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.

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.

Hepatic epithelioid hemangioendothelioma—report of three cases of a puzzling tumor

Hepatic epithelioid hemangioendothelioma (EHE) is a rare malignant vascular neoplasm with unpredictable clinical behavior. These lesions are frequently misdiagnosed owing to its non-specific symptomatology, ambiguous radiological features, and overlapping histomorphology. We report three cases of hepatic EHE, of which one was male and two were female patients. While all three patients presented with abdominal pain, the male patient gave an additional history of weight loss and was jaundiced. The radioimaging showed multiple nodules in the liver and two of the patients also had pulmonary metastasis. The biopsies of the liver nodules revealed a tumor composed of spindle, epithelioid, and stellate tumor cells, some with characteristic intracytoplasmic vacuolations/lumina surrounded by myxohyaline stroma. Some of these intracytoplasmic vacuoles/lumina showed erythrocytes, suggesting its vascular origin which was confirmed by CD31 and CD34 positivity. The article highlights the importance of histopathology and IHC in the precise diagnosis of EHE.

A case of epithelioid hemangioendothelioma diagnosed by bone marrow cell morphology / 中华检验医学杂志

Epithelioid hemangioendothelioma (EHE) is a rare malignant vascular tumor. Its malignancy is between benign hemangioma and highly malignant angiosarcoma. It originates from vascular endothelial cells or pre-endothelial cells. It is characterized by the proliferation of vascular endothelial cells with a skin-like or histiocyte-like appearance. The incidence of EHE is less than 1% in all vascular tumors, and it can occur in multiple parts of the body, most often in the liver, followed by simultaneous involvement of the liver and lung, the lung alone, and the bone alone. At present, there is no report of epithelioid hemangioendothelioma diagnosed by bone marrow cell morphological examination in China. In this case, abnormal cells were found through bone marrow cell morphological examination, which guided the direction of further diagnosis and treatment. And finally the patient was diagnosed as epithelioid hemangioendothelioma. The bone marrow cell morphological examination can provided an important basis for clinical diagnosis and treatment. Epithelioid hemangioendothelioma needs to be differentiated from a variety of benign and malignant angiogenic tumors, especially other types of epithelioid angiogenic tumors. At present, it has been found that the disease has characters of cytogenetic and molecular biological abnormalities. Combined with histopathological morphology and immunohistochemical examination, we can make the diagnosis and differential diagnosis.

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.

Progress of liver transplantation in treating hepatic epithelioid hemangioendothelioma / 中华肝胆外科杂志

Epithelioid Hemangioendotheliomais a rare, low-grade malignant vascular tumour. It’scalled hepatic epithelioid hemangioendothelioma(HEHE), when it occurs in liver. It can be metastatic and postoperative recurrence. There are few cases have been reported in the literature at home and abroad because of its rarity. The treatment of HEHE is also controversial. With the continuous improvement of surgical techniques of liver transplantation, it is increasingly applied to treat liver failure patients caused by HEHE. Our paper reviews the literature on disease characteristics of HEHE, and liver transplantation for HEHE indications, immunotherapy and prognosis, to illustrate the status and progress of liver transplantation for HEHE.

Research Progress of Pulmonary Epithelioid Hemangioendothelioma / 中国肺癌杂志

Epithelioid hemangioendothelioma (EHE) is a rare vascular neoplasm that develops from vascular endothelial cells. It has been reported to occur many sites of body, but the most common EHE presentations are soft tissue (limbs), bone, liver and lung. Compared with other pulmonary tumors, pulmonary epithelioid hemangioendothelioma (P-EHE) is relatively rare. According to a literature review, more than 100 cases have been described all over the world. Due to the low incidence of P-EHE, lack of specificity in clinical symptoms and radiological findings, it is often misdiagnosed. Meanwhile, many patients do not receive appropriate treatment, resulting in poor prognosis in some cases. Histology and immunohistochemical methods are essential for diagnosis. However, there is no established standard treatment for P-EHE, because of the rarity of the disease. When the lesions are small and limited in number, surgical is the best treatment, achieving the purpose of diagnosis and treatment at the same time. This article tries to present the etiopathogenesis, clinical manifestations, diagnosis, treatment and prognosis of P-EHE.
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A Case of Epithelioid Hemangioendothelioma on the Choana

Epithelioid hemangioendothelioma is a rare vascular tumor with intermediate malignity and metastasis risk. It presents epithelioid cells with intracytoplasmic vacuoles and low mitotic activity. Its vascular nature can be confirmed by immunohistochemical studies (vimentin, CD31, CD34, and factor VIII). It is extremely rare in the nasal cavity, with only one case reported on the middle turbinate in Korea. The authors present a case of epithelioid hemangioendothelioma on the choana with a size of 2mm, which easily coult have been misdiagnosed as a blood clot.

MRI feature of hepatic epithelioid hemangioendothelioma / 中华肝胆外科杂志

Objective To discuss the MRI findings of hepatic epithelioid hemangioendothelioma (EHE).Methods MRI and clinical data of 8 EHE patients confirmed by pathology in PLA General Hospital were retrospectively analyzed.Results 8 patients included 3 male and 5 female.1 patient had single lesion and 7 patients had multiple lesions.A total of 162 lesions were detected and most of the lesions were in the peripheral liver.For T1WI,100 lesions (61.7％) showed slightly low signal,and 62 lesions (38.3％) showed lower signal intensity in the center of the lesion and appeared as "target sign".For T2WI,the center of 29 lesions (17.9％) showed two-loop "target sign",66 lesions (40.7％) showed three-loop "target sign",and 67 lesions (41.4％) showed slightly homogeneous higher signal.For DWI,116 lesions (71.6％) showed halo-like high signal and 46 lesions (28.4％) showed uniform high signal.For dynamic enhancement,the lesions showed slightly enhancement in the arterial phase,and persistent enhancement in portal venous phase,the center of 122 lesions (about 75.3％) showed enhancement and 40 lesions (24.7％) showed no enhancement in delayed phase.1 patient with hepatobiliary specific contrast was enhanced in hepatobiliary phase.“Hepatic capsule depression” was observed in 30 lesions.7 lesions appeared as “lollipop sign”,and were enveloped in 16 lesions.Conclusion MRI,DWI and dynamic contrastenhanced scanning of EHE in liver are characteristic,which is helpful for qualitative diagnosis before surgery.

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.

Diagnosis and treatment of hepatic epithelioid hemangioendothelioma / 中华肝胆外科杂志

Objective To study the clinical features and treatment results of hepatic epithelioid hemangioendothelioma (HEHE).Methods A retrospective study was conducted on 26 patients with HEHE who were diagnosed and treated at the Eastern Hepatobiliary Surgical Hospital in Shanghai from November 2003 to December 2015.Results The main clinical manifestations of these 26 patients included abdominal pain (13),dizziness (1),anorexia (1),the other 11 patients were asymptomatic.Ultrasound test revealed mixed echogenicity in 17 patients (17/25,68.0％).CT study showed hypointensity lesions in 10 patients (10/12,83.3％).MRI scans identified T1WI hypointense signals in 12 patients (12/14,85.7％) and T2WI hyperintense signals in 11 patients (11/14,78.6％).Platelet distribution width (PDW) assay indicated 18 patients ＜ 15.5 fl (18/22,81.8％),P ＜0.05.all the patients performed tumor makers test,including AFP,CEA,CA19-9.Slightly elevation of CA19-9 was merely identified in one patient.All the patients were confirmed CD34 positive through immunohistochemistry stains.The 1,3,5-year survival rates of these patients were 87.7％,57.0％,43.4％,respectively.Median survival was 61 months.Conclusions HEHE should be considered based on the suggestive imaging findings for the patients with negative tumor markers after exclusion of hemangioma.PDW assay may have a key role in HEHE diagnosis.

Comparative study of contrast enhanced ultrasound versus contrast enhanced MRI in hepatic epithelioid hemangioendothelioma / 中华肝胆外科杂志

Objective To analyze and compare the imaging characteristics of contrast-enhanced ultrasound (CEUS) and contrast enhanced magnetic resonance imaging (CEMRI) in diagnosing hepatic epithelioid hemangioendothelioma (HEHE) for improving radiological diagnostic accuracy.Methods The imaging characteristics of CEUS and dynamic CEMRI in 17 patients with histopathological diagnosis of HEHE on specimens obtained after surgical resection or fine needle biopsy were retrospectively analyzed and compared.Results On CEUS,20 lesions in 13 patients demonstrated different enhancement patterns in the arterial phase:13 lesions (65.0％) displayed gross enhancement and 7 lesions (35.0％) displayed a rimlike enhancement.Synchronous enhancement was observed in 17 lesions (85.0％) and rapid enhancement was observed in 3 lesions (15.0％).All these 20 lesions manifested as a hypoechoic mass in the portal venous and delayed phases.Other characteristics included speculated enhancement in the inner margin of the lesions and a vessel sign.On CEMRI,45 lesions (71.4％) manifested slight enhancement in the arterial phase and continuous enhancement in the portal venous and delayed phases.The remaining 18 lesions (28.6％) manifested no enhancement in the arterial phase but progressive enhancement in the portal venous and delayed phases.47 lesions (74.6％) displayed a rim-like enhancement and 16 lesions (25.4％) displayed gross enhancement.Other imaging characteristics included a halo sign,a lollipop sign,a capsular retraction sign or avessel sign.Conclusions On CEUS,characteristic manifestations of HEHE were synchronous enhancement with a quick wash-out.At peak time,the enhancement degree was equal to the normal liver parenchyma.On CEMRI,the main radiological feature of HEHE was a rim-like enhancement.In the arterial phase,the enhancement degree was not high and there was either a continuous enhancement or a progressive enhancement in the portal venous and delayed phases.Furthermore,HEHE lesions always occurred under the liver capsule and showed acapsular retraction sign,ahalo sign,a lollipop sign or a vessel sign.The combination of CEUS and CEMRI helped to improve the diagnostic rate and contributed to selection of clinical treatment.

Orthotopic liver transplantation for treatment of hepatic epithelioid hemangioendothelioma:report of two cases and literature review / 器官移植

Objective To investigate the clinical efficacy and therapeutic progress of orthotopic liver transplantation for the treatment of hepatic epithelioid hemangioendotheliom(a EHE). Methods Clinical data of 2 patients diagnosed with hepatic EHE were retrospectively analyzed. One patien(t case 1) was diagnosed with multiple hepatic EHE complicated with multiple infarction lesions of the spleen, and underwent orthotopic liver transplantation combined with splenectomy. The other cas(e case 2) was diagnosed with multiple hepatic EHE and received orthotopci liver transplantation alone. Literature review was performed. Pathological characteristics, clinical efficacy of liver transplantation and clinical prognosis of hepatic EHE patients were analyzed. Results Two patients successfully underwent surgery and were discharged postoperatively. The diagnosis of hepatic EHE was confirmed by pathological examinaiton and case 1 was complicated with EHE of the spleen . For case 1, tacrolimus was replaced by sirolimus at postoperavtie 1 month. At postoperative 4 months, capecitabine was orall y administered( chemotherapy) for EHE recurrence. At 6 months after surgery, the patient wa sdiagnosed with recurrent hepatic EHE complicated with multiple bone metastases, and waso rally administered with sorafenib. At postoperative 7 months, the patient died from cachexia and liver failure. Case 2 was followed up until the submission date( 8 months after surgery). No postoperative complications and tumor recurrence were observed. Previous studies had demonstrated that surgical resection was the primary therapy of hepatic EHE. Liver transplantation was highly recommended for patients with multiple unresectable hepatic EHE and extra-hepatic lesions. Moreover, chemotherapy, percutaneous puncture combined with transcatheter arterial chemoembolization and anti-angiogenesis treatment exerted certain clinical efficacy.C onclusions Surgical resection remains the primary therapy of hepatic EHE. For patients with multiple intrahepatic EHE, liver transplantation is considered as the optimal treatment. Much attention should be diverted to the prevention and treatment o f recurrent hepatic EHE following liver transplantation, aiming to improve the clinical efficacy.

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.

Pulmonary hemangioendothelioma with osteoclast‑like giant cells: A rare observation.

Pulmonary epithelioid hemangioendothelioma (PEH) is a rare vascular neoplasm, predominantly encountered in women, more often in the age group of 40 years and below. It is a tumor of borderline malignant potential with a clinical course intermediate between hemangioma and angiosarcoma. The tumor has variable prognosis, and treatment options include surgical excision in operable cases and chemotherapy in disseminated ones. The present report describes complete clinical, radiological, and histopathological features of PEH with osteoclast‑like giant cells and metaplastic ossification in a 20‑year‑old boy who presented with dyspnea and episodes of hemoptysis with review of literature.

A Case of Epithelioid Hemangioendothelioma of the Nasal Cavity / 대한이비인후과학회지

Epithelioid hemangioendothelioma (EHE), a rare vascular tumor that is both clinically and histologically an intermediate between angiosarcoma and hemangioma, was first described by Sharon Weiss and Franz Enzinger. It is characterized by proliferation of a distinct type of endothelial cells, which exhibit epithelioid morphology. It typically occurs in the 20-40 age range with no sex predilection, although the overall age range involved is much broader. This indolent tumor is potentially recurrent, but it rarely metastasizes. There are very few cases of EHE of nasal cavity described in the English literature. We describe here a case of EHE on the left middle turbinate of a 17-year-old male who presented with history of intermittent epistaxis.

Hepatic epithelioid hemangioendothelioma：imaging features and experience in clinical diagnosis and treatment / 中国癌症杂志

Background and purpose:Hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare, vascularly original tumor, and would be misdiagnosed easily in the clinical and imaging characteristics. This study aimed to investigate the imaging features of HEHE and our experience in clinical diagnosis and treatment, and to provide important reference for the diagnosis and treatment of HEHE in the future.Methods:This study retrospectively analyzed clinical manifestations, imaging features, pathological characteristics and treatment effects of 5 HEHE cases confirmed by pathology at Department of Integrative Cancer, Fudan University Shanghai Cancer Center.Results:The ratio of male to female patients was 2 to 3. HEHE predominantly occurred in middle-aged female patients whose ages range from 26 to 65 (mean=45.6). Imaging features of HEHE included multifocal hepatic disease (n=2), systemic multi-center multi-tissue occurrence (n=3). The ultrasound images showed isoechoic or hypoechoic lesions with no obvious blood lfow signal in the lesions. CT plain scan showed isointensity or hypointensity, while MR unenhanced with hypointense T1 signal and hyperintense T2 signal. The density or signal was uneven. Contrast-enhanced CT and MR images showed “slow in slow out”, obvious and variable degrees of peripheral rim enhancement. High FDG uptake showed delayed imaging characteristics (PET/CT). DSA angiography showed the tumor blood vessels were slim. After transcatheter arterial chemoembolization (TACE) surgery, lipiodol deposition within the lesion was not ideal. Under the microscope, tumor cells showed epithelial differentiation; angiogenesis was also visible. Immunohistochemistry staining showed CD31 and CD34 positive in all the 5 cases. Two cases treated with TACE combined with high intensity focused ultrasound (HIFU) and/or radiofrequency ablation (RFA) had good result.Conclusion:The clinical and radio-logical characteristics of HEHE are distinctive. Currently, for patients with systemic multi-organizational multi-center lesions, TACE combined with HIFU and/or RFA might be the most effective treatment method.

Farber Disease Misdiagnosed as Hemangioendothelioma / 소아외과

Farber disease (FD) is a rare lysosomal storage disorder that shows autosomal recessive inheritance. We report the case of a 58-month-old girl with FD, who was misdiagnosed with epithelioid hemangioendothelioma. The patient had undergone five surgeries for sacrococcygeal masses and three surgeries for scalp masses owing to misdiagnosis. Here, we describe this rare case of FD.

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.