparoscopia. Conclusión. Existe una variedad de métodos para realizar la resección de los linfangiomas abdominales, pero la cirugía sigue siendo la más efectiva, especialmente cuando se cuenta con la laparoscopia como una herramienta terapéutica.


Introduction. Lymphangiomas are benign abnormalities of the lymphatic system, which correspond to cystic dilations of these vessels and are located especially in the neck. Only 10% of all these malformations are found in the abdomen and present variable symptoms according to size and their specific location, with abdominal pain being the main symptom. Methods. Five pediatric patients with abdominal lymphatic malformations are presented. Their clinical presentation, location, treatment and experience in the management of this pathology in a referral hospital are described. Results. The most appropriate methods to make a diagnostic approach are ultrasound, computed tomography and magnetic resonance imaging. Among the options described for treatment are pharmacological, sclerotherapy and surgical resection, both open and laparoscopic. Conclusion. There are a variety of methods for resecting abdominal lymphangiomas, but surgery remains the most effective, especially when laparoscopy is used as a therapeutic tool.


Asunto(s)
Humanos , Linfangioma , Enfermedades Linfáticas , Escleroterapia , Laparoscopía , Sistema Linfático
11.
Cambios rev. méd ; 20(2): 53-59, 30 Diciembre 2021. ilus, tabs.
Artículo en Español | LILACS | ID: biblio-1368287

RESUMEN

INTRODUCCIÓN. Los linfangiomas son una malformación infrecuente a escala mundial y constituye una preocupación para los padres del infante; está asociado a problemas estéticos y a posibles efectos deletéreos debido a la obstrucción o compresión de órganos vitales. Se ha descrito a la escleroterapia como la mejor opción de tratamiento. OBJETIVO. Determinar la eficiencia del tratamiento con bleomicina en linfangiomas en la población pediátrica de 0 a 18 años. MATERIALES Y MÉTODOS. Estudio transversal analítico retrospectivo. Población y muestra conocida de 20 datos de Historias Clínicas electrónicas de pacientes diagnosticados con linfangiomas y tratados con bleomicina en el Hospital de Especialidades Carlos Andrade Marín, desde enero 2015 a enero 2018. Criterios de inclusión: pacientes de 0 a 18 años de edad con diagnóstico de linfangioma mediante ecografía y angiotomografía computarizada. Criterios de exclusión: pacientes mayores de 18 años de edad o sospecha diagnóstica de linfangioma sin estudios de imagen, y/o que no acudieron a la cita de control, pacientes diagnosticados de linfangioma que no recibieron bleomicina para su tratamiento, niños con otras malformaciones vasculares. El análisis de datos se realizó en el programa estadístico International Business Machines Statistical Package for the Social Sciences. RESULTADOS. La mediana de edad en mujeres fue de 6,25 años y 3,8 años en hombres. 10 pacientes fueron hombres. El promedio de seguimiento fue de 26,86 +/- 16,78 meses. El tamaño promedio de los linfangiomas fue de 5,77 +/- 3,73 cm. La localización más frecuente fue cervical con un 52,38%. La mayoría fueron macronodulares con un 85,71%. La respuesta fue buena o excelente en el 81,00% de los casos con la primera infiltración y subió al 95,00% con la segunda y tercera, según requerimiento. CONCLUSIÓN. El tratamiento de los linfangiomas con bleomicina fue muy efectivo en la población estudiada.


INTRODUCTION. Lymphatic malformation is a rare malformation worldwide and is a concern for the parents of the infant; it is associated with aesthetic problems and possible deleterious effects due to obstruction or compression of vital organs. Sclerotherapy has been described as the best treatment option. OBJECTIVE. To determine the efficiency of bleomycin treatment in lymphangiomas in the pediatric population aged 0 to 18 years. MATERIALS AND METHODS. Retrospective analytical cross-sectional study. Population and known sample of 20 data from Electronic Medical Records of patients diagnosed with lymphangiomas and treated with bleomycin at the Carlos Andrade Marín Specialties Hospital, from January 2015 to January 2018. Inclusion criteria: patients aged 0 to 18 years with diagnosis of lymphangioma by ultrasound and computed angiotomography. Exclusion criteria: patients older than 18 years of age or diagnostic suspicion of lymphangioma without imaging studies, and/or who did not attend the control appointment, patients diagnosed with lymphangioma who did not receive bleomycin for treatment, children with other vascular malformations. Data analysis was performed in the statistical program International Business Machines Statistical Package for the Social Sciences. RESULTS. The median age in women was 6,25 years and 3,8 years in men. Ten patients were men. The average follow-up was 26,86 +/- 16,78 months. The average size of the lymphatic malformations was 5,77 +/- 3,73 cm. The most frequent location was cervical with 52,38%. Most were macronodular with 85,71%. The response was good or excellent in 81,00% of cases with the first infiltration and rose to 95,00% with the second and third, as required. CONCLUSION. The treatment of lymphangiomas with bleomycin was very effective in the population studied.


Asunto(s)
Humanos , Masculino , Femenino , Lactante , Preescolar , Niño , Adolescente , Bleomicina/uso terapéutico , Extremidad Inferior , Extremidad Superior , Neoplasias de Cabeza y Cuello/tratamiento farmacológico , Linfangioma/tratamiento farmacológico , Antibióticos Antineoplásicos/uso terapéutico , Axila , Estudios Transversales , Estudios Retrospectivos , Resultado del Tratamiento , Cuello
12.
Belo Horizonte; s.n; 2021. 78 p. ilus, tab.
Tesis en Portugués | BBO - Odontología | ID: biblio-1382197

RESUMEN

As malformações linfáticas orais são anomalias vasculares incomuns. Portanto, compreender suas principais características clínicas e demográficas pode auxiliar o entendimento e diagnóstico dessas lesões. A partir de um estudo multicêntrico e revisão de literatura, este trabalho avaliou a ocorrência e analisou as características demográficas e clínicas de malformações linfáticas orais. O estudo multicêntrico foi aprovado pelo Comitê de Ética em Pesquisa da Universidade Federal de Minas Gerais (parecer nº 3.313.870 e CAAE 10723019.0.1001.5149) e replicado para os Comitês de Ética dos demais centros. Foram acessados os arquivos de biópsia de sete laboratórios brasileiros de Patologia Oral e Bucomaxilofacial, compreendendo 228.150 laudos, os quais eram datados entre o ano de inauguração do centro e 2018. Foram selecionados os casos com diagnóstico histopatológico de malformação linfática oral. Adicionalmente, foi realizada uma revisão de literatura sobre o tema. Nesta, a partir de uma estratégia de busca estruturada, foram selecionados os casos de malformação linfática oral reportados em língua inglesa. Posteriormente foram extraídos dados similares aos do estudo multicêntrico, permitindo sua comparação. A análise dos dados foi realizada no programa estatístico SPSS versão 22.0. Para estatísticas analíticas o nível de significância foi estabelecido em p<0,05. No total, o estudo multicêntrico compreendeu 208 casos, representando 0,09% de todo o arquivo investigado, e a revisão de literatura 1035 casos. Em ambos houve concordância de que há distribuição semelhante entre os sexos e a maioria acomete a língua. O estudo multicêntrico e os estudos de relato de casos apresentaram idade mediana de 22 e 10 anos, respetivamente. Já o tamanho das lesões e proporção de pacientes sintomáticos foi maior entre os casos da revisão de literatura. Ao avaliar tais divergências, foi observado que em ambos houve associação estatisticamente significativa (p<0,05) entre idade e tamanho de lesão, em que pacientes mais jovens tiveram lesões maiores. O presente estudo multicêntrico conta com a maior amostra de malformações linfáticas orais até o momento e revelou que este diagnóstico foi incomum em todos os centros participantes. O estudo multicêntrico e a revisão de literatura mostraram que homens e mulheres são afetados igualmente, o sítio preferencial de acometimento é a língua e há relação entre idade do paciente e tamanho da lesão.


Oral lymphatic malformations are uncommon vascular anomalies. Thus, recognizing the main demographical and clinical characteristics of oral lymphatic malformation can help to understand and diagnose these lesions. This multicenter study and literature review assessed the occurrence, clinical and demographic characteristics of oral lymphatic malformation. The multicenter study was approved by the Research Ethics Committee from Universidade Federal de Minas Gerais (CAAE 10723019.0.1001.5149) and replicated to the Ethics Committees of the other centers. The biopsy files from seven Oral and Bucomaxilofacial Pathology Services were acessed. All cases with histopathological diagnosis of lymphatic malformation dated from 1953 to 2018 were selected. Additionally, was performed a literature review, based on a structured search strategy. Were selected cases of oral lymphatic malformation in the English language. Data extracted were similar to those from the multicenter study, allowing their comparison. Data analysis was performed with the software Statistical Package for the Social Sciences (SPSS), version 22.0 (SPSS Inc., Armonk, USA). The level of significance was set at p< 0.05 for analytical statistics. The multicenter study retrieved 208 cases, representing 0.09% of the total sample. The literature review retrieved 1035 cases. Both affected male and female individuals equally and most cases were at tongue. The multicenter study and the case report studies showed median age of 22 and 10 years, respectively. On the other hand, the lesion size and proportion of symptomatic patients were higher among cases from the literature review. Cases, in both multicenter study and the literature review, revealed an association (p<0.05) among age and lesion size, showing young patients with larger lesions. This multicenter study represented the larger sample of oral lymphatic malformations to date and evidenced that this is an uncommon diagnosis in Brazilian Oral and Bucomaxilofacial Pathology Services. The multicenter study and the literature review showed that men and women are affected equally, the main location site is the tongue and patient age is related to the lesion size.


Asunto(s)
Revisión , Estudio Multicéntrico , Anomalías Linfáticas , Diagnóstico , Linfangioma
13.
Belo Horizonte; s.n; 2021. 76 p. ilus, tab.
Tesis en Portugués | BBO - Odontología | ID: biblio-1391487

RESUMEN

As malformações linfáticas são distúrbios do desenvolvimento caracterizados pelo crescimento excessivo de vasos linfáticos. Essa condição acontece principalmente em região de cabeça e pescoço devido à presença fisiológica de grande concentração de vasos linfáticos na região. As malformações linfáticas podem ocorrer de maneira isolada ou associadas a síndromes como de CLOVES e Klippel-Trènaunay. A presença de mutações no gene PIK3CA e a ativação das vias PI3K/AKT e MAPK/ERK pode ocorrer tanto nas malformações linfáticas esporádicas, como naquelas associadas a síndromes. As mutações em PIK3CA ocorrem nos éxons 10 e 20, sendo as mutações no códon 1047 (éxon 20) as mais frequente nas malformações linfáticas esporádicas. No entanto, em malformações linfáticas orais não se sabe se estes fenômenos ocorrem. O objetivo deste estudo foi avaliar a presença de mutações no códon 1047 do gene PIK3CA e avaliar a ativação das vias PI3K/AKT e MAPK/ERK em amostras de malformações linfáticas orais. Uma amostra de conveniência de 14 tecidos de malformações linfáticas orais fixados em formol e embebidos em parafina foram submetidas a reações de imuno-histoquímica para as formas fosforiladas de AKT1 (pAKT-Ser473) e ERK1/2 (pERK1/2-Thr202/Tyr204), marcadores de ativação das vias PI3K/AKT e MAPK/ERK, respectivamente. Quatro destas amostras foram submetidas a Sequenciamento de Sanger para o códon 1047 do gene PIK3CA. Foram observados padrões de marcação positivas para pAKT1 e pERK1/2 nas células endoteliais de todas as amostras de malformações linfáticas orais avaliadas. Todas as amostras submetidas ao Sequenciamento de Sanger apresentaram sequência selvagem para a região de interesse. Com os resultados obtidos sugere-se que as vias PI3K/AKT e MAPK/ERK estão envolvidas na patogênese das malformações linfáticas orais.


Lymphatic malformations are developmental disorders characterized by excessive growth of lymphatic vessels. This condition occurs mainly in the head and neck region due to the physiological presence of high concentration of lymphatic vessels in the region. Lymphatic malformations may occur in isolation or associated with syndromes such as CLOVES and Klippel-Trènaunay. Mutations in the PIK3CA gene and activation of the PI3K/AKT and MAPK/ERK pathways may occur both in sporadic lymphatic malformations and in those associated with syndromes. Mutations in hotspot 1047 are the most frequently found in isolated lymphatic malformation. However, in oral lymphatic malformations it is not known whether these mutations occur. The aim of this study was to evaluate the presence of mutations in codon 1047 of the PIK3CA gene and to evaluate the activation of PI3K/AKT and MAPK/ERK pathways in samples of oral lymphatic malformations. A convenience sample of 14 tissues of oral lymphatic malformations formalin fixed paraffin embedded were submitted to immunohistochemistry reactions to the phosphorylated forms of AKT1 (pAKT-Ser473) and ERK1/2 (pERK1/2-Thr202/Tyr204), activation markers of PI3K/AKT and MAPK/ERK pathways, respectively. Four of these samples were submitted to Sanger Sequencing for codon 1047 of the PIK3CA gene. Positive marking patterns for pAKT1 and pERK1/2 were observed in the endothelial cells of all samples of oral lymphatic malformations evaluated. All samples submitted to Sanger Sequencing were wild type for the region of interest. With the results obtained it is suggested that the PI3K/AKT and MAPK/ERK pathways are involved in the pathogenesis of oral lymphatic malformations.


Asunto(s)
Inmunohistoquímica , Transducción de Señal , Anomalías Linfáticas , Linfangioma
14.
Autops. Case Rep ; 11: e2021290, 2021. graf
Artículo en Inglés | LILACS | ID: biblio-1249028

RESUMEN

Lymphangiomas are rare and correspond to 0.7% to 4.0% of mediastinal tumors, and isolated mediastinal location occurs in 1% of cases. They are benign tumors that originate from a congenital malformation of the lymphatic vessels and are diagnosed more frequently in children less than 2 years of age. Chylous ascites is a clinical manifestation of thoracic duct lymphangioma and is composed of lymph accumulation caused by dilation of this lymphatic channel. It appears milky in the peritoneal cavity, containing triglyceride levels higher than 200 mg/dl. We report the case of a young patient with chylous ascites and lymphangioma of the thoracic duct, who was conservatively treated with octreotide and a low-fat diet with medium-chain triglycerides.


Asunto(s)
Humanos , Femenino , Adulto , Ascitis Quilosa/terapia , Linfangioma/terapia , Neoplasias Abdominales/terapia , Conducto Torácico/patología , Octreótido
15.
Rev. cuba. med. mil ; 49(4): e603, graf
Artículo en Español | LILACS, CUMED | ID: biblio-1156524

RESUMEN

Introducción: La linfangiectasia renal es una afección infrecuente en la que existe dilatación de los vasos linfáticos renales. Usualmente es bilateral y de buen pronóstico. Objetivos: Presentar un caso de linfangiectasia renal bilateral, sus características clínicas y aspectos del diagnóstico imaginológico. Caso clínico: Paciente femenina de 59 años de edad, hipertensa controlada, que asistió a la consulta externa de urología por dolor lumbar bilateral, de moderada intensidad y coloración rojiza de la orina, ambos de forma intermitente. El examen físico general y regional fue negativo. Se confirmó microhematuria en el examen general de la orina. Los estudios de la analítica sanguínea fueron normales. El ultrasonido renal reportó pielocaliectasia bilateral moderada y quistes parapiélicos, bilaterales, a predominio izquierdo. La tomografía axial computarizada renal contrastada, reveló la presencia de formaciones hipodensas renales bilaterales, de aspecto quístico parapiélicas y en el seno renal, compatibles con linfangiectasia bilateral. La paciente ha evolucionado favorablemente. Conclusiones: La linfangiectasia renal se debe tener en cuenta en el diagnóstico deferencial de la enfermedad quística renal. Para el diagnóstico imaginológico de certeza es esencial la tomografía axial computarizada renal contrastada(AU)


Introduction: The renal linfangiectasia is an uncommon, in which dilatation of the lymphatic renal vessels exists. It is usually bilateral and of good presage. Objectives: To present a case of renal bilateral lymphangiectasia, their clinical characteristics and aspects of the imaginologic diagnosis. Clinical case: Patient feminine of 59 years of age, with controlled hypertension that attended the external consultation of Urology for lumbar bilateral pain, of moderate intensity, and reddish coloration of the urine, both in an intermittent way. The general and regional physical exam was negative. Microhematuria was confirmed in the general exam of the urine. The sanguine studies of the analytic one were all normal ones. The renal ultrasound reported bilateral moderate pielocaliectasia and parapielic cysts, with left prevalence. The renal contrasted computed tomography revealed the presence of bilateral renal hipodenses formations, of aspect cystic parapielic and in the renal sinus, and compatible with bilateral linfangiectasia. The patient has evolved favorably. Conclusions: The renal linfangiectasia it should be kept in mind in the deferential diagnosis of the cystic renal illness. For the imaginologic diagnostic of certainty it is essential the renal contrasted computed tomography(AU)


Asunto(s)
Humanos , Femenino , Persona de Mediana Edad , Anomalías Congénitas , Tomografía Computarizada por Rayos X/métodos , Riñón/anomalías , Linfangiectasia/diagnóstico por imagen , Linfangioma/diagnóstico
16.
An. bras. dermatol ; An. bras. dermatol;95(4): 477-479, July-Aug. 2020. graf
Artículo en Inglés | LILACS, Coleciona SUS | ID: biblio-1130906

RESUMEN

Abstract Lymphangioma is a rare and understudied pathology that is usually detected in the first decade of life, and its appearance in adults is rare. This report details a 51-year-old man with morbid obesity who presented, for the last eight months, multiple asymmetric tumor lesions with extension to the scrotal region. The diagnosis of circumscribed lymphangioma with associated infection was confirmed. This case report demonstrates an unusual presentation of the characteristics of the lymphangioma that are seldom described in the literature.


Asunto(s)
Humanos , Masculino , Adulto , Obesidad Mórbida , Hiperqueratosis Epidermolítica , Linfangioma , Escroto , Células Endoteliales , Persona de Mediana Edad
17.
An Bras Dermatol ; 95(4): 477-479, 2020.
Artículo en Inglés | MEDLINE | ID: mdl-32482545

RESUMEN

Lymphangioma is a rare and understudied pathology that is usually detected in the first decade of life, and its appearance in adults is rare. This report details a 51-year-old man with morbid obesity who presented, for the last eight months, multiple asymmetric tumor lesions with extension to the scrotal region. The diagnosis of circumscribed lymphangioma with associated infection was confirmed. This case report demonstrates an unusual presentation of the characteristics of the lymphangioma that are seldom described in the literature.


Asunto(s)
Hiperqueratosis Epidermolítica , Linfangioma , Obesidad Mórbida , Adulto , Células Endoteliales , Humanos , Masculino , Persona de Mediana Edad , Escroto
20.
Acta odontol. Colomb. (En linea) ; 10(2): 112-126, 2020. ilus, tab, ilus, tab
Artículo en Español | COLNAL, LILACS | ID: biblio-1123481

RESUMEN

Objetivo: identificar el manejo y respuesta a tratamientos que presentaron los pa-cientes pediátricos diagnosticados con hemangiomas y malformaciones vasculares en cabeza y cuello, que acudieron al servicio de Cirugía Oral y Maxilofacial de la Fundación Hospital de la Misericordia (HOMI) durante el período 2012­2019. Métodos: 11 p a c i e n-tes fueron atendidos en el servicio de cirugía oral y maxilofacial de la Fundación HOMI, entre enero de 2012 y noviembre de 2019, con diagnóstico de hemangioma y/o malfor-maciones vasculares. De estos, 9 fueron tratados con doxiciclina como agente esclero-sante. Resultados: se atendieron 5 pacientes masculinos y 6 femeninos con rango de edad entre los 8 meses y 13 años, con un promedio de 86,8 meses. La escleroterapia se realizó con doxiciclina en dosis de 100 mg disuelta en una ampolla de bicarbonato de sodio de 10 ml, inyectada en 5 pacientes con ecografía y en 4 por aspiración directa. De los 11 pacientes, 9 fueron tratados con escleroterapia; de estos 5 recibieron una sesión de escleroterapia, mientras que a los otros 4 se les realizó entre 2 y 3 sesiones. En 5 pacientes fue posible hacer seguimiento, ya que la atención depende de la autorización de la EPS. Conclusiones: la doxiciclina como agente esclerosante utilizado en lesiones vasculares ha demostrado ser un medicamento seguro, de bajo costo y efectivo como tratamiento inicial de hemangiomas y malformaciones vasculares en la cara. El número de aplicaciones depende del tipo de lesión a tratar.


Objective: Identify the treatment provided to patients with hemangioma and vascular deformities diagnosis .in head and neck who resorted to Oral and Maxillofacial service in Fundación Hospital de la Misericordia (HOMI) from 2012-2019 and the outcome of this treatments. Methods: Eleven (11) patients were admitted and treated by the surgical and maxillofacial team in "Fundación Hospital la Misericordia HOMI", between January 2012 and November 2019 with the hemangioma and vascular deformities diagnosis. Nine (9) of them were treated using doxycycline as a sclerosing agent. Results: Out of the eleven (11) patients, five (5) were males and the other six (6) were females, with an age range going from 8 months, up to 13 years, for an average of 86,8 months. A sclerotherapy was performed using doxycycline, with a dosage of 100 mg dissolved in a syringe with 10 mL of sodium bicarbonate on five (5) patients with ultrasound, and four (4) with direct aspiration. Out of the eleven (11) patients, five (5) received one sclerotherapy session the remaining four (4) received 2 or 3 sessions. In five (5) of the patients it was possible to complete follow-ups since the attention depends on their healthcare provider. Conclusions: Doxycycline as a sclerosing agent used in vascular lesions has demonstrated to be a safe treatment, with low cost, and effective as an initial treatment for hemangiomas and vascular deformities on the face. The number of sessions depend on the lesion that is going to be treated.


Asunto(s)
Humanos , Lactante , Niño , Doxiciclina , Linfangioma , Terapéutica , Escleroterapia , Malformaciones Vasculares
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