RESUMO
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.
Assuntos
Humanos , Feminino , Adulto , Neoplasias Hipofaríngeas/diagnóstico por imagem , Linfangioma/diagnóstico por imagem , Biópsia , Neoplasias Hipofaríngeas/patologia , Tomografia Computadorizada por Raios X/métodos , Linfangioma/patologiaRESUMO
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
Assuntos
Humanos , Feminino , Adulto Jovem , Neoplasias Peritoneais/diagnóstico , Hemangioma/diagnóstico , Linfangioma/diagnóstico , Mesentério/patologia , Neoplasias Peritoneais/cirurgia , Neoplasias Peritoneais/patologia , Células Endoteliais/patologia , Malformações Vasculares/diagnóstico , Malformações Vasculares/patologia , Hemangioma/cirurgia , Hemangioma/patologia , Linfangioma/cirurgia , Linfangioma/patologiaAssuntos
Linfangioma/etiologia , Neoplasias do Mediastino/etiologia , Osteólise Essencial/complicações , Adulto , Humanos , Linfangioma/diagnóstico por imagem , Linfangioma/patologia , Masculino , Neoplasias do Mediastino/diagnóstico por imagem , Neoplasias do Mediastino/patologia , Osteólise Essencial/diagnóstico por imagem , Tomografia Computadorizada por Raios XRESUMO
Background: Lymphatic vascular malformations (LVM) or formerly called lymphangiomas are congenital malformations present in about 1 out of 6000 to -16000 births. The most relevant classification system for lymphangioma management is based on the size of the cysts. Spontaneous resolution is uncommon; thus, expectant management is not recommended. The classic treatment is excisional surgery, but it can affect adjacent structures or have relapses, so, sclerosing substances like OK-432 are being studied. The majority of the studies are small in number of patients and are from Japan; the largest studies in Mexico are focused on specific lesions (macrocystic) or a determined anatomical region. To date, there are no studies of the population of the north of Mexico. Method: The experience with OK-432 was described through a retrospective, descriptive study in patients with LVM, from 2011 to 2016, in a reference hospital of northern Mexico. Results: A total of 26 patients with LVM were treated with OK-432. The majority of the lesions were macrocystic (69 %), microcystic (19 %) and mixed (12 %). From the total number of patients, 11 fully healed, and 72 % of the study population had >50 % reduction in lesion size, with only 2 applications. There were no recurrences. Complications were reported in 2 patients who had skin hyperpigmentation. Conclusions: OK-432 probed to be an effective treatment for LVM in a reference hospital in the north of Mexico.
Introducción: Las malformaciones vasculares linfáticas (MVL), anteriormente llamadas linfangiomas, son malformaciones congénitas que se presentan en uno de cada 6,000 a 16,000 nacimientos. El sistema de clasificación más útil para el manejo del linfangioma se basa en el tamaño de los quistes. La resolución espontánea es infrecuente, por lo que el tratamiento expectante no se recomienda. El tratamiento clásico es la cirugía de escisión, pero puede afectar a estructuras vecinas o haber recidivas, por lo que se empezaron a estudiar sustancias esclerosantes, como el OK-432. La mayoría de los estudios incluyen pocos pacientes; los más grandes realizados en México se enfocan a lesiones específicas (macroquísticas) o únicamente a una región anatómica. Hasta la fecha, no existen estudios del uso de este fármaco en la población del norte de México. Método: Se describe la experiencia con OK-432 mediante un estudio retrospectivo, descriptivo, en los pacientes con MVL, de 2011 a 2016, en un hospital de referencia del norte de México. Resultados: Veintiséis pacientes con MVL recibieron tratamiento con OK-432. La mayoría fueron macroquísticos (69%), microquísticos (19%) y mixtos (12%). Del total de pacientes, 11 presentaron curación total. El 72% de la población estudiada tuvo una reducción de > 50% del tamaño de las lesiones con solo dos aplicaciones de tratamiento; no se presentaron recidivas. Se reportaron complicaciones en dos pacientes (hiperpigmentación de la piel). Conclusiones: El manejo con OK-432 demostró ser efectivo para el tratamiento de las MVL en un hospital de referencia del norte de México.
Assuntos
Antineoplásicos/uso terapêutico , Linfangioma/tratamento farmacológico , Anormalidades Linfáticas/tratamento farmacológico , Picibanil/uso terapêutico , Antineoplásicos/efeitos adversos , Feminino , Humanos , Hiperpigmentação/induzido quimicamente , Linfangioma/patologia , Anormalidades Linfáticas/patologia , Masculino , México , Picibanil/efeitos adversos , Estudos Retrospectivos , Resultado do TratamentoRESUMO
Resumen Introducción: Las malformaciones vasculares linfáticas (MVL), anteriormente llamadas linfangiomas, son malformaciones congénitas que se presentan en uno de cada 6,000 a 16,000 nacimientos. El sistema de clasificación más útil para el manejo del linfangioma se basa en el tamaño de los quistes. La resolución espontánea es infrecuente, por lo que el tratamiento expectante no se recomienda. El tratamiento clásico es la cirugía de escisión, pero puede afectar a estructuras vecinas o haber recidivas, por lo que se empezaron a estudiar sustancias esclerosantes, como el OK-432. La mayoría de los estudios incluyen pocos pacientes; los más grandes realizados en México se enfocan a lesiones específicas (macroquísticas) o únicamente a una región anatómica. Hasta la fecha, no existen estudios del uso de este fármaco en la población del norte de México. Método: Se describe la experiencia con OK-432 mediante un estudio retrospectivo, descriptivo, en los pacientes con MVL, de 2011 a 2016, en un hospital de referencia del norte de México. Resultados: Veintiséis pacientes con MVL recibieron tratamiento con OK-432. La mayoría fueron macroquísticos (69%), microquísticos (19%) y mixtos (12%). Del total de pacientes, 11 presentaron curación total. El 72% de la población estudiada tuvo una reducción de > 50% del tamaño de las lesiones con solo dos aplicaciones de tratamiento; no se presentaron recidivas. Se reportaron complicaciones en dos pacientes (hiperpigmentación de la piel). Conclusiones: El manejo con OK-432 demostró ser efectivo para el tratamiento de las MVL en un hospital de referencia del norte de México.
Abstract Background: Lymphatic vascular malformations (LVM) or formerly called lymphangiomas are congenital malformations present in about 1 out of 6000 to -16000 births. The most relevant classification system for lymphangioma management is based on the size of the cysts. Spontaneous resolution is uncommon; thus, expectant management is not recommended. The classic treatment is excisional surgery, but it can affect adjacent structures or have relapses, so, sclerosing substances like OK-432 are being studied. The majority of the studies are small in number of patients and are from Japan; the largest studies in Mexico are focused on specific lesions (macrocystic) or a determined anatomical region. To date, there are no studies of the population of the north of Mexico. Methods: The experience with OK-432 was described through a retrospective, descriptive study in patients with LVM, from 2011 to 2016, in a reference hospital of northern Mexico. Results: A total of 26 patients with LVM were treated with OK-432. The majority of the lesions were macrocystic (69 %), microcystic (19 %) and mixed (12 %). From the total number of patients, 11 fully healed, and 72 % of the study population had >50 % reduction in lesion size, with only 2 applications. There were no recurrences. Complications were reported in 2 patients who had skin hyperpigmentation. Conclusions: OK-432 probed to be an effective treatment for LVM in a reference hospital in the north of Mexico.
Assuntos
Feminino , Humanos , Masculino , Picibanil/uso terapêutico , Anormalidades Linfáticas/tratamento farmacológico , Linfangioma/tratamento farmacológico , Antineoplásicos/uso terapêutico , Picibanil/efeitos adversos , Estudos Retrospectivos , Resultado do Tratamento , Hiperpigmentação/induzido quimicamente , Anormalidades Linfáticas/patologia , Linfangioma/patologia , México , Antineoplásicos/efeitos adversosRESUMO
Lymphangioma is an uncommon benign vascular tumour that involves lymphatic vessels. It can be acquired or, most frequently, congenital. The acquired form presents with dilated lymphatic channels due to an obstruction. These lesions have no risk of malignant transformation, but they have a high rate of recurrence whether removed. We present a case of a 52-year-old woman with acquired lymphangiomas mimicking warts. She came to our observation for some keratotic lesions on her feet. Clinically, we found three warts on the sole of her left foot, but we also noticed the presence of swelling and papillomatous wart-like papules on both halluces. The hallux papules were studied by performing an excisional biopsy and were found to be lymphangiomas.
Assuntos
Hallux/patologia , Linfangioma/patologia , Neoplasias Cutâneas/patologia , Verrugas/patologia , Biópsia , Diagnóstico Diferencial , Feminino , Humanos , Vasos Linfáticos/patologia , Pessoa de Meia-IdadeRESUMO
Lymphangioma-like Kaposi's sarcoma (LLKS) is a rare histologic variant of KS. Kaposi's sarcoma is also known as human herpesvirus type 8. The clinical presentation of the LLKS lesion is highly unusual and similar to that of classic KS but with multinodular vascular tumors and lymphedema. We present a 63-year-old native Haitian man with multiple slowly progressive exophytic ulcerated lesions covering more than 60% of his left lower extremity with no systemic involvement. Much confusion surrounded the clinical presentation of these wounds, and we postulated several differential diagnoses. Herein we describe the evaluation, clinical appearance, and progression of LLKS. Due to the rarity of LLKS, treating physicians need to be aware of the clinical presentation and diagnostic criteria of this variant. Despite being incurable, early diagnosis of LLKS can lead to long-term treatment options and a major reduction in symptoms.
Assuntos
Pé Diabético/patologia , Linfangioma/patologia , Sarcoma de Kaposi/patologia , Neoplasias Cutâneas/patologia , Biópsia por Agulha , Pé Diabético/diagnóstico , Pé Diabético/cirurgia , Diagnóstico Diferencial , Progressão da Doença , Eletrocoagulação/métodos , HIV , Haiti , Humanos , Imuno-Histoquímica , Linfangioma/diagnóstico , Pessoa de Meia-Idade , Monitorização Fisiológica , Invasividade Neoplásica/patologia , Estadiamento de Neoplasias , Prognóstico , Doenças Raras , Medição de Risco , Sarcoma de Kaposi/cirurgia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/cirurgia , Falha de TratamentoRESUMO
Introduction Lymphangioma is a rare congenital vascular malformation of the head and neck region isolated from the systemic circulation. It has a benign etiology, and represents 13% of all orbital tumors. These hamartomas often present in the pediatric population with a slightly female predilection. They have a lymphocytic composition, and may increase in size with episodes of viral infection, causing proptosis. Discussion The management of this lesion is controversial, hardly curative, and depends on the clinical presentation. The treatment options include partial surgical resection of the major cyst, needle aspiration, surgical debulking, systemic steroids, sildenafil, intralesional injection of the sclerosing agents, and local radiotherapy. Case Report In the present report, we describe an uncommon case of lymphangioma in a 6-year-old female who was first submitted to neurosurgery for tumor resection and received sildenafil therapy later, with promising results. Conclusion The treatment of orbital lymphangiomas remains a controversial topic, and the use of sildenafil along with needle aspiration and microsurgical removal is a viable option of treatment. However, many issues, such as the ideal duration of the therapy, the dosage regimen and the recurrence rate, still remain unclear. Our case report adds promising data on this pathology, even though larger trials are needed to properly elucidate the remaining questions.
Introdução Linfangioma é uma malformação vascular rara congênita da cabeça e da região cervical isolada da circulação sistêmica. Apresenta uma etiologia benigna e representa 13% de todos tumores orbitais. Estes hematomas geralmente se apresentam na população pediátrica com uma pequena predileção pela população feminina. Têm uma composição linfocítica e podem aumentar em tamanho com episódios de infecções virai causando proptose. Discussão O manejo destas lesões é controverso, dificilmente curativo e depende na apresentação clínica. Opções de tratamento compreende ressecção cirúrgica parcial do cisto de maior volume, aspiração por agulha de punção, redução cirúrgica, esteroides sistêmicos, Sildenafil, administração intralesional de agentes esclerosantes, e radioterapia local. Relato de Caso No presente relato, descrevemos um caso incomum de linfangioma em uma paciente feminina de seis anos de idade, inicialmente submetida a procedimento neurocirúrgica para ressecção tumoral, com posterior terapia com Sildenafil, apresentando resultados promissores. Conclusão O tratamento de linfangiomas de órbita permanece um tópico controverso, e o uso de Sildenafil em conjunto com aspiração por agulha de punção e ressecção microcirúrgica é uma opção viável de tratamento. Entretanto, muitas quesitos tais como tempo de terapia, regime de dose e taxa de recorrência permanecem incertos. Nosso relato de caso contribui com dados promissores referente a esta patologia, ainda que ensaios maiores são necessários para elucidação apropriada acerca das questões pendentes.
Assuntos
Humanos , Feminino , Criança , Órbita , Linfangioma , Linfangioma/patologiaRESUMO
Abstract: Lymphangioma is an uncommon benign vascular tumour that involves lymphatic vessels. It can be acquired or, most frequently, congenital. The acquired form presents with dilated lymphatic channels due to an obstruction. These lesions have no risk of malignant transformation, but they have a high rate of recurrence whether removed. We present a case of a 52-year-old woman with acquired lymphangiomas mimicking warts. She came to our observation for some keratotic lesions on her feet. Clinically, we found three warts on the sole of her left foot, but we also noticed the presence of swelling and papillomatous wart-like papules on both halluces. The hallux papules were studied by performing an excisional biopsy and were found to be lymphangiomas.