Trasplante hepático por hemangioendotelioma hepático difuso / Liver transplantation for diffuse epithelioid hemangioendothelioma

RESUMEN El hemangioendotelioma epiteloide hepático (HEH) es un tumor vascular, de etiología no aclarada, extraordinariamente infrecuente. La ausencia de características clínicas, analíticas y radiológicas especificas dificulta su correcto diagnóstico. El tratamiento del HEH depende del tamaño y localización tumoral, la extensión extrahepática y la condición médica del paciente. Entre las posibles opciones se encuentra el trasplante hepático, que obtiene unos buenos resultados clínicos, aunque el riesgo de recidiva no es despreciable. Presentamos un nuevo caso de HEH tratado mediante trasplante hepático.

ABSTRACT Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare vascular tumor of unclear etiology. The diagnosis is difficult due to the absence of specific clinical characteristics, laboratory tests results and radiological findings. The management of HEHE depends on tumor size, location, extrahepatic extension, and patients' medical status. Liver transplantation is one of the possible options with good clinical results, although the risk of recurrence is not negligible. We present a new case of HEHE managed with liver transplantation.

Tratamiento multidisciplinario de un sarcoma recurrente de la fosa isquiorrectal / A successful multidisciplinary approach to a recurrent ischiorectal fossa sarcoma

Los tumores de la fosa isquiorrectal son poco frecuentes, habitualmente descritos en reportes o series de casos. Estas lesiones son un reto terapéutico, requiriendo un estudio preoperatorio apropiado, además de discusión y manejo guiado por un comité multidisciplinario, que permiten lograr resultados óptimos tanto oncológicos como funcionales. Presentamos un caso de un hombre de 73 años con antecedentes de resección de un tumor glúteo izquierdo en otro centro 5 años antes. La biopsia fue compatible con un sarcoma epiteloideo (SE) de alto grado, con margen quirúrgico < 0,5 mm que requirió ampliación de los márgenes posteriormente. Además, se realizó radioterapia adyuvante. Al cuarto año de seguimiento el paciente desarrolla dolor e induración con retracción en relación a cicatriz quirúrgica, siendo objetivada una recidiva tumoral local. Luego de una discusión multidisciplinaria, se realizó una desfuncionalización con ileostomía en asa laparoscópica y resección tumoral con preservación del ano y del piso pélvico. El defecto fue cubierto por el equipo de cirugía plástica utilizando un colgajo perforante de la arteria glútea superior. La biopsia confirmó la recidiva tumoral y los márgenes quirúrgicos fueron negativos. El paciente es dado de alta a los 25 días postoperatorios por cuidados del colgajo, sin complicaciones. Al año de seguimiento el paciente no presenta recidiva tumoral, la ileostomía fue cerrada, y sus resultados funcionales en términos defecatorios y de la herida son buenos.

Ischiorectal fossa tumors are rare lesions, mostly described in case reports or case series. These lesions represent a diagnostic and therapeutic challenge. Hence, an appropriate preoperative study and multidisciplinary discussion are essential to achieve good oncologic and functional results. We report a case of a 73-year-old male operated on five years before in another health center due to the diagnosis of a left gluteal tumor. The lesion was excised, and biopsies confirmed a high-grade epithelioid sarcoma with a close margin, requiring a subsequent wider excision of the surgical margins. The patient received adjuvant radiotherapy. After four years of follow-up, the patient developed mild pain with skin retraction around the former incision. A local recurrence was diagnosed by imaging. In a multidisciplinary team meeting, a decision to resect the lesion with preservation of the anus and the pelvic floor was taken. The patient underwent a laparoscopic defunctioning loop ileostomy and a resection of the recurrent tumor in the ischiorectal fossa with preservation of the anal sphincter. The defect was covered utilizing a superior gluteal artery perforator flap and a partial gluteus maximus muscle rotation. The tumor was completely excised with negative margins. The patient was discharged without complications after 25 days due to flap management. After one year of follow-up, the patient is recurrence-free, and the ileostomy was closed.

Trasplante hepático en hemangioendotelioma epiteloide hepático con metástasis pulmonares: reporte de un caso / Liver transplantation in hepatic epitheloid hemangioendothelioma and lung metastases: a case report

El hemangioendotelioma epiteloide hepático (HEHE) es un tumor vascular raro de menor malignidad que el hemangiosarcoma. En los poco frecuentes casos unilobulares, puede indicarse hepatectomía parcial con riesgo de recurrencia agresiva; en enfermedad hepática extensa, incluso con compromiso extrahepático, el trasplante hepático ha resultado efectivo. Las metástasis son más frecuentes en pulmón,peritoneo, ganglios linfáticos, bazo y sistema nervioso. Se presenta el caso de un adolescente asintomático con HEHE con metástasis pulmonares y compromiso ganglionar abdominal que recibió trasplante hepático con evolución favorable.

Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor of less malignancy than hemangiosarcoma. In the rare unilobar cases, partial hepatectomy may be indicated with risk of aggressive recurrence; in extensive liver disease, even with extrahepatic involvement, liver transplantation has been performed successfully. Metastases are more common in the lung, peritoneum, lymph nodes, spleen, and nervous system. We present the case of an asymptomatic adolescent with HEHE with lung metastases and abdominal lymph node involvement who received a liver transplant with a favorable outcome.

Sarcoma epitelioide de tipo proximal de vulva

El sarcoma epitelioide es un tipo raro de tumor mesenquimal. El sarcoma epitelioide de tipo proximal de vulva es un tumor maligno extremadamente raro de los tejidos blandos, con frecuentes recurrencias por su comportamiento agresivo. Debido a que sus características son similares a otras lesiones benignas, esto lleva a retrasos diagnósticos y terapéuticos. Las características histológicas de este tumor incluyen células epitelioides prominentes y aparición de rasgos rabdoides con marcadas atipias nucleares. Debido a su baja incidencia, no existen algoritmos diagnósticos basados en la evidencia ni recomendaciones publicadas para el tratamiento. El pronóstico es generalmente malo. Se presenta un caso de sarcoma epitelioide de tipo proximal de vulva.

Epithelioid sarcoma is a rare type of mesenchymal tumor. Proximal-type epithelioid sarcoma of the vulva is an extremely rare malignant tumor of the soft tissues with frequent recurrences due to its aggressive behaviour. Because its characteristics are similar to other benign lesions, this leads to diagnostic and therapeutic delays. Histologic features of this tumor include prominent epithelioid cells and the appearance of rhabdoid features with marked nuclear atypia. Due to its low incidence, there are no evidence-based diagnostic algorithms or published recommendations for treatment. The prognosis is generally poor. A case of proximal type epithelioid sarcoma of the vulva is presented.

Portal hypertension as an uncommon presentation of hepatic epithelioid hemangioendothelioma: a case report

Hepatic epithelioid hemangioendothelioma (HEHE) is a vascular tumor with a low incidence rate. We report a case of a 26-year-old man who was referred to our hospital with a misdiagnosis of liver cirrhosis. On physical examination, ascites was noted. Chest and abdominal computer tomography scans showed coalescent lesions involving the peripheral liver with heterogeneous contrast enhancement and portal vein dilation due to portal hypertension. Extrahepatic metastasis was not observed. The biopsy with immunohistochemical stains suggested HEHE (Factor VIII, CD31, and CD34). This report describes an uncommon case of HEHE with non-cirrhotic portal hypertension.

El hemangioendotelioma epitelioide hepático (HEHE) es un tumor vascular con una tasa de incidencia baja. Presentamos el caso de un varón de 26 años que fue remitido a nuestro hospital con un diagnóstico erróneo de cirrosis hepática. En el examen físico se observó ascitis. La tomografía computarizada de tórax y abdomen mostró lesiones coalescentes que afectaban al hígado periférico con realce heterogéneo de contraste y dilatación de la vena porta por hipertensión portal. No se observó metástasis extrahepática. La biopsia con tinciones inmunohistoquímicas sugirió HEHE (Factor VIII, CD31 y CD34). Este informe describe un caso poco común de HEHE con hipertensión portal no cirrótica.

Consideraciones del Leiomioma y su Variante Intraósea en Mandíbula, Algoritmo de Trabajo Según Análisis de Literatura / Considerations of Leiomyoma and its Intraosseous Variant in the Jaw, Working Algorithm According to Literature Analysis

RESUMEN: El Leiomioma es una neoplasia benigna originada en el tejido muscular liso por lo que puede manifestarse en cualquier región del cuerpo humano que contenga músculo liso, siendo el sitio más común el útero, la piel y en el tracto gastrointestinal. La escasa cantidad de tejido muscular liso en boca hace que su manifestación oral sea infrecuente, representando solo 0.06 % del total de este tipo de tumores. Los sitios más afectados suelen ser los labios, las mejillas, el paladar, la lengua y encías. Histológicamente se distinguen tres tipos: el Leiomioma Sólido, Angioleiomioma (Vascular) y Leiomioma Epitelioide (Leiomioblastoma). El origen de esta neoplasia en boca suele ser la túnica media de los vasos sanguíneos. El Leiomioma intraóseo suele ser aún menos frecuente, y con un diagnóstico diferencial complejo, con histopatología que en varias ocasiones no suele ser fácil de clasificar. Si bien es definida como un tumor benigno, su manifestación intraósea puede llegar a ser localmente agresiva y con un diagnóstico controversial, debiendo abordarse muchas veces como una neoplasia maligna. El objetivo de este artículo es presentar una revisión de la literatura de esta variante intraósea de Leiomioma situada en mandíbula, sus consideraciones clínicas y un algoritmo de tratamiento.

ABSTRACT: Leiomyoma is a benign neoplasm, the origin is the smooth muscle tissue that can be found in any area of the human body, which contains smooth muscle tissue. The most common regions it can be located, are the uterus, the skin and the gastrointestinal tract. The low quantity of muscle tissue in the mouth leads to infrequent oral manifestation, representing only 0.06 % of these tumors. The most affected regions are the lips, cheeks, palate, tongue and gums. The Histologic classification is: Solid Leiomyoma, Angioleiomyoma (vascular) and Epithelioid Leiomyoma (Leiomyoblastoma). The origin of this tumor in the mouth is the tunica media of the blood vessels. Nevertheless, and in spite of being defined as a benign tumor, it can be extremely aggressive, be subject to controversial diagnosis, and must often be treated as a malign neoplasm. The Intraosseous Leiomyoma is infrequent and presents a complicated differential diagnosis, with a histopathology that many times cannot be easily classified. The aim of this article is to present a review of intraosseous variant Leiomyoma in the mandible, the clinicians´ considerations and a treatment algorithm.

Hemangioendotelioma epitelioide hepático de presentación como lesión focal incidental única: reporte de caso / Hepatic epithelioid hemangioendothelioma of presentation as single focal incidental lesion: case report

El hemangioendotelioma epitelioide hepático (HEH) es un tumor vascular maligno poco frecuente, de origen endotelial, de lenta progresión y de bajo grado de malignidad. Suele presentarse con mayor compromiso en mujeres, sin una etiología conocida. La clínica es variable, desde casos asintomáticos hasta dolor abdominal. Las características imagenológicas más frecuentes son lesiones nodulares hepáticas coalescentes, de tamaño variable, que muestran apariencia de "lesión en diana" en tomografía computarizada (TC) y resonancia magnética (RM), con retracción capsular. El principal diagnóstico diferencial son las metástasis hepáticas. El curso evolutivo es variable e imprevisible, con una mortalidad posterior al diagnóstico de hasta el 50% de los pacientes. Las modalidades terapeúticas incluyen la extirpación quirúrgica de la lesión y el trasplante hepático como opciones más útiles, además de la quimioterapia regional/sistémica y tratamiento inmunológico. El presente caso describe las principales características clínico-patológicas de este raro tumor, con la particularidad de su forma de presentación como lesión focal incidental única, además de la modalidad terapéutica utilizada (extirpación quirúrgica) en este tipo de lesión.

Hepatic epithelioid hemangioendothelioma (HEH) is a very rare malignant vascular tumor of endothelial origin, of slow progression and low malignity degree. It is more common in women, of unknown etiology. Clinical manifestations of HEH are non-specific, from asymptomatic to abdominal pain. The more frequent radiological features are nodular hepatic lesions, of variable size, which show appearance of "target sign" in computed tomography (CT) and magnetic resonance imaging (MRI), with capsular retraction. Hepatic metastases are the principal differential diagnosis. The clinical course is variable, with a mortality rate to the diagnosis of up to 50 % of the patients. The therapeutical options include the surgical extirpation (removal) of the tumor and hepatic transplant as the more useful, besides the regional /systemic chemotherapy and immunological treatment. The present case describes the principal clinical pathological characteristics of this rare tumor, with the particularity of its form of presentation as focal lesion, besides the therapeutical modality used (surgical removal) in this type of illness.

Hemipelvectomy with laparoscopic abdominoperineal excision for epithelioid sarcoma treatment / Hemipelvectomia com amputação laparoscópica do reto para tratamento de sarcoma epitelioide

Abstract The epithelioid sarcoma (ES) is an unusual variant of the sarcoma, not reaching 1% of all soft tissue sarcomas. Initially reported in 1970, it has a more aggressive variant, which was classified as the "proximal-type" in 1997. These are aggressive tumors with high rates of distant metastasis and local relapses. Isolated radio and chemotherapy responses are poor and free-margin surgical resection is the treatment of choice.This is the case report of a 25-year-old male patient diagnosed with "proximal-type" ES in the perineal region. He underwent surgical resection in another institution and was later admitted to our institution with local recurrence with rectal and left thigh muscle invasion. Neoadjuvant radio-chemotherapy was performed, followed by laparoscopic abdominoperineal rectal resection and partial left hemipelvectomy associated with left lower-limb amputation. The patient had no postoperative complications and is currently undergoing outpatient follow-up. The anatomopathological analysis showed tumor-free margins.The "proximal-type" ES is a rare soft-tissue sarcoma subtype. High local recurrence, as well as the metastasis rates, makes its treatment a challenging task.

Resumo O sarcoma epitelióide (SE) é um subtipo incomum de sarcoma, não chegando a 1% dos sarcomas de partes moles. Foi inicialmente descrito em 1970, sendo que em 1997 uma variante mais agressiva foi classificada como "tipo proximal". São tumores agressivos, com altas taxas de metástases e recidiva local, com resposta pobre à quimioterapia e radioterapia isoladas. A ressecção cirúrgica com margens livres é o tratamento padrão.Apresentamos o caso de um paciente de 25 anos com SE tipo proximal avançado em região perineal, submetido a ressecção em outro serviço, evoluindo com recidiva local com invasão do canal anal e musculatura da coxa esquerda. Foi realizado tratamento radioquimioterápico neoadjuvante seguido de ressecção cirúrgica com amputação abdominoperineal do reto por videolaparoscopia associada a hemipelvectomia parcial esquerda incluindo o membro inferior esquerdo (MIE). O paciente apresentou boa evolução pós operatória e encontra-se em segmento ambulatorial. O anatomopatológico evidenciou margens cirúrgicas livres.O SE proximal é um tipo raro de sarcoma de partes moles, cujo tratamento curativo é desafiador, em virtude dos altos índices de recidiva local e metástases à distância.

Hepatic epithelioid hemangioendothelioma: a report from three university centers / Hemangioendotelioma epitelioide hepático: pesquisa em três centros universitários

Abstract Objective: To determine common imaging findings of hepatic epithelioid hemangioendothelioma on magnetic resonance images. Materials and Methods: A search was made of three institutional databases between January 2000 and August 2012. Seven patients (mean age, 47 years; range, 21-66 years; 6 women) with pathology-confirmed diagnosis of hepatic epithelioid hemangioendothelioma who had undergone magnetic resonance imaging were identified. None of the patients had received any treatment for hepatic epithelioid hemangioendothelioma at the time of the initial magnetic resonance imaging examination. Results: Hepatic epithelioid hemangioendothelioma tumors appeared as focal masses in 7/7 patients, greater than 5 in number, with a coalescing lesion in 1/5, and peripheral localization in 6/7. Capsular retraction was present in 4/7, and was associated with peripherally located lesions. Early ring enhancement was appreciated in the majority of lesions in 7/7 patients. Centripetal progressive enhancement was shown in 5/7 patients on venous phase that exhibited a distinctive thick inner border of low signal on venous phase images, and a central core of delayed enhancement. Small lesions did not show this. Conclusion: The combination of multifocal round-configuration lesions that are predominantly peripheral and exhibit early peripheral ring enhancement and late appearance of an inner thick border of low signal and central core of high signal may represent an important feature for hepatic epithelioid hemangioendothelioma.

Resumo Objetivo: Determinar os achados comuns de hemangioendotelioma epitelioide hepático em imagens de ressonância magnética. Materiais e Métodos: Uma pesquisa foi feita em três bases de dados institucionais entre janeiro de 2000 e agosto de 2012. Sete pacientes (média de idade, 47 anos; variação, 21-66 anos; 6 mulheres) com diagnóstico confirmado por exame patológico de hemangioendotelioma epitelioide hepático submetidos a ressonância magnética foram identificados. Nenhum dos pacientes havia recebido tratamento para hemangioendotelioma epitelioide hepático antes do exame inicial por ressonância magnética. Resultados: Tumores de hemangioendothelioma epitelioide hepático apareceram como massas focais, maiores que 5 em número, em 7/7 pacientes, com uma lesão coalescente em 1/5 e localização periférica em 6/7 pacientes. Retração capsular esteve presente em 4/7 pacientes e foi associada com lesões perifericamente localizadas. Realce precoce em anel foi visto na maioria das lesões, em 7/7 pacientes. Realce progressivo centrípeto foi demonstrado em 5/7 pacientes na fase venosa, que exibia uma borda interna espessa distinta de baixo sinal nas imagens de fase venosa e um núcleo central de realce tardio. As lesões pequenas não mostraram isso. Conclusão: A combinação de lesões de configuração arredondada multifocais que são predominantemente periféricas e exibem realce precoce em anel periférico e aparecimento tardio de uma borda espessa interna de baixo sinal e um núcleo central de alto sinal pode representar uma característica importante para hemangioendotelioma epitelioide hepático.

Hemangioendotelioma epitelioide de pulmón / Pulmonary epitheloid hemangioendothelioma

El hemangioendotelioma epitelioide es un tumor vascular raro, de bajo grado de malignidad, con origen en las células endoteliales, que se puede desarrollar en cualquier tejido. En el pulmón surge habitualmente como secundario, pero también puede aparecer como primario, lo que es extremadamente inusual. Normalmente aparece como múltiples nódulos bilaterales entre jóvenes del sexo femenino. Raramente se desenvuelve como un nódulo pulmonar solitario. Los nódulos únicos y unilaterales pueden ser removidos quirúrgicamente. Se relata el caso de una paciente de 11 años que ingresó con impresión diagnóstica de neumonía lobular, pero que con una evolución desfavorable, necesitó realizar biopsia de la lesión, con el resultado de hemangioendotelioma epitelioide. Se discute también el diagnóstico y el tratamiento de esta entidad patológica.

Epitheloid hemangioendothelioma is a rare vascular tumor of low level of malignancy that emerges in the endothelial cells and may develop in any other tissue. It is generally secondary tumor in the lung but it may also appear as primary one, being very unusual. Generally speaking, it appears in the form of multiple bilateral nodules in young women. It rarely develops as a single pulmonary nodule. The single unilateral nodules may be surgically removed. This is the case of a 11-years old girl who was hospitalized with a diagnostic suspicion of lobular pneumonia, but her unfavorable progression required biopsy of the lesion with resulting epitheloid hemangioendothelioma. The diagnosis and the treatment for this pathological entity were also discussed.

Tumores hepáticos malignos no hepatocelulares. 2.a parte. Aspectos morfológicos y estudios auxiliares de importancia útiles para el diagnóstico histopatológico / Malignant Hepatic Neoplasms. Part 2. Morphology and important ancillary studies useful for histopathological diagnosis

Se continúa con la revisión de los tumores malignos hepáticos primarios. Se hablará de los aspectos más importantes de los tumores primarios no hepatocelulares, siendo más frecuentes los originados en el epitelio del ducto biliar como el colangiocarcinoma, el cistoadenocarcinoma y los tumores mesenquimales, y los más infrecuentes el hemangioendotelioma epitelioide y el angiosarcoma. La principal dificultad radica en lograr el diagnóstico definitivo, el cual se basa en la exclusión de una neoplasia primaria extrahepática o de lesiones hepáticas benignas. Estudios adicionales de inmunohistoquímica, estudios de clonalidad o moleculares pueden ser de mucha utilidad.

We continue with the review of primary malignant liver tumors. This article covers the most important aspects of primary tumors that are not hepatocellular. Those that originate in the epithelium of the bile duct such as cholangiocarcinoma, cystadenocarcinoma and mesenchymal tumors occur most frequently. Epithelioid hemangioendothelioma and angiosarcoma occur less frequently. The main difficulty lies in making a definitive diagnosis which must be based on the exclusion of extrahepatic primary neoplasms and benign liver lesions. Additional immunohistochemistry and molecular studies as well as diagnostic cloning of cells can be very useful.

Trasplante hepático en hemangioendotelioma epitelioide: reporte de dos casos y revisión de la literatura / Two Case Reports and a Literature Review of Liver Transplantation for Epithelioid Hemangioendothelioma

El hemangioendotelioma epitelioide hepático es un tumor raro, de naturaleza agresiva y comportamiento impredecible, con una incidencia de menos de 1 caso por millón de personas al año. Se presenta especialmente en mujeres jóvenes y con síntomas no específicos como dolor abdominal y pérdida de peso. La presentación radiológica es con múltiples nódulos o masas periféricas y bilaterales, con retracción de la cápsula, en algunos casos con calcificaciones y compromiso vascular. Debe realizarse biopsia hepática para confirmar el diagnóstico y para descartar diagnósticos alternativos como angiosarcoma, hepatocarcinoma, colangiocarcinoma y metástasis. El manejo depende de la extensión al momento de presentación, aunque solo el 10% de los casos es susceptible de resección. En las últimas décadas, el trasplante hepático se ha convertido en una opción de manejo, con series de casos aisladas reportadas por centros de trasplante en el mundo. En este artículo se presenta la experiencia de 2 pacientes con diagnóstico de hemangioendotelioma epitelioide hepático sin posibilidad de resección, confirmado histológicamente, sin compromiso extrahepático y que fueron llevados a trasplante de hígado exitoso y sin recurrencia de la enfermedad a 4 y 6 años después del trasplante.

Hepatic epithelioid hemangioendothelioma is a rare, aggressive and unpredictable tumor. Its incidence is less than one case per million people per year. Most commonly, it occurs in young women who exhibit nonspecific symptoms such as abdominal pain and weight loss. Radiologically it presents as multiple bilateral and peripheral nodules or masses with retraction of the capsule of the liver. In some cases there is calcification and vascular compromise. A liver biopsy should be performed to confirm the diagnosis and to rule out alternative diagnoses such as angiosarcoma, hepatocellular carcinoma, cholangiocarcinoma and metastasis. Management depends on the extension at presentation, but only 10% of cases are amenable to resection. Although in recent decades liver transplantation has become a management option, only studies of isolated cases reported by various transplant centers around the world have been published so far. In this article, we report our experience with two patients who were diagnosed with hepatic epithelioid hemangioendothelioma in which there were no possibilities of resection. Diagnoses were histologically confirmed, and since the patients had no extra-hepatic compromises, liver transplantations were performed. Both were successful and without recurrence at 4 and 6 years after transplantation.

Hemangioendotelioma hepático multifocal e metastático: relato de caso e revisão da literatura / Multifocal and metastatic hepatic hemangioendothelioma: case report and literature review

O hemangioendotelioma epitelioide é uma neoplasia rara de origem vascular, cujo acometimento de órgãos como o pulmão, o baço, o osso, a mama e o fígado já foi relatado. O quadro clínico e o prognóstico deste tumor são bastante variáveis. A partir de um relato de caso, procuramos contribuir com os dados existentes na literatura, revisando características clínicas e radiológicas que podem ser úteis para inseri-lo no diagnóstico diferencial das lesões hepáticas.

Epithelioid hemangioendothelioma is a rare neoplasm of vascular origin whose involvement of organs such as lung, spleen, bone, breast and liver has already been reported. The clinical signs and the prognosis of this tumor are quite variable. From the present case report, the authors have sought to contribute with literature data, by reviewing clinical and radiological features which may be useful to include such a condition in the differential diagnosis of liver lesions.

Hemangioendotelioma epitelioide hepático: un desafío diagnóstico para el médico radiólogo / Hepatic epithelioid hemangioendothelioma: a diagnostic challenge for the radiologist

Presentamos el caso de una paciente derivada a nuestra institución por presentar un cuadro clínico de dolor abdominal de más de un año de evolución, asociado al hallazgo de múltiples lesiones hepáticas sólidas, sugestivas de metástasis. Tras la biopsia percutánea, se diagnosticó hemangioendotelioma epitelioide hepático. Describimos las características clínico-imagenológicas de este tumor, aportando un nuevo caso de hemangioendotelioma epitelioide hepático, y analizamos los distintos hallazgos en los métodos de estudio por imágenes. Dado que se trata de una entidad poco frecuente en la que hay que pensar cuando se detectan lesiones hepáticas múltiples, es importante reconocerla e incluirla en el diagnóstico diferencial de las metástasis hepáticas.

We report a case of a patient referred to our institution presenting with 1 year of abdominal pain, associated to the finding of multiple solid liver lesions suggestive of metastases. Diagnosis of hepatic epithelioid hemangioendothelioma was performed after percutaneous liver biopsy.We describe clinical and imaging features of this tumor. Imaging findings are analized, providing a new case of hepatic epithelioid hemangioendothelioma. Since this is a rare entity that must be taken into account when multiple liver lesions are detected, it is important to recognize it and include it in the differential diagnosis of liver metastases.

Epithelioid hemangioendothelioma: 15 years at the National Cancer Institute. Literature review / Hemangioendotelioma epitelioide: 15 anos no Instituto Nacional de Câncer. Revisão da literatura

INTRODUCTION: Hemangioendotheliomas are locally aggressive vascular tumors with intermediate malignity and metastasis risk. The epithelioid variant, the most aggressive one, equally affects men and women at any age and it is rare in children. It occurs as a solitary tumor, which is usually painful, affecting superficial or deep soft tissues. Furthermore, it is less frequent in the liver, lung, bones, skin, lymph nodes and central nervous system. Microscopically, they present epithelioid cells with intracytoplasmic vacuoles, low mitotic activity and little or no necrosis. Additionally, its vascular nature is confirmed by immunohistochemical studies (CD31, CD34 and factor VIII). OBJECTIVE, MATERIAL AND METHODS: Through search in the archives of the Pathology Division of the National Cancer Institute (Instituto Nacional de Câncer [INCA]) from 1996 to 2011, 13 cases of epithelioid hemangioendothelioma (HEE) were identified and analyzed. RESULTS AND DISCUSSION: seven cases occurred in male patients and six in female patients, mean age 42 years, ranging from 7-66. The most common locations were: soft tissue (three patients; 23%); head and neck, mediastinum, bone and lung (two patients each; 15%); liver and lymph nodes (one patient each; 8%). There was clinical follow-up of nine patients: five were alive and disease-free (one to six years after diagnosis); three out of four patients with aggressive disease progressed to death (one month to five years after diagnosis); one relapsed two years after diagnosis and is alive with disease. CONCLUSION: This series of 13 cases of HEE, whose diagnoses were based on morphological and/or immunohistochemical analyses, demonstrates the different patterns of clinical presentation and biologic behavior of this disease.

INTRODUÇÃO: O hemangioendotelioma é um tumor vascular de malignidade intermediária, localmente agressivo e com risco de metástase. A variante epitelioide, a mais agressiva, acomete igualmente homens e mulheres em qualquer idade e é rara na infância. Apresenta-se como tumoração solitária, geralmente dolorosa, em partes moles, superficiais ou profundas; menos frequentemente, acomete fígado, pulmão, ossos, pele, linfonodos e sistema nervoso central. Microscopicamente, mostram células epitelioides com vacúolos intracitoplasmáticos, baixa atividade mitótica, pouca ou nenhuma necrose, tendo sua natureza vascular comprovada por estudos imuno-histoquímicos (CD31, CD34, Fator VIII). OBJETIVOS E MATERIAL E MÉTODOS: Por meio de busca nos arquivos da Divisão de Patologia (DIPAT) do Instituto Nacional de Câncer (INCA), de 1996 a 2011, foram encontrados e revisados 13 casos de hemangioendotelioma epitelioide (HEE). RESULTADOS E DISCUSSÃO: Sete casos ocorreram em homens e seis, em mulheres, com idade média de 42 anos, variando de 7 a 66 anos de idade. As localizações mais frequentes foram partes moles (três pacientes; 23%); cabeça-pescoço, mediastino, osso e pulmão, (dois pacientes cada; 15%); fígado e linfonodo (um paciente cada; 8%). Houve seguimento clínico de nove pacientes: cinco estavam vivos e livres de doença (de um a seis anos após o diagnóstico); dos quatro pacientes que apresentaram HEE agressiva, três evoluíram a óbito com doença (de um mês a cinco anos) e um recidivou dois anos após o diagnóstico e está vivo com a doença. CONCLUSÃO: Esta série de 13 casos de HEE, cujos diagnósticos foram firmados em bases morfológicas e/ou imuno-histoquímicas, demonstra os diferentes padrões de apresentação clínica e o comportamento biológico da doença.

Hemangioendotelioma de intestino delgado: causa poco frecuente de hemorragia digestiva alta / Hemangioendothelioma of the small intestine: infrequent cause of high digestive bleeding

El hemangioendotelioma es un tumor infrecuente, sobre todo a nivel intestinal, de naturaleza mesenquimal, de causa desconocida, clínica muy variable y pronóstico y comportamiento biológico muy diverso, es clasificada como una neoplasia de grado intermedio de malignidad, y se caracteriza por la presencia de células epitelioides. Objetivo: presentar el primer caso con esta afección reportado en el país. Caso clínico: se presenta el caso de una paciente de 68 años de edad que había presentado varios cuadros de hemorragia digestiva alta dados por melena y anemia, los estudios demostraron la presencia de una tumoración a nivel de intestino delgado, en el acto quirúrgico se constató la tumoración en el ángulo duodeno-yeyunal, la que se resecó e informó como un hemangioendotelioma epitelioide de intestino delgado. Conclusiones: el hemangioendotelioma epitelioide de intestino delgado es una causa de hemorragia digestiva alta que debe investigarse cuando no estén presentes las causas más frecuentes.

Hemangioendothelioma is an infrequent tumor, especially at intestinal level, mesenchymal nature, unknown cause, very variable clinical signs, highly diverse prognosis and biological behavior. It is classified as an intermediate degree of malignancy tumor, and is characterized by the presence of epithelioid cells. Objective: to present the first case with this affection reported in our country. Clinical case: a 68-year-old patient who had suffered several pictures of high digestive bleeding, given by melena and anemia, studies demonstrated the presence of a tumor at small intestine level, in the surgical act was found the tumor in the duodenojejunal flexure, which was resected and reported as an epithelioid hemangioendothelioma of small intestine. Conclusions: epithelioid hemangioendothelioma of small intestine is a cause of high digestive bleeding that should be investigated when the most frequent causes are not presen.

Hemangioendotelioma epitelioide hepático: reporte de caso / Hepatic epithelioid hemangioendothelioma: case report

El hemangioendotelioma epitelioide hepático es una neoplasia poco común, de origen vascular y comportamiento variable, y potencialmente maligno. Se presenta el caso de unamujer de 33 años, quien consultó por dolor en hipocondrio derecho y fiebre, y cuya impresióndiagnóstica extrainstitucional fue absceso hepático; posteriormente fue posible considerareste tumor dentro del diagnóstico diferencial, gracias a sus características imaginológicas,las cuales se confrontan en este reporte de caso con los hallazgos descritos en las mayores series publicadas en la literatura. El diagnóstico se confirmó mediante biopsia de la lesióny estudio histopatológico del hígado explantado. Se presentan, además, los hallazgos en Resonancia Magnética (RM) de otra paciente evaluada en la Unidad de Trasplantes de la Fundación Cardioinfantil y la evidencia acerca del tratamiento de esta entidad mediante trasplante hepático.

Hepatic epithelioid hemangioendothelioma is a rare neoplasm of vascular origin withmalignant potential. We present the case of a 33 year old woman with abdominal pain and fever, initially diagnosed as a hepatic abscess, who was admitted for further study. Imagingfeatures in ultrasound and computed tomography included a peripheral and multifocal liver lesion with capsular retraction, diagnosis was confirmed with a liver biopsy an a pathologicanalisis of the explanted liver. Magnetic Resonance findings from a second patient with a rapidly progressive disease are also reviewed and evidence regarding the surgical treatmentof this condition with liver transplantation are discussed.

Tumor del estroma gastrointestinal (TEGI): un estudio de su frecuencia y perfil de inmunohistoquímica / Gastrointestinal stromal tumors: analysis of 51 cases, immunohistochemical expression

Background: Gastrointestinal Stromal Tumors (GIST) have a mesenchymal origin and correspond to 1 percent of all gastrointestinal tumors. They have a benign behavior in approximately 75 percent of cases. They express CD117, CD34, smooth muscle actin, S-100 and desmin, markers that are useful in the differential diagnosis of smooth muscle tumors and those of neurogenic origin. Aim: To report our experience with GIST. Material and Methods: A retrospective, observational study. The pathology reports of GIST in the period 2004-2008 were reviewed. Immunohistochemical expression, pathological grade, mitotic index and histological patterns were reviewed. The medical records of patients were reviewed to obtain age and gender, location, size and presence of metastases. Results: A total of 51 GIST were identified, coming from 21 males and 30 females. Nineteen tumors were located in the small bowel, 18 in the stomach, four in the rectum, two in the colon and in five, the location was not specified. In 28 cases, the pathological pattern was spindle cell, in 12 mixed, in six epithelioid, in three pleomorphic, in one signet ring cell and giant cell in one. Forty nine percent of tumors were of high grade. Metastases were found in the liver in two cases, in the omentum in two and in the spleen, kidney, retroperitoneum and pancreas, in one case each. Two had lymph node involvement. Conclusions: GIST tumor corresponded to a 0.12 percent of all pathology reports during the study period. Most tumors in this series were of high grade.

Introducción: Los Tumores del Estroma Gastrointestinal (TEGI) son de origen mesenquimal comprendiendo el 1 por ciento de todos los tumores GI. Son benignos del 70 a 80 por ciento. Expresan CD117, CD34, actina de músculo liso, S-100 y desmina, marcadores útiles en el diagnóstico diferencial de tumores de músculo liso y tumores de origen neurogénico. Material y Método: Es un estudio retrospectivo y descriptivo. Se revisaron los reportes en el período 2004-2008 registrados como TEGI, valorando la expresión Inmunohistoquímica, grado histológico, índice mitótico, y patrones histológicos. Del reporte histológico se obtuvo la edad y sexo del paciente, localización, tamaño y metástasis. Resultados: Se recolectaron 51 casos corroborados como TEGI. Encontrando una prevalencia del sexo femenino (30) y una edad media de 52 años. Las localizaciones fueron: Intestino delgado (19), estómago (18), no especificado (5), recto (4), colon (2), retroperitoneo (2), no encontramos en esófago. Los patrones encontrados fueron el fusocelular (28), mixto (12), epitelioide (6), pleomórfico (3), células en anillo de sello (1), células gigantes (1). La mayoría (49 por ciento) fue de alto grado, presentando metástasis a hígado (2), ganglios (2), epiplón (2), bazo (1), riñón (1), retroperitoneo (1) y páncreas (1). Discusión: Se realizaron un total de 41.035 estudios histopatológicos, de los cuales 51 casos corresponden a LEGI, esto equivale al 0,12 por ciento. Encontramos tumores en los que su morfología, tamaño e índice mitótico fueron de bajo grado y presentaron metástasis y recidivas al momento del diagnóstico. Veinticinco casos fueron de alto grado (49 por ciento), lo cual es mayor a lo reportado por la literatura 20-30 por ciento, probablemente porque este es un hospital de concentración y generalmente los pacientes acuden a atención médica en una etapa avanzada de la enfermedad.

Melanoma de tipo animal / Animal-type melanoma

Lo que se ha dado en llamar melanoma de tipo animal es un tumor aún no bien determinado ni clasificado. Tiene similitudes clínicas e histopatológicas con el melanoma que se observa en los caballos de pelaje tordillo: un infiltrado alarmante, denso y extenso, de células que ocupan toda la dermis y aún el celular subcutáneo, acompañado sin embargo de un pronóstico benigno y larga sobrevida. El nombre propuesto por Zembowicz et ál., melanocitoma epitelioide pigmentado, parece más adecuado para esta rara variante de melanoma.

Animal-type melanoma is a rare distinct variant of melanoma, charac-terized by a dense proliferation of epithelioid and spindle-shaped mela-nocytes occupying the dermis and the hypodermis, and resembles theheavily pigmented melanomas as seen in grey horses. Only a limitednumber of cases have been reported and, as such, the clinical character-istics of this melanoma variant are incompletely understood. Despite thehigh mean thickness of the tumors, reports indicate a less aggressive behavior and a better outcome of this tumor when compared with conven-tional melanoma, but the underlying pathways related to this particularoutcome are still unknown. As proposed by Zembowicz et ál., the term‘pigmented epithelioid melanocitoma’ seems much more suitable.

Características radiológicas de los hemangioendoteliomas epitelioides de partes blandas, hígado y hueso / Radiologic features of epithelioid hemangioen-dothelioma of soft tissues, liver and bone

Objetivo: Contribuir al conocimiento de las características radiológicas de los hemangioen-doteliomas epitelioides (HEE) en partes blandas, hígado y hueso. Se obtuvieron las imágenes radiológicas de los pacientes con diagnóstico histológico de HEE en nuestra institución en los últimos veintitrés años. Revisamos los hallazgos en tomografía computarizada, resonancia magnética y ecografía: tamaño, número, comportamiento con y sin contraste intravenoso, signos de afectación local y a distancia. Correlacionamos los hallazgos de imágenes con los histológicos y revisamos la evolución clínica. Hallazgos: Los HEE de partes blandas aparecieron como masas sólidas sin hallazgos específicos. Los HEE hepáticos mostraron una localización característica periférica y con realce progresivo. Los HEE óseos aparecieron como lesiones líticas con masa de partes blandas adyacentes o edema. Dos HEE hepáticos y uno óseo fueron interpretados inicialmente como metástasis. Conclusión: El HEE puede crear problemas de diagnóstico diferencial, por lo que conociendo sus características radiológicas es posible sugerir el diagnóstico y tratamiento adecuados.

Objective: To contribute to the awareness of imaging features of epithelioid hemangioendothelioma (EHE) in soft tissues, liver and bone. Radiographic images were obtained from patients with histológica! diagnosis of EHE treated in our institution over the past twenty years. We reviewed the Computed Tomography, MRI, and ultrasound findings concerning size, number, behavior with and without intravenous contrast agent, signs of local involvement, and distant metastases. Imaging findings were correlated with histológica! results and clinical evolution was analyzed. Findings: Soft tissue EHE appeared as solid masses without specific features. Liver EHE showed a characteristic peripheral location and progressive enhancement. Osseous EHE behaved as lytic lesions with soft tissue mass or adjacent edema. Two liver EHE and one epithelioid hemangioendothelioma of bone were initially diagnosed as metastasis. Conclusion: The epithelioid hemangioendothelioma may pose problems in differential diagnosis; therefore, having awareness of its radiological features enables us to determine proper diagnosis and treatment.