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.

Hemangioendotelioma epitelioide pulmonar: Reporte de un caso / Lung epithelioid hemangioendothelioma: Report of one case

Epithelioid hemangioendothelioma is a multifocal tumor that rarely metastasizes. It is difficult to diagnose, most often it is an incidental finding in young asymptomatic women. The radiologic pattern is heterogeneous. Histologic confirmation of Weibel-Palade bodies or immunohistochemistry based on specific tumor markers such as factor VIII and CD34 are the most important finding to confirm the diagnosis. We report a 21 years old woman Presenting with cough and dyspnea. A chest X ray was suggestive of tuberculosis. Sputum smears were negative for acid fat bacilli and the tuberculin test was negative. A chest CAT scan showed multiple nodular lesions. A surgical biopsy of the lesions confirmed the presence of a hemangioendothelioma. The patient was initially treated with prednisone and azathioprine without response. Thereafter, the patient is without treatment and without evidence of disease progression.

Multifocal epithelioid hemangioendothelioma presenting with hemoptysis.

We present a case of multifocal epithelioid hemangioendothelioma occurring in an adolescent boy who presented with massive hemoptysis and showed a pulmonary perihilar mass with multiple lesions in the liver and two lytic lesions in the ribs on imaging. The diagnosis was confirmed by lung and liver biopsy. He was treated with oral steroids.

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.

Epithelioid hemangioendothelioma / 대한간학회지

No abstract available.

A case of primary hepatic epithelioid hemangioendothelioma with spontaneous rupture / 대한간학회지

Primary hepatic epithelioid hemangioendothelioma is a rare neoplasm of endothelial origin. The clinical manifestations are nonspecific, ranging from complete absence of symptoms to hepatic failure and death. Spontaneous rupture of a hepatic epithelioid hemangioendothelioma is an extremely rare presentation. We present a case of primary hepatic epithelioid hemangioendothelioma in a 65-year-old male patient with alcoholic liver cirrhosis. He was hospitalized due to epigastric pain and multiple liver masses on abdominal ultrasound. Dynamic liver CT imaging revealed multiple peripheral nodular enhanced mass lesions with delayed centripetal enhancement, and the adjacent collection of high-attenuation fluid along the liver capsule. Abdominal tapping revealed blood in the peritoneal cavity. Primary hepatic epithelioid hemangioendothelioma with spontaneous rupture was finally diagnosed based on a histopathologic examination revealing positive immunohistochemical staining for CD34.

Hemangioendotelioma epitelioide de pleura: presentación de un caso con hemotórax / Epithelioid hemangioendothelioma of the pleura: presentation of a case with hemotorax

Los hemangioendoteliomas son tumores vasculares que pueden afectar pulmón. Abarcan desde lesiones benignas o de baja malignidad hasta lesiones de malignidad intermedia, como el hemangioendotelioma epitelioide o el polimorfo. El hemangioendotelioma epitelioide es un tumor muy raro que afecta principalmente a mujeres menores de 40 años, ha sido asociado al uso de anticonceptivos orales y a la inhalación de cloruro de vinilo. Sólo se describen en la literatura unos cincuenta casos de hemangioendotelioma epitelioide pulmonar y muy pocos pleurales. Inmunomarcadores permiten caracterizar este tipo de neoplasias. El diagnóstico diferencial incluye tumores benignos y malignos y el pronóstico es poco predecible. No hay consenso en cuanto al tratamiento, el cual ha incluído cirugía, carboplatino más etopósido e interferón. Se presenta una paciente de 37 años, se discuten los hallazgos clínicos su tratamiento y evolución.

The hemangioendotheliomas are vascular tumors that may involve lungs. The malignancy of these tumours can be benign, low or intermediate, such as the epithelioid or the polymorph hemangioendothelioma. The epithelioid hemangioendothelioma is an unusual tumour that appears more frequently in females, younger than 40 years of age. It has been associated to the use of oral contraceptives and the inhalation of vinile chloride. Only some 50 cases of pulmonary epithelioid hemangioendothelioma and few of pleural origin have been described in the literature. Immunomarkers may help to characterize this kind of tumours. The differential diagnosis includes malignant and benign tumours and its prognosis is hard to predict. Its therapy remains controversial, surgery, chemotherapy with carboplatin plus etoposide and interferon were used. The case of a 37 year old female is presented; clinical findings, therapy and outcome are discussed.

A case of hepatic epithelioid hemangioendothelioma that caused extrahepatic metastases without intrahepatic recurrence after hepatic resection / 대한간학회지

Epithelioid hemangioendothelioma is a neoplasm of vascular origin with a low-to-intermediate malignant potential and is one of the rare sarcomas arising from the liver. Its etiology is unknown and its clinical outcome is unpredictable. There is no generally accepted therapeutic strategy because of its rarity and the variable natural course between hemangioma and angiosarcoma. We report a case of a 64-year old woman who underwent hepatic resection due to epithelioid hemangioendothelioma in the right lobe that progressed to extrahepatic metastases of the bone, pleura, and peritoneum 22 months later. However, after resection there was no primary hepatic recurrence.

Pleural Epithelioid Hemangioendothelioma

Epithelioid hemangioendothelioma (EHE) is a rare tumor of vascular origin. While it can be found in any tissue, it is most often found in lung and liver and usually has an intermediate behavior. EHEs originating from pleural tissue have been less frequently described than those from other sites. Furthermore, to date, all of the cited pleural EHEs were described as highly aggressive. In the present report, we describe a rare case of pleural EHE extending to lung and bone in a 31-year-old woman. The histological diagnosis was confirmed by both conventional examination and immunohistochemistry. Her disease stabilized during the 4th course of adriamycin (45mg/m2, day 1-3), dacarbazine (300mg/m2, day 1-3) and ifosfamide (2,500mg/m2, day 1-3) with mesna, and she survived for 10 months after the diagnosis.

Epithelioid hemangioendothelioma of the liver / 대한간학회지

No abstract available.

Hemangioendotelioma epitelióide hepático: relato de caso com tratamento multimodal / Epithelioid hemangioendothelioma of the liver: report of a case with multimodal treatment

Os autores apresentam um caso raro de hemangioendotelioma epitelióide hepático multicêntrico de uma paciente oligossintomática, tratada inicialmente com ressecção e alcoolização. Na sua recidiva, foi submetida à radioablação. Revisando a literatura, os autores concluem que a ressecção permanece como o principal tratamento, associada ou não a métodos ablativos, e que o transplante tem indicação na doença multinodular bilateral.

A Case of Primary Hepatic Epithelioid Hemangioendothelioma Mimicking Metastatic Carcinoma / 대한소화기학회지

Epithelioid hemangioendothelioma is a rare vascular origin tumor which usually occurs in soft tissues, liver, and lung. It usually affects adult women and presents as multiple hepatic nodules with mainly peripheral distribution. It is difficult to diagnose and treat because of non-specific clinical manifestations and findings on the imaging study. Moreover, pathological misdiagnosis is common. We report a case of this rare tumor that was detected incidentally. Final diagnosis was based on histological evidence. A 52-years old man suffered from right upper quadrant abdominal pain for 3 months, and was initially misdiagnosed as a metastatic carcinoma. Physical examination revealed superior cervical lymphadenopathy with mild hepatomegaly. Finally, hepatic epithelioid hemangioendothelioma was diagnosed on the basis of positive immunohistochemical staining for factor VIII, CD34, and VEGF. Our case highlights the importance of a histological diagnosis to avoid misdiagnosis.

Hemangioendotelioma epiteloide / Ephitelioid haemangioendotheliom

El hemangioendotelioma epiteloide es una neoplasia poco común que ocurre en mujeres jóvenes, con menos de 50 casos descritos a nivel mundial. Describiremos el caso de un hombre de 54 años con historia de disnea, tos seca y dolor torácico, que consultó por historia de neumonía basal derecha un mes antes y dolor torácico derecho. En la radiografía de tórax se encontraron tractos fibrosos basales derechos y aumento de la arteria pulmonar derecha, diagnosticados inicialmente como tromboembolismo pulmonar no resuelto, evolución posterior con aparición de masa pulmonar que luego de múltiples estudios se diagnosticó como un hemangioendotelioma epiteloide metastásico originado posiblemente en la arteria pulmonar derecha. Describimos las radiografías de tórax, las tomografías, y las teorías actuales sobre el origen celular de esta neoplasia. Discutimos la apariencia citológica, presentación clínica y pronóstico de esta entidad. Este reporte puede contribuir para nueva información y hallazgos de esta poco común presentación de una neoplasia.

Hemangioendotelioma epitelióide de pleura / Epithelioid hemangioendothelioma of the pleura

Relata-se o caso de um paciente exposto profissionalmente a asbesto por dez anos e portador de um tumor pleural muito raro, o hemangioendotelioma epitelióde. O paciente apresentava derrame pleural serohemorrágico, sem evidência de células neoplásicas e com predomínio de linfócitos. A biópsia pleural por agulha revelou processo inflamatório crônico inespecífico, com áreas de tecido mixóide. A videotoracoscopia mostrou nódulos difusos nas pleuras parietal e visceral. A biópsia revelou neoplasia mesenquimal e eram semelhantes às áreas focais observadas na primeira biópsia. O estudo imunohistoquímico demonstrou a presença dos marcadores vasculares CD31, CD34 e Fator VIII, caracterizando a origem vascular do tumor. O paciente foi tratado com cisplatina e ectoposide, tendo o óbito ocorrido três meses após o diagnóstico.

Pulmonary Epithelioid Hemangioendothelioma: A Tumor Presented as a Single Cavitary Mass

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare tumor that occurs among young women and typically presents as bilateral multiple nodules. In the present report, we describe an uncommon case of PEH presented as a single cavitary nodule in a 33-yr-old asymptomatic man. This is the first case of PEH presented as a single cavitary nodule in the English literature. Three years of the follow-up without treatment was performed. Overall histologic findings were accord with conventional PEH, but some atypical features such as, increased mitotic activity (mean; two per ten high power fields), necrosis, spindling, and pleural and vascular invasion were recognized. Immunohistochemically, the tumor cells were positive for CD34. This report may contribute to the data on clinical findings and natural history of this rare tumor.

Intracranial epithelioid haemangioendothelioma

Epithelioid hemangioendothelioma is a true neoplasm of vascular origin of intermediate malignancy. It can occur in many sites but it is extremely rare within the cranium. Four cases with different ages and presentations have been operated upon and received a full dose of radiation. The clinical courses were discussed in details

Inmunología en la reproducción. Segunda parte / Immunology of reproduction. Second part

Se revisa la interacción entre los procesos reproductivos e nmunológicos. En la primera parte hicimos énfasis en el conocimiento de los aspectos generales del sistema inmune, especialmente de los factores humorales y celulares de la respuesta inmune, y revisamos la reactividad inmunológica durante el embarazo y el papel de la placenta en la interrelación. en esta segunda parte, completamos el último aspecto, repasando la interacción de los sistemas inmunológicos materno y fetal. Se continúa revisando los conocimientos sobre los anticuerpos antiespermatozoides y antiovario, la infertilidad masculina inmunológica, el aborto recurrente y las pérdidas fetales, y se explora los aspectos inmunológicos de la hipertensión inducida del embarazo, la isoinmunización Rh, la hidropesía fetal, las enfermedades del tejido conectivo vascular y embarazo, la purpura trombocitopénica autoinmune, el trasplante de órganos y las drogas inmunosupresoras y las infecciones durante el embarazo, la inmunidad y el parto pretérmino y se hace un alcance sobre la aplicación de la inmunología en la anticoncepción.