Primary localized malignant biphasic mesothelioma of the liver in a patient with asbestosis

We report a case of primary localized malignant biphasic mesothelioma of the liver in a 66-year- old man associated with asbestosis. The tumor was detected as a hepatic nodule, 4 cm in diameter, in the right lobe （S8 segment） on CT scan. Histopathological examination demonstrated an intrahepatic tumor with central necrosis consisting of a papillary epithelioid pattern on the surface of the liver, microcystic （microglandular or adenomatoid） pattern mainly in the subcapsular area and sarcomatoid pattern intermingled with microcystic pattern in the major part of the hepatic nodular tumor. Tumor cells, especially of epithelioid type, showed distinct immunoreactivity for mesothelial markers （WT-1, calretinin, D2-40, CK5/6, mesothelin, thrombomodulin） and no immunoreactivity for epithelial （adenocarcinoma） markers （CEA, CD15, BerEP4, BG8, MOC31）. P53 immunoreactivity was detected focally in papillary epithelioid tumor cells and extensively in microcystic and sarcomatoid components, suggesting that the papillary epithelioid mesothelioma arose on the surface of the liver, and tumor cells showing microcystic and sarcomatoid patterns invaded and grew into the liver. To date, this is the first case of primary localized malignant biphasic mesothelioma of the liver, since all three primary hepatic mesotheliomas reported so far were epithelioid type.     

Hepatic Angiomyolipoma Mimicking Hepatocellular Carcinoma: Magnetic Resonance Imaging and Clinical Pathological Characteristics in 9 Cases

Hepatic angiomyolipoma (HAML) is a rare mesenchymal tumor of the liver with marked histological diversity. The present study was to review the magnetic resonance imaging (MRI) and clinical pathological features of HAML resembling hepatocellular carcinoma (HCC).Nine patients who underwent surgical resection and had pathological diagnosis of HAML were retrospectively analyzed.All of 9 patients (5 males and 4 females) had a solitary hepatic mass with a median size of 4 cm (from 1.4 cm to 15.3 cm). Seven cases were identified as incidental liver tumors during health screening and 2 patients were diagnosed for hepatic mass when visited hospitals with unspecific abdominal discomfort. Before resection, 6 cases were diagnosed as HCC on MRI. MRI on chemical shift imagings showed a large amount of lipids in 5 cases. The enhancement pattern of MRI was classified into 2 types: in 2 cases, lesions with small or no vessels that demonstrated prolonged enhancement (1 mixed subtype and 1 myomatous subtype) and in 7 cases, lesions with abundant central vessels that show rapid washout (3 mixed subtypes and 4 myomatous subtypes) in the portal venous/delayed phase. All patients underwent resection of hepatic tumor and no recurrence was observed during follow-up (range: 2–24 months) of median 10 months. By immunohistochemistry, the tumor cells demonstrated positive immunostaining for human melanoma black-45, smooth muscle actin, and CD34.In conclusion, all of 9 patients with HAML presented with none or nonspecific clinical manifestations. The diagnosis of HAML relies on disease and immunohistochemistry, but not MRI due to its resemblance to HCC.     

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 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.     

Chronic hepatitis C is a common associated with hepatic granulomas

AIM： To determine the most frequent etiologies of hepatic epithelioid granulomas, and whether there was an association with chronic hepatitis C virus （HCV）. METHODS： Both a retrospective review of the pathol- ogy database of liver biopsies at our institution from 1996 through 2006 as well as data from a prospective study of hepatic fibrosis markers and liver biopsies from 2003 to 2006 were reviewed to identify cases of hepatic epithelioid granulomas. Appropriate charts, liver biopsy slides, and laboratory data were reviewed to determine all possible associations. The diagnosis of HCV was based on a positive HCV RNA. RESULTS： There were 4578 liver biopsies and 36 （0.79%） had at least one epithelioid granuloma. HCV was the most common association. Fourteen patients had HCV, and in nine, there were no concurrent condi- tions known to be associated with hepatic granulomas. Prior interferon therapy and crystalloid substances from illicit intravenous injections did not account for the finding. There were hepatic epithelioid granulomas in 3 of 241 patients （1.24%） with known chronic HCV enrolled in the prospective study of hepatic fibrosis markers. CONCLUSION： Although uncommon, hepatic granu- Iomas may be part of the histological spectrum of chronic HCV. When epithelioid granulomas are found on the liver biopsy of someone with HCV, other clini- cally appropriate studies should be done, but if nothing else is found, the clinician can be comfortable with an HCV association.     

Improving recognition of hepatic perivascular epithelioid cell tumor:Case report and literature review

A 58-year-old man presented with the chief complaint of abdominal bloating and was incidentally found to have a liver tumor.As diagnostic imaging studies could not rule out malignancy,the patient underwent partial resection of segment 3 of the liver.The lesion pathologically showed eosinophilic proliferation,in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A,thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor(PEComa).A PEComa arising from the liver is relatively rare.Moreover,the name ‘PEComa' has not yet been widely recognized,and the same disease entity has been called epithelioid angiomyolipoma(EAML),further diminishing the recognition of PEComa.In addition,PEComa imaging findings mimic those of malignant liver tumors,and clinically,this tumor tends to enlarge.Therefore,a PEComa is difficult to diagnose.We conducted a systematic review of PEComa and EAML cases and discuss the results,including findings useful for differentiating perivascular epithelioid cell tumors from malignant liver tumors.     

High resolution CT findings of pulmonary epithelioid hemangioendothelioma: unusual manifestations in 2 cases

Pulmonary epithelioid hemangioendotheliomas (PEH), also known as intravascular sclerosing bronchoalveolar tumor, is a rare vascular tumor of the lung common among young women. Primitive lumena lined by single cells is the characteristic pathologic feature. The endothelial nature of these cells is confirmed by positive staining with factor VIII and CD34. PEH usually presents as single or multiple pulmonary nodules. The present report describes high resolution CT (HRCT) findings of 2 cases with unusual manifestations of PEH. One case was a 54-year-old woman with multiple pulmonary nodules with irregular thickening of both the bronchovascular bundles and perilobular structures, representing intensive lymphangitic spread on HRCT. The other was an 18-year-old woman who had multiple minute peripheral nodules in the lungs bilaterally. These HRCT findings demonstrated the presence of tumor nodules in the lymphatic spaces, which is quite an unusual histologic presentation for this tumor. Both cases also showed hepatic lesions on abdominal CT; the former showing hypoattenuating masses with coarse calcifications and the latter showing multiple tiny calcifications in the hepatic parenchyma. Recognition of these features in the appropriate clinical setting may allow the clinician and the pathologist to consider this rare tumor.     

Hepatic angiomyolipoma growing to cause epigastric discomfort: a case report

We report herein a case of hepatic angiomyolipoma growing to cause epigastric discomfort. A 33-year-old man was referred for excisional operation of hepatic hypervascular tumor, complaining of epigastric discomfort. The tumor had enlarged from the time 3 years before when the tumor was diagnosed as hepatic hemangioma in the lateral segment. Partial hepatectomy with tumor resection was carried out. Pathological diagnosis was confirmed as hepatic angiomyolipoma with a finding of enriched vessels, extramedullary hematopoiesis, fatty tissue, and most epithelioid cells immunoreactive to HMB-45. Further analysis also revealed positive staining cells for granulocyte colony-stimulating factor. It was suggested that growth of hepatic angiomyolipoma in this case might be caused by extramedullary hematopoiesis induced by granulocyte colony-stimulating factor.     

Incidental solitary hepatocellular carcinomas smaller than 1 cm in size found at autopsy: a morphologic study

The morphologic features and growth pattern of single hepatocellular carcinomas less than or equal to 1 cm in size, found incidentally at autopsy, were studied in nine cases. In all but one case, the hepatic parenchyma showed advanced cirrhosis. In three cases, the hepatocellular carcinomas were localized within a regenerative nodule as a form of "nodule within nodule." The carcinoma was rimmed by nonneoplastic hepatic tissue. A fourth carcinoma consisted of an expansile hepatocellular carcinoma nodule enclosed by a fibrous band of cirrhotic stroma. The remaining five cases consisted of hepatocellular carcinomas which infiltrated the surrounding regenerative nodules or hepatic lobules. These observations suggested that hepatocellular carcinomas arise within regenerative nodules, some of which still retain residual nonneoplastic tissue around the tumor. Others invaded the adjacent liver tissue. The grossly visible fibrous capsule, often seen in more advanced hepatocellular carcinomas, was absent in all cases. All of the hepatocellular carcinomas were well-differentiated. Four cases showed a trabecular pattern with slight sinusoidal dilatation, 3 showed a scirrhous pattern and 2 showed a compact pattern. Their histologic features included marked bile production, Mallory body formation by clusters of tumor cells, resistance to hemosiderin deposition in a markedly siderotic background and loss or decrease of reticulin fibers. These features were hallmarks of small hepatocellular carcinomas. Pathologists should study cirrhotic livers carefully so as not to miss small carcinomas. Clinicians should be aware that even small liver nodules may be hepatocellular carcinomas.     

Visceral bacillary epithelioid angiomatosis: possible manifestations of disseminated cat scratch disease in the immunocompromised host: a report of two cases

Opportunistic infection with the causative agent of cat scratch disease may be responsible for an unusual vascular proliferative lesion, referred to as bacillary epithelioid angiomatosis, previously described only in human immunodeficiency virus (HIV)-infected patients. We present a case of an HIV-infected patient with bacillary epithelioid angiomatosis involving the liver and bone marrow causing progressive hepatic failure. We also report a case of a cardiac transplant recipient with hepatic and splenic bacillary epithelioid angiomatosis manifesting as a fever of unknown origin, a previously unreported event in a non-HIV-infected patient. These cases represent the first documentation of bacillary epithelioid angiomatosis with visualization of cat scratch-like organisms involving internal organs.     

Giant gastrointestinal stromal tumour of rare sarcomatoid epithelioid subtype:Case study and literature review

Gastrointestinal stromal tumours(GISTs) are the most common mesenchymal tumours of the gastrointestinal tract,but they represent less than 3% of all gastrointestinal tract malignancies.This is a detailed case study of a 52-yearold male patient treated for very uncommon histological subtype of gastric GIST with atypical clinical presentation,asymptomatic progress and late diagnosis.The resected tumour,giant in diameters,was confirmed to represent the most rare histopathologic subtype of GISTs- sarcomatoid epithelioid GIST.We report this case and review the literature with a special focus on pathomorphological evaluation,biological aggressiveness and prognostic factors.To our knowledge this is the first report of giant GIST of very uncommon sarcomatoid epithelioid subtype.It is concluded that clinicians should pay attention to the fact that initial diagnosis may be delayed due to mildly asymptomatic and non-specific clinical presentation.Asymptomatic tumours diagnosed at a late stage,which is often the case,can be large on presentation.Prognosis for patients diagnosed with GIST depend on tumour size,mitotic rate,histopathologic subtype and tumour location.That is why early diagnosis and R0 resection,which is usually feasible and safe even in giant gastric sarcomatoid epithelioid subtype of GISTs,are the key factors for further treatment and good prognosis.     

L’atteinte hépatique au cours de la sarcoïdose

We report three cases of hepatic attack in sarcoidosis patients aged 43, 45, and 47years, respectively. The patients were two men and a woman. The hepatic attack appeared by pains to the right hypochondrium. Physical examination found an enlarged liver in all patients. The biology was characterized by anicteric cholestasis found in every case and hepatic cytolysis in two cases. The histological analysis of the hepatic biopsy showed epithelioid granulomas and gigantocellulars without necrosis caseum, evoking the sarcoidosis. The sarcoidosis was associated to a chronic hepatitis C virus to the stage of pre cirrhosis in a case (score of Metavir A1 and F2). All patients were put under corticoids. The outcome was favorable after about five months of treatment, but recurrence was observed after early dropout of treatment.     

Podoplanin expression in advanced-stage gastric carcinoma and prognostic value of lymphatic microvessel density

In gastric cancer, lymph node metastasis is a major prognostic factor. Tumor lymphangiogenesis promotes metastasis in experimental models, but in human tumors data about the presence and clinical significance of lymphatic vessels in the tumor area are controversial. We investigated 70 patients with advanced-stage gastric carcinoma and the pathological examination showed 40 cases with intestinal subtype and 30 cases with diffuse subtype. Forty three from 70 cases had regional lymph node metastasis. Additional slides were stained with an antibody against podoplanin, and lymphatic microvessel density (LMVD) was evaluated in the tumoral and peritumoral areas. Lymphatic vessels were identified in tumor area in all cases and LMVD was higher in the peritumoral than in the tumor area. Podoplanin-positive vessels in tumor area were usually small, with narrow lumen. A significant correlation was found between LMVD and stage of the tumor (p<0.002) and lymph node metastasis (p<0.031), but not with the pathological subtype and grade of the tumor. We found tumor cells in the lumen of lymphatic vessels in 11 cases, whereas tumor cells expressing podoplanin were found in 4 cases of less differentiated diffuse subtype gastric carcinoma. In conclusion, our results suggest that LMVD predicts tumor stage and lymph node metastasis, and podoplanin-positive tumor cells select a subgroup of tumors with high potential of invasion and metastasis. Key words: gastric cancer, podoplanin, lymphatic microvessel density (LMVD), lymphangiogenesis, prognosis.     

肝上皮样血管平滑肌脂肪瘤2例

目的:探讨肝上皮样血管平滑肌脂肪瘤的病理诊断及鉴别诊断.方法:收集武钢总医院病理科2007-2009年手术切除肝上皮样血管平滑肌脂肪瘤标本2例,对2例肝上皮样血管平滑肌脂肪瘤相关临床资料、病理学特征及免疫组织化学进行观察分析并复习相关文献.结果:2病例均为女性,巨检境界较清楚,镜下见肿瘤主要由弥漫的上皮样细胞构成,排列...     

Pleural metastases of sclerosing epithelioid fibrosarcoma

Sclerosing epithelioid fibrosarcoma is a rare tumor recently described. The histological presentation can be confused with certain soft tissue benign tumors and certain sarcomas. Metastatic spread is usually late in the natural course of the disease. We report a case of recurrent sclerosing epithelioid fibrosarcoma with pleural metastases which developed ten years after surgical resection of the primary tumor. The tumor was formed by small uniform round epithelioid cells with a clear cytoplasm. The tumor cells were strongly positive for vimentin. This clinical case is discussed in light of other cases reported in the literature.     

Hepatic perivascular epithelioid cell tumor in three patients

Perivascular epithelioid cell tumor (PEComa) is a rare, soft tissue tumor that can occur in various locations. The present report included three patients (one male and two females; age range, 25-51 years) with hepatic PEComas. The collected data included the clinical manifestations, diagnosis, management, treatment, and prognosis. Since it is dififcult to diagnose hepatic PEComas by imaging, the patients were diagnosed by tumor tissue examination such as immunohisto-chemistry, which was positive for HMB-45, Melan-A, and SMA on all slides. The tumor was composed of diverse tissues in-cluding smooth muscle, adipose tissue, and thick-walled blood vessels. During the follow-up period, one of the tumors was malignant (double-positive for CD34 and Ki-67) and recurred 3 months after surgery. In addition, malignant hepatic PECo-mas were reviewed in the literature, indicating that the major-ity of hepatic PEComas are benign, but few hepatic PEComas exhibit malignant behaviors in older female patients (>50 years of age) with abdominal discomfort and pain, larger tu-mor size (>10 cm), or positive staining for CD34 and Ki-67. In conclusion, there is no effective method to diagnose PEComas. Currently, the diagnosis of PEComas depends on immunohis-tochemical staining. Tumor resection and close follow-up are the principal methods for the management of PEComas.     

A case of resected squamous cell carcinoma of the lung complicated with sarcoidosis]

A 64-year-old man with uveitis was admitted to our hospital for detailed investigation of an abnormal shadow on his chest X-ray. Chest radiography and computed tomography of the chest showed mediastinal lymphadenopathy and a tumor shadow in the left hilum. Transbronchial tumor biopsy revealed squamous cell carcinoma. Left upper lobectomy and drainage of bilateral hilar and mediastinal lymph nodes were performed. Histopathological examination revealed the coexistence of squamous cell carcinoma with many non-caseating epithelioid cell granulomas in all hilar and mediastinal drainage lymph nodes, but no metastasis. Non-caseating epithelioid cell granulomas were also seen in the interstitium and alveolar spaces. Coexistence of sarcoidosis and lung cancer in the same patient is not common, and only 29 cases, including ours, have been reported. This case also provides the concept that surgical tumor resection should be considered even if bilateral mediastinal lymphadenopathy is found in a case of lung cancer complicated with sarcoidosis.     

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.     

The clinical features of hepatic angiosarcoma: a report of four cases and a review of the English literature.

Four cases of hepatic angiosarcoma are reported with a review of 99 other cases in the English literature. Angiosarcoma of the liver is associated with chronic exposure to thorotrast, vinyl chloride, arsenicals, radium and possibly copper and with chronic idiopathic hemochromatosis. Although 40% of patients have hepatic fibrosis or cirrhosis at autopsy, the nature of the association between chronic liver disease and hepatic angiosarcoma is unknown. The clinical presentation of hepatic angiosarcoma is nonspecific with abdominal pain, weakness and weight loss common complaints and with hepatomegaly, ascites and jaundice common findings. Liver function tests are usually abnormal but there is no one liver function test or set of tests specific for the tumor. The occurrence of thrombocytopenia and disseminated intravascular coagulation is characteristic of hepatic angiosarcoma and may be related to local consumption of clotting factors and formed blood elements in the tumor. Catastrophic intraabdominal bleeding is also characteristic and occurs in one-fourth of all cases. This complication is likely related to the high incidence of clotting abnormalities and the vascular nature of the neoplasm. Selective hepatic arteriogram and open liver biopsy are the foundations of diagnostic evaluation. Percutaneous liver biopsy should be avoided. Failure to appreciate the possibility of hepatic angiosarcoma in the proper clinical setting, leading to blind percutaneous biopsy, may result in failure to make the diagnosis at the cost of significant morbidity and mortality. Survival of patients with hepatic angiosarcoma is brief; only 3% live longer than 2 years. Treatment of the tumor to date is empirical. There are probably a few patients who might benefit from radical surgery with curative intent. For all others chemotherapy is indicated. Adriamycin is active against hepatic angiosarcoma, but optimal dose and mode of administration require further investigation. Further study is also required to delineate the cause of hepatic angiosarcoma in the 60% of cases without definite epidemiologic association.