Diagnostic value of contrast-enhanced ultrasound in hepatic epithelioid hemangioendothelioma / 中华肝脏病杂志

Objective: To investigate the features of contrast-enhanced ultrasound (CEUS) in hepatic epithelioid hemangioendothelioma (HEHE) in order to improve the preoperative diagnosis rate. Methods: CEUS images of 32 pathologically-proven cases of hepatic epithelioid hemangioendothelioma from January 2004 to August 2021 were collected. Lesions were analyzed to observe the features of enhancement mode, enhancement intensity, and distinct enhancement phases. Results: Among the 32 cases, one had a solitary lesion, 29 had multiple lesions, and two had diffuse-type lesions. Contrast-enhanced ultrasound revealed a total of 42 lesions in 32 cases. In terms of arterial phase enhancement, 18 lesions had overall enhancement, six lesions had uneven dendritic enhancement, 16 lesions had rim-like enhancement, and two lesions had just slight peripheral spot enhancement around the lesions. Among the three cases, there were multiple lesions that had overall enhancement and ring enhancement. In terms of the enhancement phase, 20 lesions showed "fast progression", 20 lesions showed "same progression", and two lesions showed "slow progression". During the late arterial or early portal venous phases with rapid washout, all lesions manifested as hypoechoic. With peaked enhanced intensity, 11 lesions had a lower enhancement intensity than the surrounding normal liver parenchyma; 11 lesions had the same enhancement degree as the surrounding normal liver parenchyma; and 20 lesions had a higher enhancement degree than the surrounding normal liver parenchyma. All 16 ring-enhancing lesions had marked hyperenhancement. In the typical enhancing lesions, four showed hyperenhancement, five showed low enhancement, and nine showed isoenhancement. In the dendrite-enhancing lesions, there were two isoenhancing and four hypoenhancing. Contrast-enhanced ultrasound delineated the boundaries of all lesions more clearly than two-dimensional ultrasound. Conclusion: Contrast-enhanced ultrasound has certain value in the diagnosis of hepatic epithelioid hemangioendothelioma.

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.

A case of pseudomyogenic hemangioendothelioma misdiagnosed as low-grade malignant fibrous histiocytoma and review of literature / 中南大学学报(医学版)

Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.

Hemangioendotelioma hepático infantil: presentación de un caso clínico / Infantile hepatic hemangioendothelioma: a clinical case

El hemangioendotelioma hepático del lactante es una patología rara y más aún que su forma de presentación tenga complicaciones graves y el paciente fallezca. Se presenta el caso clínico de una lactante menor de 3 meses de edad que ingresó al Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga con diagnóstico de intoxicación herbácea (hierbabuena) e íleo metabólico. La hepatomegalia fue el signo más llamativo en la exploración clínica asociado a valores elevados de alfafetoproteína sérica. Los hallazgos ecosonográficos y tomográficos reportaron múltiples lesiones redondeadas que impresionaron metástasis hepáticas. La paciente fallece a los 23 días de iniciada la enfermedad actual siendo el diagnóstico postmortem hemangioendotelioma cavernoso gigante hepático, Se concluye que en general el diagnóstico de esta enfermedad se puede plantear sin una confirmación histológica, particularmente en lactantes menores, cuando los hallazgos clínicos, analíticos e imagenológicos son muy sugestivos aun cuando hay excepciones como lo presentado en este caso clínico(AU)

Infantile hemangioendothelioma is a rare, usually benign, disease. We present a case of a 3-month-old infant who was admitted in the Servicio Desconcentrado Hospital Pediátrico Dr. Agustín Zubillaga with the diagnosis of herbaceous poisoning (peppermint) and metabolic ileus. Hepatomegaly was the most characteristic sign in clinical examination and was associated with elevated levels of alpha-fetoprotein. Echographic and tomographic findings revealed multiple rounded lesions that impressed liver metastases. The patient died 23 days after admission and the postmortem diagnosis showed giant hepatic cavernous hemangioendothelioma. Diagnosis of hemangioendothelioma usually can be done without histopathological confirmation, especially in infants, when clinical, laboratory and imaging features are very suggestive of this disease, however there are exceptions as the one presented in this case(AU)

Epithelioid Hemangioma Involving Three Contiguous Bones: a Case Report with a Review of the Literature

An epithelioid hemangioma involving three contiguous bones in continuity has, to the best of our knowledge, not been reported in the literature. A case of a 48-year-old man presented with radiating pain to the lower thoracic region for two years. A radiograph and CT scan revealed both permeative osteolytic and multiple trabeculated lesions involving the left posterior part of the 10th rib as well as the 9th and 10th vertebral bodies in continuity and was misled as a malignant or infectious lesion. The histopathology and immuno-histochemistry of the lesion confirmed the diagnosis of an epithelioid hemangioma. The lesion was still stable as of three years after surgery.

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.

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.

Epithelioid sarcoma: a diagnostic challenge.

Epithelioid sarcoma is an uncommon slow-growing soft tissue malignancy, associated with a high incidence of local recurrence and metastasis. We report a 26-year-old male with epithelioid sarcoma on the right palm with a long history of over seven years, which was initially misdiagnosed as cutaneous tuberculosis and epithelioid hemangioendothelioma, as a result of which the treatment was delayed. No metastasis was found in our patient. The patient was referred to the oncology centre where he underwent wide excision of the lesion followed by radiotherapy. The review of the literature including clinical and histological differential diagnosis is presented as it mimics inflammatory, benign tumors as well as other malignant conditions.

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.

Epithelioid and retiform hemangioendothelioma of the skull bone--report of four cases.

Hemangioendothelioma (HE) is a borderline or intermediate type of vascular neoplasm. We report clinical and histopathological characteristics of four cases of HE arising from the skull bones because of its extreme rarity in this location. The age of the patients ranged from 6-45 years. Three patients presented with a painless swelling over the head and one case had sphenoid wing mass with dimness of vision and proptosis. Radiographic images showed a well-demarcated, osteolytic lesion in the skull bone in all the four, one case in addition had sclerotic edges and another had specks of calcification. Grossly, the tumour was very vascular with hemorrhagic areas. Histologically, three cases showed features of an epithelioid variant of HE, with short strands and solid nests of rounded to slightly spindled, eosinophilic endothelial cells, some of them having small intracellular vacuoles. The stroma was myxoid--hyalinised with focal mixed inflammatory infiltrate. One case had features of a 'retiform' histological variant composed of numerous elongated vessels lined by a single layer of hobnail endothelial cells, focal lymphocytic infiltrate and papillae with hyaline collagenous cores. The tumour cells in all the four were immuno-labelled by antibody to factor VIII-associated protein. The tumour cells lacked cytological atypia and mitosis was sparse. These features were important in prognostication as low-grade tumours can be cured by complete wide-resection.

Epithelioid haemangioendothelioma of the rib--a case report.

Epithelioid haemangioendothelioma is a rare primary malignant tumour of the bone that accounts for less than 1% of all primary bone malignancies. The case discussed here is of a 35 years old male who presented with gradually increasing left infrascapular mass attached to 10th rib. X-ray showed an expansile lytic lesion in 10th rib. On FNA the diagnOsis of fibrous dysplasia or fibrous-histiocytic lesions was suggested. The lesion was excised along with adjacent rib. Histopathological examination showed features of epithelioid haemangioendothelioma. Immunohistochemistry revealed focal factor VIII related antigen positivity.

Epithelioid haemangioma (benign haemangioendothelioma of bone)--a case report.

A case of epithelioid Haemangioma of the femur is presented. The patient, a 29 year old woman, was treated by curettage and bone grafting, following which two "recurrent" tumours appeared. These were successfully removed by repeat curettage.