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.

Asociación de hemangioma en zona cérvico facial y hemangioma subglótico en un neonato / Association of cervical-facial and subglottic hemangioma in neonates / Associação de hemangioma cervicofacial e subglótico em neonatos

Se presenta un neonato con hemangioma cérvico facial y posterior diagnóstico de hemangioma subglótico. Los hemangiomas en el período neonatal y los primeros meses de vida requieren una atención cuidadosa. Debido a su patrón de crecimiento y la futura aparición de nuevas lesiones, son considerados imprevisibles en esta etapa. Se encontró una fuerte asociación entre los hemangiomas difusos de localización cérvico facial y los hemangiomas sintomáticos de la vía aérea alta. El riesgo está relacionado con el grado de extensión de la afectación cutánea en un área que incluye la piel de la región mandibular, el mentón, el labio inferior y la parte anterior del cuello. Los hemangiomas infantiles requieren tratamiento cuando presentan riesgo vital y alteraciones funcionales, como compromiso de la vía aérea.

This is the case of a newborn with cervical hemangioma and a subsequent diagnosis of subglottic hemangioma. Hemangiomas in neonates and infants require careful attention. Due to their growth pattern and the potential appearance of new lesions, they are considered unpredictable at this stage. A strong link was found between diffuse cervical-facial and symptomatic upper airway hemangiomas. The risk is related to the extent of skin involvement in a given area, which might include the jaw, chin, lower lip, and front of the neck skin. Infant hemangiomas require treatment when they present life-threatening and functional alterations, such as airway compromise.

Relatamos o caso de um recém-nascido com hemangioma cervical com diagnóstico posterior de hemangioma subglótico. Hemangiomas em recém-nascidos e lactentes requerem atenção cuidadosa. Devido ao seu padrão de crescimento e ao potencial aparecimento de novas lesões, são considerados imprevisíveis nessa fase. Uma forte associação foi encontrada entre hemangiomas cervicofaciais difusos e hemangiomas sintomáticos das vias aéreas superiores. O risco está relacionado à extensão do envolvimento da pele da mandíbula, o queixo, o lábio inferior e a pele da frente do pescoço. Os hemangiomas infantis necessitam de tratamento quando apresentam alterações funcionais ou risco de vida, como comprometimento das vias aéreas.

Manejo de hemangioma ulcerado segmentario: combinación de tratamiento sistémico y tópico en dos casos pediátricos / Management of ulcers on a segmental hemangioma: A combination of systemic and topical therapies in two pediatric cases

Los hemangiomas infantiles (HI) son los tumores benignos más frecuentes de la infancia; la variante segmentaria es rara y se asocia con un mal pronóstico. Una de sus principales complicaciones es la ulceración durante la fase de crecimiento del tumor, a pesar de no presentar características macroscópicas compatibles con una lesión agresiva. El manejo en estos casos es dificultoso e impone la necesidad de asociar múltiples estrategias, algunas orientadas específicamente a impedir la proliferación del hemangioma y otras orientadas a la curación de la herida, el manejo del dolor y la prevención de la infección agregada. Presentamos dos casos a fin de comunicar nuestra experiencia respecto del manejo de dicha patología y su evolución final.

Infantile hemangiomas (IHs) are the most common benign tumors of childhood, and segmental ones are rare and associated with a poor prognosis. While these tumors look harmless, one of their main related complications is ulceration during tumor growth. The management in these cases is extremely challenging, requiring a combination of multiple approaches, some specifically aimed at preventing the proliferation of the hemangioma and others aimed at wound care, pain management, and prevention of further infection. Here we discuss two cases to narrate our experience on the management of this condition and its outcome.

Morning glory disc anomaly associated with large facial infantile hemangioma as the presenting signs of PHACE syndrome / Anomalia de Morning Glory associada à extenso hemangioma infantil como apresentação da Síndrome PHACE

ABSTRACT Infantile hemangioma, the most common benign tumor in infancy, is usually an isolated condition occurring in many different locations in the body. However, large infantile hemangioma may be associated with other systemic malformations, including central nervous system, cerebrovascular, cardiac, and ophthalmology abnormalities, a condition termed PHACE syndrome. In this paper, we describe a case of PHACE syndrome that was presented with the unique association of a large facial infantile hemangioma and morning glory anomaly.

RESUMO O hemangioma infantil é a causa mais comum de tumor benigno na infância e usualmente é uma condição isolada podendo ocorrer em diferentes regiões do corpo. No entanto, hemangiomas infantil extensos podem ser associados com outras malformações sistêmicas incluindo anomalias no sistema nervoso central, cerebrovasculares, cardíacas e oftalmológicas, uma condição denominada síndrome PHACE. Neste trabalho, descrevemos o caso de um paciente com síndrome PHACE que se apresentou com um extenso hemangioma facial e anomalia de "morning glory".

Hemangioma infantil: actualización del tratamiento tópico y sistémico / Infantile hemangioma: an update in the topical and systemic treatments

Resumen Los hemagiomas infantiles (HI) son los tumores de tejidos blandos más frecuentes de la infancia. Se caracterizan por un crecimiento significativo durante los primeros meses de vida, seguido de una involución lenta y espontánea a lo largo de un periodo que puede durar algunos años. Usualmente, la regresión de la mayor parte del tumor termina a los 4 años de edad. Sin embargo, algunos de los HI desarrollan complicaciones, lo que resulta en alteraciones funcionales, dolor y desfiguramiento. La decisión de administrar tratamiento a un paciente con HI y elegir la mejor opción terapéutica para ese paciente (tratamiento tópico o sistémico) debe ser individualizada, dependiendo de varios factores: el tamaño de la lesión, la localización, la presencia de complicaciones como ulceración, el riesgo de cicatrización o desfiguramiento, la edad del paciente, la tasa de crecimiento o de involución al momento del diagnóstico, los riesgos y beneficios de administrar el tratamiento, la disponibilidad del medicamento, los costos y la experiencia del médico tratante.

Abstract Infantile hemagiomas (IH) are the most common soft tissue tumors in infancy. They are characterized by significant growth during the first months of life, followed by slow spontaneous involution over the ensuring years. The process of involution takes several years, but usually the regression of most of the tumors ends at 4 years of age. Unfortunately, some of the IH develop complications, resulting in functional impairment, pain and disfigurement. The decision to start treatment and the choice of the best therapeutic option (topic or systemic) should be individualized depending on several factors: the size of the lesion, the location, the presence of complications such as ulceration, the risk of scarring or disfigurement, the age of the patient, the rate of growth or regression at the time of diagnosis, the risks and benefits of the treatment, the availability of the medication, the costs, and the experience of the attending physician.

Tiempo de respuesta terapéutica al propranolol sistémico en el manejo de hemangiomas subglóticos pediátricos: serie de casos y revisión de la literatura / Therapeutic time of response of systemic propranolol in the management of pediatric subglottic hemangiomas: case series and review of the literature

Resumen: Introducción: Los hemangiomas de la vía aérea son neoplasias congénitas de baja incidencia. Su presentación clínica consiste en una obstrucción de la vía aérea superior con disnea progresiva y estridor, ocasionando una obstrucción grave, aguda y grave de la vía aérea. Los hemangiomas infantiles tienen una incidencia al nacimiento del 2%, incrementándose al 9% a los 12 meses de vida. Casi el 60% de los hemangiomas infantiles afectan la región de la cabeza y el cuello. Sin embargo, la localización en la vía aérea superior no es tan frecuente y solo se informa en el 1.8% de los pacientes con hemangiomas cutáneos. El tratamiento tradicional de los hemangiomas laríngeos ha sido la administración de corticoides sistémicos por periodos largos, la cirugía endolaríngea con láser de CO2, la cirugía abierta, la traqueostomía y, recientemente, el propranolol oral. Casos clínicos: Se presentan cinco casos de hemangiomas subglóticos tratados con propranolol sistémico y la evaluación del tiempo de respuesta terapéutica, definido como el control satisfactorio de los síntomas obstructivos y aseguramiento de la vía aérea. En estos casos, el propranolol mostró ser una opción efectiva y segura para el tratamiento de hemangiomas laríngeos, con un tiempo de respuesta terapéutica de 48 a 72 horas. Conclusiones: El tratamiento con propranolol oral se ha convertido en los últimos años en la primera opción terapéutica debido a su alta eficacia y su buen perfil de seguridad.

Abstract: Background: Airway hemangiomas are congenital neoplasms of low incidence. Its clinical presentation consists of obstruction of the upper airway with progressive dyspnea and stridor and the life of the patient at risk. Infantile hemangiomas have an incidence at birth of 2%, increasing to 9% at 12 months of life. Almost 60% of childhood hemangiomas affect the head and neck region. However, localization in the upper airway is not as frequent and it is only reported in 1.8% of patients with cutaneous hemangiomas. The traditional treatment of laryngeal hemangiomas has been the administration of systemic corticosteroids for long periods, endolaryngeal surgery with CO2 laser, open surgery, tracheostomy or, recently, oral propranolol. Case report: Five cases of subglottic hemangioma treated with systemic propranolol are presented. The therapeutic time of response with the satisfactory control of obstructive symptoms and assurance of the airway was evaluated. In these cases, propranolol was shown to be an effective and safe option for the treatment of laryngeal hemangiomas with a therapeutic response time of 48 to 72 hours. Conclusions: In recent years, the treatment with oral propranolol has become the first therapeutic option due to its high efficacy and safety profile.

Quistes subamnióticos en el contexto de restricción de crecimiento fetal / Subamniotic cysts in the context of growth restriction fetal

RESUMEN La restricción de crecimiento fetal (RCF) es el término con el que se define a aquellos fetos que no alcanzan el potencial de crecimiento intraútero esperado debido a factores genéticos o ambientales. Dentro de las causas de RCF asociadas a la placenta encontramos mosaicismo confinado a la misma, enfermedad isquémica placentaria y anomalías estructurales a este nivel. Se presenta el caso de una paciente de 32 años con diagnóstico de RCF que asocia múltiples quistes econegativos subamnióticos en la cara fetal de la placenta. Se finaliza la gestación en semana 34 mediante cesárea electiva por ausencia de diástole en el estudio Doppler de la arteria umbilical, evidenciando cinco quistes subamnióticos de 4 a 6 cm que alteran la superficie del amnios.

ABSTRACT Fetal growth restriction (FGR) is the term used to define those fetuses that do not reach the expected intrauterine growth potential due to genetic or environmental conditions. The causes of FGR associated with the placenta are confined placental mosaicism, placental ischemic disease and placental structural abnormalities. We report a case of a 32-year-old patient with a diagnosis of FGR associated with multiple subamniotic econegative cysts overlaying the fetal plate of the placenta. The gestation is ended in week 34 by elective cesarean section due to absence of diastolic flow in the umbil-ical artery, showing five subamniotic cysts from 4 to 6 cm that disrupt the surface of the amnion.

Hemangioma de crecimiento mínimo o detenido con lipoatrofia: presentación de un caso clínico / Hemangioma with minimal or arrested growth

Los hemangiomas de la infancia (HI) son tumo-res vasculares que presentan un patrón evolutivo característico. Existe un subgrupo de HI que pre-senta un tipo de crecimiento distinto denominados hemangiomas de la infancia con crecimiento míni-mo o detenido (HI-CMD). En estos, el componen-te proliferativo no supera el 25% de la superficie total; además, afecta a niños de al menos dos meses de edad en la consulta inicial o a niños menores de dicha edad en los que se realiza un seguimiento mayor a 2 meses. Los HI- CMD con lipoatrofia son una variante poco frecuente de HI de la cual exis-ten escasos reportes en la literatura. El objetivo de nuestro trabajo es presentar un caso de HI-CMD con lipoatrofia en una lactante. Los HI-CMD presentan un desafío diagnóstico ya que deben ser reconocidos precozmente y diferencia-dos de otras lesiones vasculares.

Infantile hemangiomas (IH) are vascular tumors that present a characteristic evolutive pattern. There is a subgroup of hemangiomas that pre-sents a different type of growth called infantile hemangiomas with minimal or arrested growth (IH-MAG). In these, the proliferative component does not exceed 25% of the total surface; it also affects children of at least two months of age in the initial consultation or children under that age in which a follow-up of more than 2 months is carried out. IH-MAG with lipoatrophy is a rare variant of IH of which there are few reports in the literature.The aim of our report is to present a case of IH-MAG with lipoatrophy in an infant. IH-MAGs present a diagnostic challenge since they must be recognized early, and differentiated from other vascular lesions.

Facial nerve hemangioma in the middle ear / Hemangioma de nervo facial em orelha média

ABSTRACT Facial nerve hemangioma is a rare and benign vascular tumor, and accounts for 0.7% of intratemporal tumors. We report the second case described in the literature of a facial nerve hemangioma in its tympanic segment. A 14-year-old male patient presented with a history of progressive right ear hearing loss with preserved facial mimicry. Pure tone audiometry showed a right ear moderate conductive hearing loss. Magnetic resonance imaging demonstrated an expansive lesion involving the tympanic segment of the right facial nerve, suggestive of hemangioma. Watchful waiting was chosen as management. In the first case of middle ear facial hemangioma described in the literature, facial palsy was the symptom that led the patient to seek medical care. In the present case, it can be inferred that the first symptom was conductive hearing loss ipsilateral to the lesion. Facial palsy may not be present and the clinical presentation may resemble otosclerosis, ossicular chain disruption, and third window abnormalities, among other differential diagnoses of conductive hearing loss. The second case of tympanic portion facial nerve hemangioma is reported, describing the specificity of conductive hearing loss as its only clinical manifestation.

RESUMO O hemangioma do nervo facial é um tumor vascular raro e benigno, que corresponde a 0,7% dos tumores intratemporais. Apresentamos o segundo caso descrito na literatura de hemangioma do nervo facial em sua porção timpânica. Paciente de 14 anos, sexo masculino, apresentando hipoacusia progressiva em orelha direita com mímica facial preservada. A audiometria evidenciou perda condutiva moderada à direita. A ressonância magnética demonstrou lesão expansiva, comprometendo o segmento timpânico do nervo facial direito, sugestiva de hemangioma do nervo facial. A conduta foi expectante. No primeiro caso descrito na literatura de hemangioma do facial em orelha média, o sintoma que levou o paciente ao atendimento médico foi paralisia facial. No presente caso, pode-se inferir que o primeiro sintoma foi a perda auditiva condutiva ipsilateral à lesão. A paralisia facial pode não estar presente, e o quadro clínico pode se assemelhar à otosclerose, disjunção de cadeia e síndrome da terceira janela, dentre outros diagnósticos diferenciais de perdas auditivas condutivas. O artigo relata o segundo caso de hemangioma do nervo facial em sua porção timpânica, mostrando a peculiaridade de perda auditiva condutiva como única manifestação clínica.

Angiomas venosos y cefalea en pediatría: un caso clínico / Venous angiomas and headache in children: A case report

Introducción: Los angiomas venosos (AV) son entidades benignas que infrecuentemente presentan síntomas. Objetivo: Reporte de un caso y revisión del tema. Caso clínico: Escolar, de sexo femenino, 6 años, que inicia cefaleas bifrontales frecuentes 3-4 veces por semana, en relación con trabajo académico, de carácter opresivo, no pulsátil, sin náuseas ni vómitos, rango de intensidad entre 4-6/10. Se inició manejo con calendario de cefaleas, apoyo escolar y evaluación psicológica. La tomografía computarizada cerebral solicitada informó de angioma venoso de núcleo caudado izquierdo, razón por la cual se realizó una resonancia magnética cerebral más angiorresonancia que confirmó angioma venoso y excluyó complicación o asociación a otra malformación vascular. La cefalea respondió bien a terapia psicopedagógica y psicológica. La frecuencia de cefaleas disminuyó a 10-12 al año, agrupadas en periodos de mayores demandas académicas. La paciente es controlada durante 12 años, hasta el egreso de la enseñanza media, sin complicaciones y con un buen manejo de la cefalea tensional. Conclusión: En el estudio de una cefalea el hallazgo de un AV puede ser incidental; una vez reunidos los criterios internacionales de cefalea tensional e iniciado el tratamiento para ello, la monitorización de los AV debe ser clínica. Las complicaciones del AV son infrecuentes y el tratamiento quirúrgico es excepcional.

Introduction: Venous angiomas (VA) are benign entities; however infrequent symptomatic cases may occur. Objective: Case report and literature review. Case report: A 6 year old girl was referred with a history of bi-frontal, non-pulsatile, headache with no nausea or vomiting. Headache intensity was 4-6/10. The episodes were frequent, 3-4 times per week. Triggers include academic work. Computed tomography showed a small VA in left caudate nucleus, which was confirmed by a brain MRI, with no evidence of inflammatory or ischaemic changes, or another vascular malformation. Psychological and psycho-pedagogic techniques were used, combined with relaxation and cognitive-behavioural techniques to reduce the intensity and frequency. There was a good outcome, and the headache decreased to 10 episodes per year. The patient was monitored for 12 years until graduation from high school. The VA remained without complications. Conclusions: In the study of a headache, a VA usually is an incidental finding. The International Classification of Headache Disorders III provides specific criteria of frequent episodic tension-type headache, and allows us begin specific therapy for it. Monitoring of non-symptomatic VA cases should be clinical. The surgical management of these entities is exceptional.

Prevalencia de las anomalías vasculares en niños menores de cinco años del municipio Cerro / Prevalence of vascular anomalies in children aged less than five years in Cerro municipality

Introducción: las anomalías vasculares son motivo frecuente de consultas en la infancia; en Cuba se desconoce su prevalencia de las mismas, así como las conductas tomadas y las complicaciones que aparecen en la edad pediátrica. Objetivo: determinar la prevalencia de las anomalías vasculares en niños menores de cinco años del municipio Cerro. Métodos: estudio descriptivo exploratorio intencionado de corte transversal con técnica de muestreo aleatorio simple, en el período entre septiembre de 2013 a febrero de 2014. Se requirió una muestra de 312 niños de los 5 343 que conformaron el universo de estudio. La tasa de prevalencia se expresó por cada 1 000 niños. Resultados: se identificaron tres pacientes del sexo femenino con antecedentes patológicos familiares y personales de hemangioma. Se encontró que la tasa de prevalencia fue de 9,6 × 1 000 niños. El cuero cabelludo fue el sitio más afectado con el 66,7 por ciento (n= 2). No se constataron complicaciones en ninguno de los casos. Solo una paciente recibió tratamiento con esteroides sistémicos (n= 1; 33,4 por ciento). Conclusión: la tasa de prevalencia encontrada (9,6 × 1 000) de las anomalías vasculares en niños menores de cinco años pertenecientes al municipio Cerro es similar a la registrada mundialmente. Su mayor frecuencia se encuentra en el sexo femenino(AU)

Introduction: vascular anomalies are frequent reason for going to the doctor's in children, but their prevalence, behaviors to be followed and the complications at pediatric ages barely known in Cuba. Objective: to determine the prevalence of vascular anomalies in children aged less than five years in Cerro municipality. Methods: intentional cross-sectional, exploratory and descriptive study performed with simple random sampling technique, in the period from September 2013 to February 2014. The final sample was 312 children out of a study universe of 5 343 ones. The prevalence rate was estimated per 1 000 children. Results: three female patients were found to have personal and family history of hemangioma, the prevalence rate was 9.6 per 1 000 children. The scalp was the most affected area with 66.7 percent (n= 2). No complications were observed. Just one patient was treated with systemic steroids (n= 1; 33.4 percent). Conclusions: the prevalence rate for vascular anomalies in children less than five years old was 9.6 per 1 000 children in Cerro municipality, which is similar to that reported worldwide. It is more frequent in females(AU)

Coriaoangioma placentero / Pleasant cariaoangioma

El corioangioma es el tumor benigno más frecuente de la placenta con una incidencia de 1%. Usualmente son asintomáticos y muchos pasan desapercibidos. Los tumores grandes pueden causar complicaciones maternas y fetales graves por lo que es importante realizar diagnóstico prenatal temprano. El pronóstico de un embarazo con corioangioma gigante de placenta, depende fundamentalmente del tamaño y de la detección oportuna.

Hemangioma: descrição de um caso clínico e sua importância no diagnóstico diferencial / Hemangioma: description of a clinical case and the importance in the differential diagnosis

O hemangioma infantil é um tumor vascular benigno que ocorre devido a uma proliferação anormal dos vasos sanguíneos. O quadro clínico apresenta três fases bem definidas: proliferativa, involutiva e involuída. O diagnóstico é realizado basicamente por meio da anamnese e do exame físico, e quando necessário preconiza-se avaliação histopatológica. O presente trabalho, descreve um caso clínico de um hemangioma presente em um bebê de 3 meses de idade que foi, de principio, diagnosticado como mucocele ou fibroma. A cirurgia excisional foi realizada eo material encaminhado para análise histopatológica, confirmando o diagnóstico de hemangioma. Nessas situações, vale ressaltar a importância do diagnostico diferencial, manobra cirúrgica adequada e a avaliação das características clínicas da lesão para evitar possíveis complicações cirurgicas.

The infantile hemangioma is a benign vascular tumor which occurs due to an abnormal proliferation of blood vessels. The clinical features three well-defined phases: proliferative, involution,and involuted. The diagnosis is made primarily by clinical history and physical examination, but when necessary, help to close the histopathological diagnosis. This paper describes a clinical case of a gift hemangioma in a baby three months old who was, in principle, diagnosed as mucocele or fibroma. The excisional surgery was performed and material sent for histopathological confirmation hemangioma. It is worth emphasizing the importance of differential diagnosis, appropriate surgical maneuver, assessment of clinical characteristics of the lesion to prevent potential surgical complications possible.

Hepatic Hemangioma with Kasabach-Merritt Syndrome in an Adult Patient / 대한소화기학회지

Hemangiomas are the most common benign tumors of the liver. They are generally asymptomatic, but giant hemangiomas can lead to abdominal discomfort, bleeding, or obstructive symptoms. Kasabach-Merritt syndrome is a rare but life-threatening complication of hemangioma, characterized by consumptive coagulopathy with large vascular tumors. More than 80% of Kasabach-Merritt syndrome cases occur within the first year of life. However, there are few reports of Kasabach-Merritt syndrome with giant hepatic hemangioma in adults and, as far as we know, no reports of Kasabach-Merritt syndrome with hepatic hemangioma treated with first line medical treatment only. The most important treatment for this syndrome is removal of the large vascular tumor. However, surgical treatment entails risk of bleeding, and the patient's condition can mitigate against surgery. We herein present a case of unresectable giant hepatic hemangioma with disseminated intravascular coagulopathy. The patient was a 60-year-old woman who complained of hematochezia, ecchymosis, and abdominal distension. She refused all surgical management and was therefore treated with systemic glucocorticoids and beta-blockers. After two weeks of steroid therapy, she responded partially to the treatment. Her laboratory findings and hematochezia improved. She was discharged on hospital day 33 and observed without signs of bleeding for three months.

Imaging findings of mimickers of hepatocellular carcinoma

Radiological imaging plays a crucial role in the diagnosis of hepatocellular carcinoma (HCC) as the noninvasive diagnosis of HCC in high-risk patients by typical imaging findings alone is widely adopted in major practice guidelines for HCC. While imaging techniques have markedly improved in detecting small liver lesions, they often detect incidental benign liver lesions and non-hepatocellular malignancy that can be misdiagnosed as HCC. The most common mimicker of HCC in cirrhotic liver is nontumorous arterioportal shunts that are seen as focal hypervascular liver lesions on dynamic contrast-enhanced cross-sectional imaging. Rapidly enhancing hemangiomas can be easily misdiagnosed as HCC especially on MR imaging with liver-specific contrast agent. Focal inflammatory liver lesions mimic HCC by demonstrating arterial-phase hypervascularity and subsequent washout on dynamic contrast-enhanced imaging. It is important to recognize the suggestive imaging findings for intrahepatic cholangiocarcinoma (CC) as the management of CC is largely different from that of HCC. There are other benign mimickers of HCC such as angiomyolipomas and focal nodular hyperplasia-like nodules. Recognition of their typical imaging findings can reduce false-positive HCC diagnosis.

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.

Puesta al día en hemangiomas de la infancia / Update in infantile haemangioma

Los hemangiomas de la infancia (HI) son los tumores de partes blandas más frecuentes de la infancia. Se presentan en un 5 por ciento- 10 por ciento de la población pediátrica. El comportamiento de estos tumores es especial, ya que, a diferencia de otras neoplasias benignas, los HI tienden a regresar e involucionar espontáneamente en un 90 por ciento. Algunos pacientes con HI pueden sufrir complicaciones tales como ulceración y hemorragia. Estas complicaciones han llevado a la búsqueda de alternativas terapéuticas, donde, por mucho tiempo fueron los esteroides la primera opción de tratamiento. Esto hasta el año 2008, cuando se descubre accidentalmente el propranolol administrado por vía oral, como alternativa terapéutica para los HI severos, convirtiéndose hasta el día de hoy, en el gold standard de tratamiento. Dado la diversidad en su presentación, y las diferentes alternativas de manejo existentes, es que esta revisión pretende abordar la patogenia, clínica y enfrentamiento de estos tumores de la infancia.

Hemangiomas of infancy (HI) are the most common soft tissue tumors of childhood. They occur in up to percent-10 percent of the pediatric population and they have a special behaviour because, unlike other benign neoplasms, HI tend to regress spontaneously in up to 90 percent of the cases. Complications such as ulceration and hemorrhage can occur. These complications have led to search for new therapeutic options. Steroids were the first choice of treatment for a long time, until 2008, when oral propranolol was accidentally discovered as a potential treatment for large HI4. Today, propranolol is the gold standard for treatment. Given their variable clinical presentation and different therapeutic options available nowadays, this paper to review the pathogenesis, clinical presentation and approach of these tumors of infancy.

Ovarian hemangioma with stromal luteinization and HCG-producing mononucleate and multinucleate cells of uncertain histogenesis: A rare co-existence with therapeutic dilemma.

A 21 year old female presented with amenorrhea, hirsutism and change in voice along with an elevated serum β-HCG (human chorionic gonadotrophin) level and normal CA-125 level. Laparotomy revealed an enlarged right ovary measuring 6 × 5 × 1 cms with presence of an ovarian hemangioma along with stromal luteinization and HCG producing mononucleate as well as multinucleate cells of uncertain histogenesis on histopathological examination. Immunohistochemistry for inhibin and calretinin were positive in the luteinized component whereas β-HCG and Ki-67 were positive in the multinucleate cell component. The diagnostic rarity and therapeutic dilemma of such a rare mixed tumor within a single ovary has proven to be an exceptional case and an excellent investigative opportunity.