MR imaging of malignant mesenchymal tumors of the liver.

Magnetic resonance (MR) features of five primary malignant mesenchymal neoplasms (plasmocytoma, leiomyosarcoma, undifferentiated sarcoma, epithelioid hemangioendothelioma, and angiosarcoma) of the liver were reported. All tumors were hypointense on T1-weighted images and hyperintense on T2-weighted images. No halo and intravenous extension were noted. A target appearance was revealed in epithelioid hemangioendothelioma. MR findings of angiosarcoma were essentially the same as those of cavernous hemangiomas (markedly hyperintense with hypointense linear septa on T2-weighted images). MR findings of these rare hepatic malignancies were nonspecific, although they were quite different from those of typical hepatocellular carcinomas. This study suggested that MR differentiation of primary hepatic mesenchymal tumors from other common benign and malignant neoplasms was difficult; however, the number of studied cases was limited.     

Pathology of primary tumors and pseudotumors of the pleura

Primary tumors are relatively rare in the pleura. Histological types include mesothelioma, epitheliod, biphasic or sarcomatoid tumors as well as primary lymphoma and mesenchymatous tumors which include solitary fibrous tumor, epithelioid hemangioendothelioma and angiosarcoma and synovialosarcoma. We detail here the new WHO classification 2004 explaining the different entities, excluding metastatic tumors which are the most frequent tumors of the pleura.     

Unusual presentation of hepatic vascular tumors as fulminant hepatic failure.

Epithelioid hemangioendothelioma and angiosarcoma of the liver are rare neoplasms of vascular origin. They can present with nonspecific symptoms such as malaise and weight loss, as well as with liver-related symptoms such as abdominal pain, tender hepatomegaly and jaundice. Portal hypertension and rarely liver failure can occur. We hereby report two cases of fulminant hepatic failure that were eventually diagnosed with epithelioid hemangioendothelioma and angiosarcoma of the liver.     

肺上皮样血管内皮瘤一例并文献复习

目的 提高对肺上皮样血管内皮瘤的认识。方法 结合1例肺上皮样血管内皮瘤患的临床资料和献复习，详细分析该病的病因、临床表现、诊断、鉴别诊断、治疗及预后等。结果 该病病因不清，临床症状较少且轻微，影像学检查主要表现为两肺多发性结节影；病理学检查主要特征是单细胞原始管腔结构；免疫组织化学提示内皮细胞源性。目前尚无有效治疗措施，患预后介于良、恶性肿瘤之间。结论 肺上皮样血管内皮瘤罕见，极易误诊，应予重视。     

746例肝脏和肝内胆管肿瘤临床发病特点分析

目的探讨肝脏和肝内胆管肿瘤的临床发病特点。方法收集2003年~2011年我科诊断的肝脏和肝内胆管肿瘤746例,分析其发病特点。结果肝脏肿瘤发病位于前九位的依次为肝细胞癌（43.4%）、血管瘤（37.3%）、肝内胆管细胞癌（5.8%）、转移性肿瘤（3.8%）、造血和淋巴样肿瘤（1.1%）、胆道乳头状瘤病（0.94%）、局灶性结节状增生（0.94%）、肝细胞不典型增生（0.67%）和不典型增生结节（0.54%）;本组未发现孤立性纤维肿瘤、淋巴管瘤病、假脂肪瘤、胚胎性肉瘤、Kaposi肉瘤、上皮样血管内皮细胞瘤、血管肉瘤、癌肉瘤、横纹肌肉瘤、畸胎瘤、卵黄囊瘤和横纹肌样瘤;肝细胞癌的发病年龄高峰在40~69岁,男性比女性高发,肝内胆管细胞癌高发年龄为50~69岁,男性与女性发病人数相当;血管瘤发病年龄高峰为30~49岁,女性比男性高发;转移性肿瘤发病高峰为40~59岁,男性与女性发病人数相当。结论肝脏肿瘤发病有逐年递增的趋势,来源于上皮的肝细胞癌是肝脏最常见的恶性肿瘤,来源于间叶的血管瘤是最常见的良性肿瘤。     

Hepatic neoplasms: computed tomography and magnetic resonance features

Over the last decade, major advances in computed tomography and magnetic resonance technology have occurred. These advances enable accurate, noninvasive detection and characterization of many hepatic neoplasms. This article illustrates the role of imaging in the evaluation of hepatic neoplasms and reviews the typical imaging features of both benign and malignant hepatic tumors. Benign tumors discussed include hemangiomas, focal nodular hyperplasia, hepatocellular adenoma, and simple cysts, as well as cysts associated with polycystic liver disease. Malignant neoplasms reviewed include metastases and conventional hepatocellular carcinoma as well as less common tumors such as fibrolamellar hepatocellular carcinoma, intrahepatic cholangiocarcinoma, angiosarcoma, and epithelioid hemangioendothelioma.     

肺上皮样血管内皮瘤(附1例报告及文献复习)

目的提高对肺上皮样血管内皮瘤的诊断和治疗。方法通过对1例肺上皮样血管内皮瘤患者的诊治,就本病的临床特点、诊断方法以及治疗和预后等情况进行全面的分析和讨论。结果肺上皮样血管内皮瘤是一种罕见的肺部肿瘤,病因不明,以干咳为主,影像学表现主要为双肺弥漫性小结节影,病理学检查特征是单细胞原始管腔结构、免疫组织化学提示内皮细胞源性,恶性程度并不低,疗效差。结论肺上皮样血管内皮瘤是一种罕见的肺部恶性肿瘤,临床无特异性,极易误诊,目前尚无确切的治疗方法,预后差,临床医生应该提高对其的认识程度。     

Epithelioid hemangioendothelioma of the liver

Epithelioid hemangioendothelioma (EH) is a rare tumor of vascular origin, which occurs at sites such as soft tissues, liver, or lung, and has a highly unpredictable malignant potential. It is an intermediate entity between well-differentiated hemangioma and angiosarcoma. We present two cases of this rare disease in which the tumor was detected fortuitously and the definitive diagnosis was based on histological evidence. Both our cases are highly illustrative of the two ways in which hepatic EH can present (nodular or diffuse) and of its diagnostic and therapeutic management. Neoplastic cells expressed the factor VIII-related antigen, CD31 or CD34. Treatment was surgical resection in one patient and liver transplant in the other. Although EH of the liver has a better prognosis than other hepatic neoplasms, conservative treatment is not recommended. Our cases highlight the importance of a histological diagnosis to avoid it being mistaken for another entity.     

Hepatic epithelioid hemangioendothelioma treated with orthotopic liver transplantation: a case report.

Hepatic epithelioid hemangiendothelioma is a rare vascular tumor. The clinical course is unpredictable and different treatment modalities are offered depending on the patients condition. Orthotopic liver tranplantation is the choice of treatment in diffuse cases without metastases. A 32 year old woman was admitted to hospital with multiple mass lesions diagnosed by ultrasonography of the liver. Physical examination was normal except for a painless hepatomegaly, and her biochemical tests were within the normal range. Computed tomographic scanning showed the presence of multiple lesions in both lobes, some of which were accompanied by a small degree of calcification. Although these findings were suggestive of hepatic epithelioid hemangioendothelioma, ultrasonographic guided fine needle aspiration biopsy failed to diagnose the exact nature of the lesions. The diagnosis of hepatic epithelioid hemangioendothelioma was confirmed by diagnostic laparotomy and immunohistochemical examination of the specimen by FVIII-RAg, CD34 and CD 31 markers. The patient was treated by orthotopic liver transplantation and had no evidence of tumor 18 months after transplantation. The problems in differential diagnosis and treatment options are discussed in this report of the first case of this rare tumor, treated by orthotopic liver transplantation in Turkey.     

肺上皮样血管内皮细胞瘤并双侧胸腔积液1例及文献复习

目的 加强对肺上皮样血管内皮细胞瘤的认识,提高诊断率。方法通过对1例肺上皮样血管内皮瘤患者的诊治和文献复习,详细分析该病的病因、临床表现、诊断、鉴别诊断、治疗及预后等。结果该病病因不清,临床症状较少且轻微,影像学检查主要表现为两肺多发性结节影,可累及胸膜并出现胸腔积液;病理学检查主要特征是单细胞原始管腔结构;免疫组织化学提示内皮细胞源性。目前尚无有效治疗措施,患者预后介于良、恶性肿瘤之间。结论肺上皮样血管内皮瘤并双侧胸腔积液罕见,极易误诊,当肺部疾病治疗效果不佳时应警惕其可能性。     

Malignant epithelioid hemangioendothelioma of the colon

Angiosarcoma of the colon with epithelioid and histiocytoid features, a malignant counterpart of epithelioid hemangioendothelioma, was observed in a 72-year-old man. The disease first manifested as a right cervical mass, with the histologic appearance of malignant, undifferentiated, large-cell epithelioid neoplasm. Light microscopy of the colonic tumor disclosed angiosarcoma, with active erythrophagocytosis and positive immunoperoxidase reactions to lysozyme, alpha-1-antitrypsin, and alpha-1-antichymotrypsin. Ultrastructural features of the tumor cells were those of intermediate between endothelial and histiocytic cells. The disease took a rapid fatal course with recurrence, peritoneal dissemination, and massive peritoneal hemorrhage. The cause remains unknown.     

Liver transplantation for benign liver tumors

Benign liver tumors are common lesions that are usually asymptomatic and are often found incidentally due to recent advances in imaging techniques and their widespread use. Although most of these tumors can be managed conservatively or treated by surgical resection, liver transplantation(LT) is the only treatment option in selected patients. LT is usually indicated in patients that present with life-threatening complications, when the lesions are diffuse in the hepatic parenchyma or when malignant transformation cannot be ruled out. However, due to the significant postoperative morbidity of the procedure, scarcity of available donor liver grafts, and the benign course of the disease, the indications for LT are still not standardized. Hepatic adenoma and adenomatosis, hepatic hemangioma, and hepatic epithelioid hemangioendothelioma are among the most common benign liver tumors treated by LT. This article reviews the role of LT in patients with benign liver tumors. The indications for LT and long-term outcomes of LT are presented.     

Vascular tumors simulating occlusive disease

Two cases of vascular tumors of large vessels with intraluminal growth simulating venous thrombosis and arterial occlusive disease are reported. One was a borderline malignant epithelioid hemangioendothelioma of the femoral vein and the other a malignant epithelioid angiosarcoma of the carotid artery. Immunohistochemical studies permitted to classify the tumors. Treatment consisted in surgical resection. No recurrence and no metastasis are noted at 24 months. Uncertainty regarding biological behaviour of vascular tumors and treatment persists.     

Malignant pancreatic tumor other than solid pseudopapillary tumor in pediatric patients: A single-center experience

Pancreatic tumors, except solid pseudopapillary tumors (SPTs), are rare in pediatric patients. Herein, we report various types of pancreatic tumors in pediatric patients and review the literature regarding their treatments and prognosis.We retrospectively reviewed the data of pediatric patients who underwent surgery for pancreatic tumors, excluding SPTs, between January 2009 and December 2019 at Seoul National University Children''s Hospital. A total of 35 pediatric patients were identified as having undergone surgery for pancreatic tumors. Of these patients, 30 were excluded because the tumor was identified as an SPT.The diagnoses of the five remaining (non-SPT) pancreatic tumors were pancreatic neuroendocrine tumor, mixed acinar neuroendocrine carcinoma, kaposiform hemangioendothelioma, and intraductal papillary mucinous neoplasm. All five patients survived; however, recurrence and liver metastasis were observed in one patient. The detailed demographics, treatments, and prognosis of each patient were reviewed.Despite the rarity and low incidence of pancreatic tumors in pediatric patients, four types of non-SPT tumors are reported here. Hence, the possibility of these should not be overlooked, especially since the diagnosis and adjuvant treatment differ vastly between the tumor types.     

A case of hepatic angiosarcoma surviving for more than 16 months after hepatic resection

Hepatic angiosarcoma is a non-epithelial malignant tumor and a rare primary neoplasm accounting for only a 0.1% of primary liver malignant tumors. Hepatic angiosarcoma progresses rapidly; therefore, most cases are discovered at an advanced stage, and less than 20% of the patients have received surgery. The lack of specific symptoms and radiological findings leads to the delay of diagnosis resulting in the poor prognosis. To the best of our knowledge, only three patients have been reported to survive for more than one year after hepatic resection for angiosarcoma. We herein report a patient with hepatic angiosarcoma, 4 cm in size, who underwent hepatic resection after confirming the diagnosis as angiosarcoma by the intraoperative frozen section examination. The patient is still alive for more than 16 months postoperatively without any signs of recurrence.     

Epithelioid hemangioendothelioma of the liver. Diagnostic features and role of liver transplantation

Five cases of epithelioid hemangioendothelioma of the liver are reported. This unusual type of vascular tumor is often difficult to diagnose; its angiogenic nature is not overt at the radiologic level and may be overlooked by conventional histologic examination. At imaging procedures, epithelioid hemangioendothelioma of the liver presents as multiple focal hypovascular areas, disseminated in both lobes. The combination of these imaging abnormalities with a peculiar set of demographic and clinical features, including young age, good general condition, and progressive course, is suggestive. However, the final diagnosis can be established only by histologic examination of appropriate material collected by guided liver biopsies and may be helped by immunohistochemistry and ultrastructural examination. The accurate diagnosis of epithelioid hemangioendothelioma of the liver is of clinical and therapeutic relevance. In view of its favorable clinical course, orthotopic liver transplantation may be considered for the treatment of this tumor when intrahepatic dissemination contraindicates partial hepatectomy and extrahepatic extension, excluded by exploratory laparotomy, is absent.     

Epithelioid hemangioendothelioma associated with nodular regenerative hyperplasia

Epithelioid hemangioendothelioma of the liver is a rare neoplasm of vascular origin. We report a case of epithelioid hemangioendothelioma occurring in a patient with nodular regenerative hyperplasia. This association suggests that some hepatic vascular changes might promote the growth of epithelioid hemangioendothelioma.     

肺上皮样血管内皮细胞瘤1例并文献复习

目的加强对肺上皮样血管内皮细胞瘤的认识,提高诊断率。方法结合广州呼吸疾病研究所2004-05-26收治的1例肺上皮样血管内皮细胞瘤患者的临床资料和文献复习,详细分析该病的病因、临床特点、病理诊断、鉴别诊断、治疗及预后等。结果该病病因不清,临床症状较少且轻微,多为咳嗽、咳痰、少量咯血;影像学检查主要表现为两肺多发性结节影;病理学检查主要特征是单细胞原始管腔结构;免疫组织化学提示内皮细胞源性。目前尚无有效治疗措施,预后介于良、恶性肿瘤之间。结论肺上皮样血管内皮细胞瘤罕见,极易误诊,应予重视。     

Clinical characteristics and outcomes of patients with hepatic angiomyolipoma: A literature review

First reported in 1976, hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumor occurring mostly in middle-aged women. Diagnosis of the liver mass is often incidental on abdominal imaging due to the frequent absence of specific symptoms. Nearly 10% of HAMLs are associated with tuberous sclerosis complex. HAML contains variable proportions of blood vessels, smooth muscle cells and adipose tissue, which renders radiological diagnosis hazardous. Cells express positivity for HMB-45 and actin, thus these tumors are integrated into the group of perivascular epithelioid cell tumors. Typically, a HAML appears on magnetic resonance imaging (or computed tomography scan) as a hypervascular solid tumor with fatty areas and with washout, and can easily be misdiagnosed as other liver tumors, particularly hepatocellular carcinoma. The therapeutic strategy is not clearly defined, but surgical resection is indicated for symptomatic patients, for tumors showing an aggressive pattern (i.e., changes in size on imaging or high proliferation activity and atypical epithelioid pattern on liver biopsy), for large (> 5 cm) biopsy-proven HAML, and if doubts remain on imaging or histology. Conservative management may be justified in other conditions, since most cases follow a benign clinical course. In summary, the correct diagnosis of HAML is challenging on imaging and relies mainly on pathological findings.     

Hepatic epithelioid hemangioendothelioma: An international multicenter study

Background and aimsHepatic epithelioid hemangioendothelioma is an ultra-rare hepatic vascular tumor, diagnosed more frequently in females. The knowledge about this tumor derives mainly from small case series with sub-optimal treatment outcomes. The aim of this study is to identify the clinical and radiological issues helpful to develop an international prospective registry.MethodsWe conducted an international multicentric and retrospective study of patients with hepatic hemangioendothelioma. The clinical, pathological and radiological images collected during follow-up were reviewed. Central radiological revision was performed and 3 patterns of contrast were defined.ResultsBetween 1994 and 2016, 27 patients with hepatic hemangioendothelioma were identified in three institutions but the final diagnosis was hepatic angiosarcoma in one. The majority were females, median age was 38.7-years and 17 patients were asymptomatic at diagnosis. No patient had Two out of ten (20%) patients had surgical specimens with positive macro-vascular invasion and 50% had extrahepatic disease, and the most frequent pattern was the progressive-central-contrast-uptake. After a median follow-up of 6.7-years, the 5- and 10-year survival rates are 91.5% and 51.9%, respectively.ConclusionsThis multicentric study shows the heterogeneous profile of patients with hepatic hemangioendothelioma, reflecting the need to establish a reference network in order to better characterize these patients and ultimately develop a personalized treatment strategy.