Linfangioma hipofaríngeo en adulto: presentación de un caso clínico / Hypopharyngeal lymphangioma in adults: Presentation of a case report

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.

Hemangiolinfangioma mesentérico: reporte de un caso / Mesenteric hemangiolymphangioma: case report

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

Experiencia con OK-432 en malformaciones vasculares linfáticas en un hospital del norte de México / Experience with OK-432 in lymphatic vascular malformations in a hospital from northern Mexico

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.

Orbital Lymphangioma: Case Report and Management Paradigms

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.

Acquired lymphangiomas mimicking multiple hallux warts

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.

Cutaneous Lymphangioma circumscriptum - dermoscopic features*

Lymphangiomas are congenital lymphatic malformations and cutaneous lymphangioma circumscriptum is the most common type. It is clinically characterized by clusters of translucent vesicles and the presence of dermoscopically yellow lacunae surrounded by pale septa, as well as reddish to bluish lacunae. In our case, the recently described hypopyon-like feature manifested, aiding in the sometimes difficult differential diagnosis of cutaneous lymphangioma circumscriptum with vascular lesions, further highlighting the importance of dermoscopy in what can be a diagnostic challenge.

Malformación linfática facial mixta: Caso clínico / Mixed facial lymphatic malformation: Case report

Lymphangiomas are a common form of vascular malformation of the lymphatic vessels, mainly in the head and neck region. Most cases are progressive evolution and require a multidisciplinary approach. Currently, the first therapeutic option is sclerotherapy, leaving surgery for the treatment of remaining lesions. Objective: To present a case of facial lymphatic malformation (LM) treated with sclerotherapy, surgery and orthodontics in a 15-year follow up. Case report: A one-year-old female patient who consulted health professionals due to a progressive volume increase of the soft parts of her right cheek. The imaging study confirmed the diagnosis of microcystic lymphatic malformation. It was managed with OK-432 sclerotherapy and Bleomycin. At 2 years of age, the patient response was considered adequate; an intralesional submandibular surgical excision was then performed, with partial resection of the lesion. The biopsy confirmed the diagnosis of microcystic LM. Six months after, a re-resection was planned using the same approach and removing the remaining lesion, with favorable development until the age of 9 years when the patient required surgery and orthodontic management due to intraoral recurrence. No major developments until the age of 13 when a new orthodontic surgery and handling are planned to perform right oral commissure suspension. Conclusion: LM management by sclerotherapy, surgery, and orthodontics has shown the advantages of a multidisciplinary long-term treatment in this case.

El linfangioma corresponde a una malformación vascular de los vasos linfáticos, preferentemente de la región de cabeza y cuello. La mayoría de los casos son de evolución progresiva y requieren un manejo multidisciplinario. Actualmente la primera opción terapéutica es la esclerosis, reservando la cirugía para el tratamiento de las lesiones remanentes. Objetivo: Presentar un caso de malformación linfática (ML) facial, tratado con escleroterapia, cirugía y ortodoncia en un seguimiento a 15 años. Caso clínico: Paciente de sexo femenino que consulta al año de edad por aumento de volumen progresivo de partes blandas en su mejilla derecha. El estudio de imágenes confirmó el diagnóstico de Malformación Linfática microquística. Se manejó con esclerosis seriada con OK-432 y Bleomicina. A los 2 años de edad se consideró que la respuesta era adecuada, y se procedió a realizar extirpación quirúrgica intralesional submandibular, con resección parcial de la lesión. La biopsia confirmó el diagnóstico de ML microquística. Seis meses después se planificó nueva resección utilizando el mismo abordaje y extirpando lesión remanente, con evolución favorable hasta la edad de 9 años en que requiere cirugía y manejo por ortodoncia, por recidiva de lesión a nivel intraoral. Evolución favorable hasta que a la edad de 13 años se planifica nueva cirugía y manejo por ortodoncia para suspender la comisura bucal derecha. Conclusión: El manejo de la ML mediante escleroterapia, cirugía, y ortodoncia muestra en este caso las ventajas de un tratamiento multidisciplinarion a largo plazo.

A Rare Case of Diffuse Pulmonary Lymphangiomatosis in a Middle-Aged Woman

Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder characterized by lymphatic channel proliferation. It is mostly reported in children and young adults. Here, we report a case involving a 52-year-old asymptomatic woman who presented with increased interstitial markings, as seen on a chest radiograph. Diffuse interstitial septal thickening was found on a serial follow-up chest computed tomography scan, and lymphangitic metastasis was the primary radiologic differential diagnosis. However, histologic sections of wedge resected lung revealed diffuse pleural and interlobular septal lymphatic proliferation characteristic of DPL.

Vulvar Lymphangioma Circumscriptum.

Lymphangioma circumscriptum (LC) is a rare benign disorder of lymphatic channels in deep dermal and subcutaneous layers. Typically fluid filled vesicles that contain lymphatic fluid with frogspawn-like appearance are seen over any part of body. Vulvar LC is seen as congenital condition or might develop secondary to radiotherapy or surgery. LC involving the vulva is very rare; only 33 cases of vulvar LC have been reported. Surgical treatment is the most commonly used method to treat vulvar LC. In this report we present a case with LC secondary to radiotherapy and lesions regressed with cryotherapy.

Les lymphangiomes : etude retrospective de 12 cas a Yaounde (Cameroun)

Objectif : Rechercher les donnees preliminaires sur les lymphangiomes au Cameroun; leurs topographies ainsi que leurs formes histologiques dans le but d'ameliorer le diagnostic et les modalitestherapeutiques de ces lesions. Materiel et methode : Il s'agit d'une etude retrospective; observationnelle et descriptive.Les patients ayant presente des lymphangiomes documentes par un rapport anatomopathologique ont ete recrutes consecutivement dans tous les laboratoires d'anatomie pathologique du Cameroun sur une periode de 06 ans; de 2004 a 2009. Les coupes histologiques colorees a l' hematoxyline-eosine ont ete revues pour confirmation diagnostique de differents types. Resultats : Douze cas ont ete recrutes. La moitie de ces cas provenait des malades originaires de la region de l'ouest du Cameroun. Le lymphangiome touchait le plus souvent l'enfant age de 1 a 5 ans avec predominance du type hygrome kystique. Les localisations les plus courantes etaient axillaires; cervicales et viscerales. Conclusion : Le lymphangiome constitueun desdiagnostics differentiels important de toute masse survenant chez les patients; en particulier chez les enfants ages de 1 a 5 ans.L'anatomie pathologique est d'une aide dans le diagnostic et la typisation

Tonsilla Lymphangioma.

Lymphagiomas are uncommon benign tumours of tonsils. Lymphangiomas are benign lymphatic tumours typically composed of dialated lymphatic channels which often occur subcutaneously in the head and neck region. Tonsillar lymphangiomas have been reported rarely in the literature which presents as a mass lesion. Histological confirmation is necessary for diagnosis. We report a case of lymphangioma of the tonsil in an otherwise well 17 year old female patient and review the clinical and histological features of this tumour. These lesions are usually cured by simple surgical excision.

Linfangioma: reporte de un caso y revisión de la literatura / Lymphangioma: a case report and literature review

Los Linfangiomas son considerados patologías benignas de vasos linfáticos que en la cavidad bucal se manifiestan como lesiones focales superficiales. Generalmente se presentan desde el nacimiento o antes de las dos primeras décadas de vida, aparentemente sin predilección por el sexo. Dicha lesión se manifiesta como formas vesiculares cuando son superficiales y nodulares cuando son profundas. La coloración puede variar desde un color similar al tejido circundante hasta rojo azulado cuando los capilares forman parte de la malformación. Su localización intraoral más común es la cara dorsal de la lengua y bordes laterales, pudiendo provocar macroglosia. Su presentación extraoral se denomina higroma quístico y afecta el cuello. Se reporta un caso de un paciente de sexo femenino de 24 años de edad presentando lesión tumoral elevada, multilobulada, sintomática al momento de la consulta, manifestando dolor y sangramiento espontáneo. La lesión es del mismo color de la mucosa circundante, localizada en tercio medio de la cara dorsal de la lengua, dificultando la fonación y deglución. Se realizó biopsia incisional de la lesión con un diagnóstico provisional de linfangioma, obteniendo como resultado histopatológico Linfangioma.

Linphangioma are considered as benign pathologies of the lymphatic vessels, when they are evident in the oral cavity, they become superficial focal lesions. Generally, they become present at birth or before the first two decades of human life. This injury becomes apparent in different vessels forms when is superficial and nodes when is deep. Its color can vary from a similar shade of the surrounding tissue to a bluish red when capillary vessels form part, of the malformation. Its most common intra-oral location is in the dorsal face of the tongue and side edges, where it could produce macroglossia. Its extra-oral presence is designated as cystic hygroma and it affects the neck area. This is the reported case of a 24 years old female patient, who presents an elevated tumor lesion, multilobulated, and symptomatic at the time of her medical visit, with evident pain and spontaneous bleeding. The injury shows the same color of the surrounding mucous, localized in the central zone of the dorsal face of the tongue, making it difficult for swallowing and phonation. An incision biopsy of the injury was performed, with a temporary diagnosis of linphangioma, and as a histopathologic result linphangioma.

MR imaging of hepatic lymphangioma / 대한간학회지

No abstract available.

Wilms tumor 1 protein is not expressed in oral lymphangiomas

Lymphangiomas are benign hamartomatous lesions of lymphatic vessels. Wilms Tumor 1 (WT1) is a transcription factor that is activated in some human neoplasias. WT1 protein expression is observed in endothelial cells during angiogenesis and is a useful marker to distinguish between vascular proliferations and vascular malformations. The purpose of the present study is to report a case series of oral lymphangiomas together with an immunohistochemical investigation of WT1. Seventeen cases of oral lymphangioma were retrieved and reviewed. Immunohistochemical analysis of WT1 protein was performed and pyogenic granuloma samples were used as positive controls. The male/female ratio was 1.125 and most of the lesions occurred in young subjects. While pyogenic granuloma showed positive staining for WT1, the endothelial cells lining the thin-walled dilated lymphatic vessels of lymphangiomas were negative for this protein. The findings strengthen the idea that oral lymphangioma is a vascular malformation characterized by lymphatic dilatation without significant endothelial proliferation.

Os linfangiomas são tumores hamartomatosos benignos dos vasos linfáticos. O Wilms Tumor 1 (WT1) é um fator de transcrição que se encontra ativo em algumas neoplasias humanas. A expressão da proteína WT1 é observada em células endoteliais durante a angiogênese e pode ser um marcador útil para distinguir as proliferações vasculares das malformações vasculares. O objetivo deste estudo foi relatar uma série de casos de linfangiomas orais e avaliar a expressão imunoistoquímica da proteína WT1. Dezessete casos de linfangiomas orais foram recuperados e revisados. A análise imunoistoquímica foi realizada e amostras de granuloma piogênico foram utilizadas como controle positivo. A relação homem/mulher foi de 1,125 e a maioria das lesões acometeram pacientes jovens. Enquanto o granuloma piogênico mostrou uma imunopositividade para WT1, as células endoteliais da fina parede dos vasos linfáticos dilatados apresentaram-se negativas para esta proteína. Tais achados reforçam a idéia de que o linfangioma oral é uma malformação vascular caracterizada por dilatação linfática sem uma proliferação endotelial significativa.

Cystic lymphangiomatous hamartoma masquerading as massive ascites.

We report a 4-year-old boy presenting with a tense massive ascites and large hydrocele. History and physical examination were unremarkable. Routine laboratory studies were normal. Abdominal ultrasonography revealed massive ascites. Contrast CT was suggestive of a large cyst covering the entire peritoneal cavity. At laparotomy, a large cystic tumor was found extending into the scrotum through the left inguinal ring. Histopathologic examination diagnosed the tumor as a cystic lymphangiomatous hemartoma. Although abdominal lymphangiomas are seen in children, but presenting as massive ascites with hydrocele is very rare.