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

Racemose hemangioma presenting as a vitreous hemorrhage: case report / Hemangioma racemoso apresentando-se como hemorragia vítrea: relato de caso

ABSTRACT This report describes a case of retinal racemose hemangioma that first presented as a vitreous hemorrhage. The authors present the case of a 47-year-old woman with a sudden 5-day painless visual loss in her left eye. At the first visit, the best-correct visual acuities were 20/20 in the right eye and hand motions in the left eyes. Ultrasonography showed an attached retina and a massive vitreous hemorrhage. Pars plana vitrectomy was performed and a dilatation of large vessels was detected bulging from the optic disc. The best-correct visual acuities on day 30 postoperatively was 20/25 in the left eye. Fundus angiography and spectral-domain optical coherence tomography angiography showed anomalous arteriovenous communications with no intervening capillaries. The diagnosis was racemose hemangioma, an arteriovenous malformation of group 2 retina based on the Archer classification.

RESUMO Este relato descreve um caso de hemangioma racemoso da retina que se apresentou inicialmente como hemorragia vítrea. Os autores apresentam o caso de uma mulher de 47 anos com perda visual súbita e indolor 5 dias antes no olho esquerdo. Na primeira visita, a melhor acuidade visual corrigida foi de 20/20 no olho direito e movimentos das mãos no olho esquerdo. A ultrassonografia mostrou uma retina aderida e uma hemorragia vítrea maciça. Foi realizada vitrectomia pars plana, sendo detectada proliferação de grandes vasos salientes do disco óptico. A acuidade visual no dia 30 de pós-operatório foi de 20/25 no olho esquerdo. A angiografia de retina e a angiotomografia de coerência óptica de domínio espectral mostraram comunicações arteriovenosas anômalas sem capilares intermediários. O diagnóstico foi hemangioma racemoso, uma malformação arteriovenosa da retina do grupo 2 com base na classificação de Archer.

Hemangioma hepático gigante único: serie de casos con seguimiento de pacientes tratados quirúrgicamente / Single giant liver hemangioma: case series with follow-up of patients treated surgically

El tratamiento del hemangioma hepático gigante (HHG), sigue siendo motivo de controversia. El objetivo de este estudio fue reportar los resultados de pacientes con HHG resecados quirúrgicamente en términos de morbilidad postoperatoria (MPO). Serie de casos con seguimiento. Se incluyeron pacientes con HHG, sometidos a cirugía de forma consecutiva, en Clínica RedSalud Mayor, entre 2011 y 2020. La variable resultado fue MPO. Otras variables de interés fueron: tiempo quirúrgico, estancia hospitalaria y mortalidad. Las pacientes fueron seguidas de forma clínica. Se utilizó estadística descriptiva, con medidas de tendencia central y dispersión. Se intervinieron 5 pacientes, con una mediana de edad de 38 años. La medianas del tiempo quirúrgico y estancia hospitalaria; fueron 75 min y 4 días respectivamente. La MPO fue 20 % (1 caso de seroma). Con una mediana de seguimiento de 41 meses, los pacientes se encuentran asintomáticos y no se ha verificado morbilidad alejada. La resección quirúrgica de un HHG se puede realizar con escasa morbilidad, tanto en términos numéricos como de gravedad de la complicación observada.

SUMMARY: Treatment of giant hepatic hemangioma (GHH) remains controversial. The aim of this study was to report the outcomes of surgically resected GHH patients in terms of postoperative morbidity (POM). Case series with follow-up. Patients with GHH who underwent surgery consecutively at the RedSalud Mayor Clinic between 2011 and 2020 were included. The outcome variable was POM. Other variables of interest were surgical time, hospital stay and mortality. The patients were followed up clinically. Descriptive statistics were used, with measures of central tendency and dispersion. Five patients underwent surgery, with a median age of 38 years. The median surgical time and hospital stay; were 75 min and 4 days respectively. The MPO was 20 % (1 case of seroma). With a median follow-up of 41 months, the patients are asymptomatic, and no distant morbidity has been verified. Surgical resection of GHH can be performed with low morbidity, both in terms of numbers and the severity of the complication observed.

Angioma de células litorales del bazo: un diagnóstico de lesión focal esplénica: caso clínico / Littoral cell angioma of the spleen: report of one case

Splenic vascular neoplasms are the most common form of spleen tumors. Among them, littoral cell angioma is rare and it is frequently an incidental finding in imaging studies. It has no specific clinical, laboratory or imaging findings. Splenectomy allows definitive diagnosis throughout a histopathological examination. We report a 52-year-old man presenting with asthenia and abdominal distension. Computed tomography with intravenous contrast showed multiple splenic hypodense masses and a prostatic enlargement. Presuming a lymphoma, a laparoscopic splenectomy was performed. Histopathologic examination diagnosed littoral cell angioma. During urological follow-up, a prostate adenocarcinoma was diagnosed.

Tratamento cirúrgico de hemangioma infantil nasal e labial na fase involuída: relato de caso / Surgical treatment of infantile nasal and labial hemangioma in the involuted phase: a case report

Hemangioma infantil (HI) é o tumor vascular mais comum e a neoplasia benigna mais frequente da infância, com maior incidência no sexo feminino e na população branca. Quase 60% dos casos ocorrem em cabeça e pescoço, sendo o tratamento ativo durante a fase proliferativa mais frequentemente indicado, em decorrência dos possíveis problemas funcionais e do potencial desfigurante. Relatamos um caso de paciente com hemangioma infantil involuído de ponta nasal e lábio superior, tratado de forma expectante durante a infância, submetida à correção da deformidade residual com técnicas de rinoplastia, associado à zetaplastia e lipoenxertia do lábio superior com bom resultado e satisfação do paciente.

Infantile hemangioma (IH) is the most common vascular tumor and the most frequent benign neoplasm in childhood, with the highest incidence in females and the white population. Almost 60% of cases occur in the head and neck, and active treatment during the proliferative phase is the most frequently indicated, due to possible functional problems and disfiguring potential. We report a case of a patient with involute infantile hemangioma of the nasal tip and upper lip, treated expectantly during childhood, submitted to residual deformity correction with rhinoplasty techniques, associated with zetaplasty and upper lip grafting with good results and patient satisfaction.

Síndrome de Klipper-Trenaunay-Weber con compromiso vesical y uterino tratado por vía endoscópica y endovascular / Klippel-Trenaunay-Weber syndrome with vesical and uterine involvement treated by endoscopic and endovascular routes

El síndrome de Klippel-Trenaunay-Weber (SKTW) es una rara malformación venosa que, en general afecta a los miembros inferiores y, más raramente, a los superiores. Se caracteriza por formaciones angiomatosas cutáneas, várices e hipertrofia del miembro afectado. El compromiso genitourinario es sumamente infrecuente. Se presenta el caso de una paciente de 14 años. Ingresó por hematuria macroscópica de 48 h de evolución y metrorragia con grave compromiso hemodinámico. Se encontraba en estudio por presentar un hemangioma en el miembro inferior izquierdo que se extendía hasta la región pelviana. La uretrocistofibroscopía demostró la presencia de múltiples lesiones angiomatosas diseminadas en forma amplia en la vejiga, algunas de ellas con sangrado activo. La angioresonancia mostró una voluminosa formación hipervascularizada en contacto con la pared vesical a la cual desplazaba y fístulas arteriovenosas a nivel pelviano y en el miembro inferior izquierdo confirmando el diagnóstico etiológico. Se realizó una embolización arterial selectiva de los territorios ilíacos interno y externo e inmediatamente después una endocoagulación láser de los focos angiomatosos sangrantes. La hematuria remitió completamente en las 24 h posteriores al procedimiento. La metrorragia asociada al SKTW fue controlada mediante la utilización de análogos LHRH y progestágenos.

Klippel-Trenaunay-Weber syndrome (KTWS) is a rare venous malformation that generally affects the lower limbs and, more infrequently, the upper limbs. It is characterized by cutaneous angiomatous formations, varicose veins and hypertrophy of the affected limb. The involvement of the genitourinary tract is extremely infrequent. We expose the case of a 14 years old female patient who was admitted for macroscopic hematuria of 48 hours of evolution and metrorrhagia with severe hemodynamic decompensation. The patient was under study for presenting a hemangioma in the lower left limb that extended to the pelvic region. Urethrocystofibroscopy showed the presence of multiple wide-spread angiomatous lesions in the bladder, some of them with active bleeding. The angio-resonance showed a voluminous hypervascular formation in contact with the bladder wall showing several arteriovenous fistulas at the pelvic level and in the left lower limb confirming the etiological diagnosis. A selective arterial embolization of the internal and external iliac territories was performed and then, a laser endocoagulation of the bleeding angiomatous foci was carried out. The hematuria completely stopped within 24 hours later of the procedure. The metrorrhagia associated with KTWS was controlled by the use of LHRH analogs and progestogens.

Cirugía transoral en hemangiomas de laringe e hipofaringe en adultos / Transoral surgery in laryngeal hemangiomas e hypopharynx in adults

Los hemangiomas son tumores benignos infrecuentes en la laringe. El tratamiento está indicado cuando el tumor produce síntomas (disnea, hemoptisis, odinofagia) y también cuando el riesgo de hemorragia severa por traumatismo es alto. Tres hemangiomas de laringe e hipofaringe fueron tratados con éxito por vía transoral con láser de C02 y radiofrecuencia. Todos los pacientes se alimentaron por vía oral a las 24/48 horas y ninguno tuvo traqueostomía. La tasa de resección completa de los hemangiomas tratados por vía transoral fue 100%. (AU)

Hemangiomas are a rare type of benign tumors of the larynx. The treatment is indicated when the tumor produces symptoms (dyspnea, hemoptysis, odynophagia) and also when the risk of severe bleeding from trauma is high. Three hemangiomas of the larynx and hypopharynx were successfully treated transorally with C02 laser and radiofrequency. All were orally fed at 24/48 hours, and none had a tracheostomy The complete resection rate of hemangiomas treated transorally was 100%. (AU)

Relato de caso: síndrome de Klippel-Trénaunay- Weber / Case Report: Klippel-Trénaunay-Weber syndrome

Introdução: A síndrome de Klippel-Trénaunay-Weber (SKTW) é caracterizada pelo conjunto de sinais que consiste em malformações capilares, malformações venosas com ou sem malformações linfáticas associado ao supercrescimento de membros. Na maioria das vezes, envolve apenas uma extremidade com malformação arteriovenosa e cerca de 75% dos pacientes manifestam antes dos 10 anos de idade. Relato de Caso: Relatamos um caso de Klippel-Trénaunay- Weber em um paciente de 7 meses em acompanhamento na enfermaria da Cirurgia Plástica do Hospital de Clínicas da Universidade Federal de Uberlândia para o qual foi proposto tratamento cirúrgico da lesão. Conclusão: Como a SKTW é uma doença com morbidade progressiva e grave, o paciente deve ser acompanhado em um centro de referência com experiência e arsenal terapêutico diversificado para atuar da melhor forma possível no tratamento.

Introduction: The Klippel-Trénaunay-Weber syndrome (KTWS) is characterized by several signs, including capillary malformations and venous malformations with or without lymphatic malformations associated with limb overgrowth. In most cases, only one extremity is involved with arteriovenous malformation, and approximately 75% of the patients manifest symptoms before 10 years of age. Case Report: We report a case of a 7-month-old patient with KTWS followed-up at the Plastic Surgery Service of the Hospital de Clínicas, Federal University of Uberlândia; surgical treatment of the lesion was proposed for the patient. Conclusion: Since KTWS is a progressive disease with severe morbidity, the patient must be followed-up at a reference center by experienced staff with diverse therapeutic arsenal.

Vulvar hemangioma: case report / Hemangioma vulvar: relato de caso

Abstract Hemangioma is a benign neoplasm that may affect the vulva, and it can cause functional or emotional disability. This article reports the case of a 52-year-old female patient with a history of a genital ulcer for the past 3 years and who had undergone various treatments with creams and ointments. The patient was biopsied and diagnosed with vulvar hemangioma and was subsequently submitted to surgical excision of the lesion. We emphasize the importance of following the steps of the differential diagnosis and proceeding with a surgical approach only if necessary.

Resumo O hemangioma é uma neoplasia benigna que pode afetar a vulva e pode causar incapacidade funcional ou emocional. Este artigo relata o caso de uma paciente de 52 anos com história de úlcera genital nos últimos 3 anos, submetida a diversos tratamentos com cremes e pomadas. A paciente foi biopsiada e diagnosticada com hemangioma vulvar e subsequentemente submetida a excisão cirúrgica da lesão. Ressaltamos a importância de seguir as etapas do diagnóstico diferencial e proceder a uma abordagem cirúrgica somente se necessário.

Manejo quirúrgico de pacientes adultos con anomalías vasculares: serie de casos / Surgical management of adult patients with vascular anomalies: series of cases

Introducción: Las anomalías vasculares son un grupo heterogéneo de patologías, que considera tanto tumores como malformaciones vasculares. Son, habitualmente, de diagnostico y manejo durante la infancia, algunas lesiones persisten hasta la edad adulta. El tratamiento habitual considera terapias poco invasivas, siendo la alternativa resecctiva un opcion infrecuente. Objetivos: El objetivo de este estudio es presentar el manejo quirúrgico de pacientes portadores de anomalías vasculares realizado por el equipo de Cirugía Plástica y Reconstructiva del Hospital Clínico de la Universidad de Chile. Material y Métodos: Serie de casos retrospectiva de pacientes con diagnóstico de anomalias vasculares, sometidos a manejo quirúrgico por el equipo de Cirugía Plástica y Reconstructiva del Hospital Clínico de la Universidad de Chile. Se incluyó todos los pacientes con diagnóstico de anomalías vasculares intervenidos entre los años 2010 ­ 2016. Se realizó una revisión del historial clínico de cada paciente elaborando un análisis descriptivo. Resultados: Serie de 9 pacientes, 6 (66,6%) de sexo femenino, con edad promedio de 34,88 ±15,70 años. De los cuales 6 (66,67%) presentaron malformaciones vasculares y 2 (22,22%) tumores vasculares. Se observo compromiso de cabeza en 6 (66,67%) casos y 2 (22,22,%) genital. Se utilizo colgajos locales de avance en 6 (66,67%) y colgajos de transposición en 2 (22,22%) casos. Conclusiones: El manejo quirúrgico es una alternativa terapéutica segura que ofrece resultados buenos a las lesiones complejas. Y por tanto, creemos que es necesario un trabajo multidisciplinario entre pediatras, dermatólogos, radiólogos fisiatras, psicólogos y cirujanos plásticos para poder ofrecer mejores resultados a nuestros pacientes.

Introduction: Vascular anomalies are a heterogeneous group of pathologies, which considers tumors and vascular malformations. Usually diagnosed and treated during childhood, some lesions persist into adulthood. The usual treatment considers non-invasive therapies, being the respective alternative an uncommon option. Objectives: Present the surgical management of adults patients with vascular anomalies performed by the Plastic and Reconstructive Surgery team of the Clinical Hospital of the University of Chile. Material and methods: Retrospective case series of patients with diagnosis of vascular anomalies submitted to surgical management by the Plastic and Reconstructive Surgery team of the Clinical Hospital of the University of Chile. All patients with diagnosis of vascular anomalies operated between the years 2010 - 2016 were included. A review of the clinical history of each patient was carried out and a descriptive analysis was done. Results: Series of 9 patients, 6 (66.6%) female, with a mean age of 34.88 ± 15.70 years. Of these, 6 (66.67%) had vascular malformations and 2 (22.22%) had vascular tumors. Head involvement was observed in 6 (66.67%) cases and 2 (22.22%) genital. Local flaps were used in 6 (66.67%) and transposition flaps in 2 (22.22%) cases. Conclusions: Surgical management is a safe therapeutic alternative that offers beneficial results to complex lesions. Therefore, we believe a multidisciplinary work between pediatricians, dermatologists, radiologists.

Hemangioma hepático gigante / Giant hepatic hemangioma

Los hemangiomas hepáticos son los tumores benignos más frecuentes, comúnmente se presentan en mujeres y son considerados gigantes cuando su diámetro supera 4 cm1.Se manifiestan con dolor abdominal y efecto de masa. Estos tumores pueden ser manejados por observación, enucleación, resección, y embolización.

Liver haemangiomas are the most common benign tumours, commonly presented in women and considered giant when their diameter surpasses 4cm1 . They manifest with abdominal pain and mass effect. These tumours can be managed by observation, enucleation, resection, and embolisation.

Hemangioma intraóseo de la mandíbula: reporte de un caso clínico / Intraosseous hemangioma of the mandible: report of a clinical case

El hemangioma intramandibular es una neoformación benigna que resulta de la proliferación anormal de vasos sanguíneos, de origen desconocido. Estos tumores, por lo general, son hallazgos radiológicos y se caracterizan por ser asintomáticos y/o presentar movilidad de los dientes afectados, asimetría facial, parestesias y dolor, entre otros síntomas. Después de las vértebras y el cráneo, la mandíbula esel sitio más frecuentemente afectado, sobre todo en su parte posterior.El propósito de este trabajo es presentar un caso clínico de esta enfermedadpoco habitual.

Intraosseous hemangioma of the mandible is a benign neoplasm that originates from the abnormal proliferation of blood vessels, the cause of which is unknown. In general, these tumors are detected by means of X-ray and are characterized as asymptomatic or presenting mobility in the affected teeth, facial asymmetry, paresthesia and pain, among other symptoms. After the vertebrae and skull, the most frequently affected site is the jaw, particularly the posterior part. The purpose of this paper is to present a clinical case of this rare disease.

Hemangioma infantil / Infantile haemangioma

Introducción: los hemangiomas infantiles suponen uno de los principales motivos de consulta pediátrica. La nomenclatura para designarlo ha sufrido constantes cambios, lo que ha derivado cierta confusión. Es fundamental el conocimiento de la clasificación actual de las lesiones vasculares que pueden tener características clínicas similares, pero distinta evolución, pronóstico y tratamiento. Objetivo: profundizar y ampliar los conocimientos sobre el diagnóstico y el tratamiento actualizado del hemangioma infantil. Métodos: se realizó una revisión bibliográfica, la localización de artículos fue en las bases de datos informatizadas on-line Medline, Cochrane Library, Lilacs, así como el metabuscador Google. La búsquede se realizó solo entre 2009-2014. Síntesis de la información: los hemangiomas infantiles son los tumores benignos más frecuentes en lactantes. Se observan entre el 1,1 y 2,6 por ciento. Son más frecuentes en la raza blanca, en el sexo femenino (3:1 o 5:1). Se presentan de forma esporádica y pueden localizarse en cualquier región anatómica, con predilección en la cabeza y el cuello. Su diagnóstico es clínico, la mayoría son pequeños y no requieren de tratamiento. Conclusiones: se considera necesario la difusión del conocimiento sobre el hemangioma infantil, ya que somos los receptores iniciales de estos pacientes y su diagnóstico oportuno es vital para iniciar un tratamiento precoz en caso necesario(AU)

Introduction: infantile hemangioma is one of the main reasons for pediatric consultation. The nomenclature used to define this type of lesion has constantly undergone changes throughout history, which has resulted in some confusion. It is essential to have knowledge on the current classification of the different vascular lesions that may have similar clinical characteristics but different progression, prognosis and treatment. Objective: to delve into and broaden knowledge on the current diagnosis and treatment of infantile hemangioma. Methods: a literature review was made to locate articles in the most important on-line databases, including Medline, Cochrane Library, Lilacs, and the meta-searcher Google. The years of publications comprised 2009 through 2014. Data synthesis: infantile hemangiomas are the most frequent benign tumors in breastfed children. They account for 1.1 to 2.6 percent. They are more common in Caucasians, in females (3:1 or 5:1). They occur occasionally and t can be found in any anatomical region, mainly in the head and the neck. Their diagnosis is clinical, most of them are small and do not require treatment. Conclusions: it is necessary to spread the knowledge on infantile hemangioma as we are the first ones to receive these patients, so timely diagnosis is vital to start an early treatment if required(AU)

An Adult Male Presenting with a Left Gluteal Region Hemangioma – An Unusual Site.

The word “hemangioma” comes from the Greek haema- (αιμα), “blood”; angeio (αγγειο), “vessel”; -oma (-ωμα), “tumor”. A Hemangioma is a benign and usually selfinvoluting tumor of the endothelial cells that lines blood vessels, and is characterized by increased number of normal or abnormal vessels filled with blood. It usually appears in the initial weeks of life and grows most rapidly over the first six months. Usually, growth is complete and involution has commenced by twelve months. Half of all infantile hemangiomas have completed involution by age five, 70% by age seven, and most of the remainder by age twelve years1. In more severe cases hemangiomas may leave residual tissue damage. In infancy, it is the most common tumor 2.

Malformación vascular de bajo flujo sinovial de la rodilla (hemangiolinfangioma): Reporte de caso / Low-low synovial vascular malformation of the knee (hemangiolymphangioma): Case report

Introducción: La malformación vascular venosa de bajo lujo se desarrolla generalmente durante la primera infancia y puede ser una causa poco frecuente de derrame recurrente de larodilla. La clínica, las pruebas de laboratorio y las radiografías suelen ser inespecíficas. Objetivo: Describir una patología infrecuente en pediatría, para enfatizar en la correcta clasificación y sospecha. Caso Clínico: Paciente de 2 años con historia de episodios de derrame articular de la rodilla derecha que requirió múltiples hospitalizaciones y tratamientos antibióticos. Exámenes de laboratorio normales. La radiografía simple de la rodilla no mostró cambios óseos. La resonancia magnética reportó malformación vascular de bajo lujo. Se realizó resección quirúrgica que evidenció lesión vascular entre las fibras musculares del vasto lateral del cuádriceps con extensión hasta la cápsula de la rodilla y disección de las fibras hasta el vasto lateral de la pierna derecha. El estudio histológico fue compatible con malformación vascular de bajo lujo. Ante el hallazgo benigno y la evolución favorable, continuó con tratamiento ambulatorio. Conclusiones: Aunque la malformación vascular de bajo lujo sinovial es una patología poco frecuente en la población pediátrica, debería considerarse la realización de un diagnóstico diferencial en pacientes con aparición repetida de hemartrosis sin historia de coagulopatía ni hemofilia.

Introduction: Low-low vascular malformation, which usually develops during the first stage of infancy, is a rare cause of recurrent effusion of the knee. History, laboratory and X-rays are usually non-specific. Objective: To describe a rare disease in pediatrics, emphasizing the correct classification and suspicion. Case Report: A case is presented of a two-year-old patient with a history of effusion of the right knee who required multiple hospitalizations and antibiotic treatments. Laboratory work-up was normal. Plain X-rays of the knee revealed no bone changes. MR imaging reported low-low vascular malformation. Surgical resection was performed, evidencing vascular lesion among the muscle fibers of the vastus lateralis of quadriceps until the capsule of the knee, as well as dissection of the fibers until the vastus lateralis of the right leg. Histology was consistent with low-low vascular malformation. Due to the benign outcome and favorable evolution, an outpatient management was possible. Conclusions: Although low-low synovial vascular malformation is a rare disease among the pediatric population, it should be considered in the differential diagnosis of patients with repeated hemarthrosis and no history of either coagulopathy or hemophilia.

Manejo quirúrgico simultáneo de una paciente con cáncer de páncreas distal, tumores del estroma gastrointestinal (GIST) gástricos y hemangioma hepático / Simultaneous surgical management of a patient with distal pancreatic cancer, gastric gastrointestinal tumors (GIST) and hepatic hemangioma

Introduction: The occurrence of gastric Gastrointestinal Stromal Tumors (GIST) associated to pancreatic adenocarcinoma has been reported in 0.2 percent pancreatic cancers. There are no published reports on distal pancreatic adenocarcinoma associated to gastric antral GIST and the surgical management of this clinical condition. Case report: Herein, we discuss a 75 years-old female patient who was admitted to our institution with upper digestive hemorrhage. The endoscopy showed large, superficial erosions over the cardia and on the posterior wall of the antrum a rounded sub-mucosal non-eroded lesion suspected of gastric GIST. An abdominal computed tomography scan found a hepatic hemangioma on the left hepatic lobe. In the pancreatic distal body and tail a solid exophytic lesion was identified. In the gastric antrum a rounded submucosal tumor in close contact with the pancreatic lesion was found. The patient was subjected to distal pancreatectomy, splenectomy, and distal gastrectomy. The biopsy identified a well-differentiated ductal adenocarcinoma localized in the pancreatic tail and the proximal part of the body, resected with negative margins. The gastric tumor was positive for CD117, CD34, and DOG-1; it had a positive Ki67 in less than 2 percent, and 2 or less mitoses per 50 high-power fields. Conclusion. This uncommon case illustrates the occurrence of synchronous tumors of different cellular origins incidentally diagnosed and their simultaneous surgical treatment. The individual incidence of these tumors is low and if associated they probably will continue to be found incidentally.

Introducción: La ocurrencia simultánea de tumores del estroma gastrointestinal (GIST) del estómago con cáncer de páncreas, ha sido reportada en 0,2 por ciento. No existen reportes publicados sobre cáncer de páncreas distal asociado a GIST gástrico y el manejo de esta situación clínica. Caso clínico: Paciente de 75 años de edad, hospitalizada en nuestra institución por hemorragia digestiva alta. La endoscopía mostró erosiones superficiales sobre el cardias y en la pared posterior del antro una lesión submucosa redondeada no-ulcerada, sospechosa de un GIST. La tomografía abdominal demostró un hemangioma hepático en el lóbulo izquierdo, en la cola del páncreas se identificó una lesión sólida y en el antro gástrico se encontró un tumor redondeado en contacto con la lesión pancreática pero sin relación íntima con la misma. La paciente fue sometida a pancreatectomía distal, esplenectomía, gastrectomía distal y resección del hemangioma. La biopsia identificó en la cola y cuerpo distal del páncreas un adenocarcinoma ductal bien diferenciado con márgenes negativos. El GIST gástrico fue positivo para CD117, CD34 y DOG-1, el Ki67 fue positivo en menos de 2 por ciento y se identificaron 2 o menos mitosis por 50 campos de aumento mayor. Conclusión: Este caso ilustra la ocurrencia sincrónica de tres tumores de estirpes celulares diferentes diagnosticados incidentalmente y su tratamiento quirúrgico simultáneo. La incidencia individual de estos tumores en estas localizaciones es baja y su diagnóstico, seguramente, seguirá siendo incidental.

Spontaneous rupture of giant hepatic hemangioma

Hemangiomas are the most common benign tumors of the liver, with a prevalence ranging from 0.5 to 7% in necropsies. Tumors larger than 5 cm are called giant hemangiomas. Rupture is a very rare complication, although it is the most severe. We report the case of a 52-year-old patient who presented with a ruptured giant hepatic hemangioma. She was treated with a two-stage surgery, with successful outcome.

Hemangioma gigante del hígado / Liver giant hemangioma

El llamado hemangioma hepático gigante suele ser menos frecuente que aquellos que miden menos de 5 cm, los primeros causan síntomas en algunos de los casos y mayor riesgo de complicaciones como la ruptura traumática, por lo que su manejo puede ser de cierta complejidad ya que en la actualidad no existe un consenso para su tratamiento. Se presentó una paciente femenina, de 18 años de edad, que consultó por presentar dolor y masa palpable en epigastrio. En los estudios imaginológicos realizados se constató un tumor en lóbulo hepático izquierdo compatible con hemangioma gigante, lo que se corroboró mediante videolaparoscopia donde se observó una imagen vascular atípica. Se discutió el caso en un equipo interdisciplinario formado por los servicios de gastroenterología, cirugía e imaginología, que decidió realizar inicialmente quimioembolización de la arteria hepática con Gel FOAM y, en un segundo tiempo, la resección quirúrgica de la lesión, lo que permitió mayor accesibilidad quirúrgica, evitó complicaciones, además de una evolución posquirúrgica favorable

The so called giant hepatic hemangioma is usually less frequent than those smaller than 5 cm. The first ones cause symptoms in some cases and they increased the risk of complications such as traumatic rupture; so that their management can be rather complex since currently there is no consensus for their treatment. A 18 year-old female patient was received at consultation due to pain and palpable mass in the epigastrium. In imaging studies performed a tumor was found in the left hepatic lobe, which was compatible with giant hemangioma. It was corroborated by videolaparoscopy which showed an atypical vascular imaging. This case was discussed in an interdisciplinary team of the gastroenterology, surgery and imaging services. Chemoembolization to hepatic artery was initially decided with Gel FOAM and, in a second stage, surgical resection of the lesion was performed, which allowed greater surgical accessibility, avoiding complications, along with a positive surgical outcome