Shiro Miyayama

IgG4-related lung and pleural disease: a clinicopathologic study of 21 cases.

Immunoglobulin G4 (IgG4)-related disorders can occur in the respiratory system. However, the clinicopathologic characteristics have not been well clarified. In this study, we examined clinical and pathologic features of, and follow-up data on, IgG4-related lung and pleural lesions. The patients group consisted of 17 males and 4 females with an average age of 69 years (range: 42 to 76). Pulmonary lesions in 16 patients and pleural lesions in 5 patients were examined. Histologically, all lesions showed diffuse lymphoplasmacytic infiltration. Irregular fibrosis and obliterative vascular changes were more common in solid areas. Nine cases (43%) had eosinophilic infiltration with more than 5 cells per high-power field. Immunostaining revealed numerous IgG4-positive plasma cells in inflamed areas. Sclerosing inflammation was distributed with intrapulmonary connective tissue. Pulmonary lesions showed a variety of morphologic changes according to the predominant area of inflammation. Serum IgG4 concentrations were elevated in 9 of 11 patients tested (average 6.9 g/L; range 0.3 to 18.0 g/L; normal range <1.35 g/L). Extra-pulmonary and extra-pleural IgG4-related lesions were identified in 9 patients (43%), and developed simultaneously or asynchronously during follow up. All patients treated with steroids responded, but some radiologic abnormalities remained in 3 patients. Interestingly, 1 patient was found to have a primary adenocarcinoma against a background of IgG4-related lung disease during follow up. In conclusion, IgG4-related diseases show a greater variety of pulmonary and pleural lesions than previously thought. It is important, therefore, to know the morphologic variety and clinicopathologic characteristics of this disorder.

JSH Consensus-Based Clinical Practice Guidelines for the Management of Hepatocellular Carcinoma: 2014 Update by the Liver Cancer Study Group of Japan

The Clinical Practice Guidelines for the Management of Hepatocellular Carcinoma proposed by the Japan Society of Hepatology was updated in June 2014 at a consensus meeting of the Liver Cancer Study Group of Japan. Three important items have been updated: the surveillance and diagnostic algorithm, the treatment algorithm, and the definition of transarterial chemoembolization (TACE) failure/refractoriness. The most important update to the diagnostic algorithm is the inclusion of gadolinium-ethoxybenzyl-diethylenetriamine pentaacetic acid-enhanced magnetic resonance imaging as a first line surveillance/diagnostic tool. Another significant update concerns removal of the term “lipiodol” from the definition of TACE failure/refractoriness.

Immunoglobulin G4–related Lung Disease: CT Findings with Pathologic Correlations

PURPOSE To retrospectively analyze radiologic findings of immunoglobulin G4 (IgG4)-related lung disease as correlated with pathologic specimens. MATERIALS AND METHODS This study was approved by the institutional review board, and all patients had consented to the use of their medical records for the purpose of research. This study included 13 patients with IgG4-related lung disease (nine men and four women; age range, 43-76 years). Computed tomographic (CT) findings were retrospectively analyzed with regard to the characteristics, shape, and distribution of the radiologic findings and were correlated with surgically resected or biopsy lung specimens in seven patients. Statistical analysis was not used in this study. RESULTS On the basis of the predominant radiologic abnormality, IgG4-related lung disease could be categorized into four major subtypes: solid nodular type having a solitary nodular lesion that included a mass (four patients); round-shaped ground-glass opacity (GGO) type characterized by multiple round-shaped GGOs (two patients); alveolar interstitial type showing honeycombing, bronchiectasis, and diffuse GGO (two patients); and bronchovascular type showing thickening of bronchovascular bundles and interlobular septa (five patients). Pathologically, solitary nodular lesions consisted of diffuse lymphoplasmacytic infiltration with fibrosis. Thickened bronchovascular bundles or interlobular septa and GGO on CT images pathologically corresponded to lymphoplasmacytic infiltration and fibrosis in peribronchiolar or interlobular interstitium and alveolar interstitium, respectively. The radiologic findings of honeycombing corresponded to disrupted alveolar structures and dilated peripleural air spaces. CONCLUSION IgG4-related lung disease manifested as four major categories of CT features. Pathologically, these features corresponded to IgG4-related sclerosing inflammation along the intrapulmonary connective tissue.

Biliary cystic tumors with bile duct communication: a cystic variant of intraductal papillary neoplasm of the bile duct.

Biliary cystic tumors, which are also called biliary cystadenoma and cystadenocarcinoma, are thought to be a heterogeneous disease entity, and some of them are known to show a luminal communication to the bile duct. In this study, we examined the clinicopathological features of nine cases of biliary cystic tumors with bile duct communication. They were composed of five males and four females with an average age of 67 years (52–84 years). They were multilocular (eight cases) or unilocular (one case), and all cases contained mucinous fluid. A direct luminal communication with the bile ducts was identified in five cases on preoperative or intraoperative cholangiographies. Biliary cystic tumors examined in this study were histologically adenoma (one case), adenocarcinoma in situ (six cases), and adenocarcinoma associated with microinvasive mucinous carcinoma (two cases). One case of adenocarcinoma in situ also had the adenoma component (adenocarcinoma in adenoma). Dysplastic mucinous epithelium proliferated in flat, micropapillary and papillary fashions within the intracystic spaces. Intraepithelial neoplasm was observed within non-dilated adjacent bile ducts, suggesting a direct luminal communication between the cystic tumors and the bile duct. Ovarian-like stroma was not observed in their walls in any cases. Immunohistochemically, seven cases expressed MUC1 or MUC2 in the neoplastic biliary epithelium. All cases except one were alive without any evidences of tumor recurrence after total excision (3–156 months after surgery). These clinicopathological features resembled those of intraductal papillary neoplasm of the bile duct, which had been reported as a biliary counterpart of pancreatic intraductal papillary mucinous neoplasm. In conclusion, biliary cystic tumors with bile duct communication could be regarded as intraductal papillary neoplasm with a prominent cystic dilatation of the bile duct and mucin retention, rather than true biliary cystic neoplasms.

Immunoglobulin G4–related Periaortitis and Periarteritis: CT Findings in 17 Patients

PURPOSE To retrospectively evaluate computed tomographic (CT) findings of immunoglobulin G4 (IgG4)-related disease involving the vascular system. MATERIALS AND METHODS This study was approved by the institutional review board, and all patients included had consented to the use of their medical records for the purpose of research. The study consisted of 17 patients (16 men and one woman; age range, 54-86 years). CT findings of IgG4-related periarterial lesions were retrospectively analyzed. Radiopathologic correlations were examined on the basis of surgically resected specimens. RESULTS A total of 22 periarterial lesions were detected in 17 patients. The lesions were located in the thoracic aorta (n = 4), abdominal aorta to iliac arteries (n = 13), superior mesenteric artery (n = 3), inferior mesenteric artery (n = 1), and splenic artery (n = 1). Radiologically, they were characterized by arterial wall thickening (mean thickness, 11 mm), relatively clear circumscription, possible association with luminal change (mostly dilated and rarely stenotic), exaggerated atherosclerotic change, and homogeneous enhancement at the late phase of contrast material-enhanced CT. Twelve patients (71%) had IgG4-related disease in other organs. Pathologically, diffuse lymphoplasmacytic infiltration, numerous IgG4-positive plasma cells, and irregular fibrosis were noted in the thickened arterial wall, especially at the adventitia. Steroid therapy administered to eight patients rapidly diminished the arterial wall thickening. One patient who did not receive steroid therapy showed spontaneous improvement at follow-up CT. CONCLUSION IgG4-related arterial lesions occur mainly in the aorta and its main branches and are radiologically characterized by homogeneous arterial wall thickening corresponding to pathologic features of IgG4-related sclerosing inflammation in the adventitia.

Extrahepatic Blood Supply to Hepatocellular Carcinoma: Angiographic Demonstration and Transcatheter Arterial Chemoembolization

PurposeTo evaluate the incidence of each extrahepatic collateral pathway to hepatocellular carcinoma (HCC) and to assess technical success rates and complications of transcatheter arterial chemoembolization (TACE) through each collateral.MethodsWe retrospective evaluated extrahepatic collateral pathways to HCC on angiography in 386 procedures on 181 consecutive patients. One hundred and seventy patients had previously undergone TACE. TACE through extrahepatic collaterals using iodized oil and gelatin sponge particles was performed when a catheter was advanced into the tumor-feeding branch to avoid nontarget embolization.ResultsA single collateral was revealed in 275 TACE procedures, two were revealed in 74, and three or more were revealed in 34. Incidences of collateral source to HCC were 83% from the right inferior phrenic artery (IPA), 24% from the cystic artery, 13% from the omental artery, 12% from the right renal capsular artery (RCA) and left IPA, 8% from the right internal mammary artery (IMA) and right intercostal artery (ICA), and 7% from the right inferior adrenal artery (IAA). Technical success rates of TACE were 53% in the right ICA, 70% in the cystic artery, 74% in the omental artery, 93% in the left IPA, 96% in the right IPA, and 100% in the right RCA, right IMA, and right IAA. Complications included skin necrosis after TACE through the right IMA (n = 1), cholecystitis after TACE through the cystic artery (n = 1), and ulcer formation after TACE through the right gastric artery (n = 1), in addition to pleural effusion and basal atelectasis after TACE through the IPA and IMA.ConclusionOur study suggests that TACE through extrahepatic collaterals is possible with high success rates, and is also relatively safe.

Identification of Small Hepatocellular Carcinoma and Tumor-feeding Branches with Cone-beam CT Guidance Technology during Transcatheter Arterial Chemoembolization

PURPOSE To evaluate the performance of transcatheter arterial chemoembolization guidance software that uses cone-beam computed tomography (CT) technology in identifying small hepatocellular carcinoma (HCC) tumors and feeding branches. MATERIALS AND METHODS Cone-beam CT and manual feeder vessel detection (MFD) software were used in chemoembolization of 68 HCCs 30 mm or smaller (mean ± standard deviation, 15.3 mm ± 5.2). Detectability of tumors and tumor-feeding sub-subsegmental arteries was compared versus that of nonselective digital subtraction angiography (DSA). Technical success of chemoembolization was divided into three grades according to 1-week CT findings: entire tumor embolized with at least a 5-mm-wide margin (ie, complete), tumor embolized without a margin in parts (ie, adequate), or entire tumor not embolized (ie, incomplete). All cone-beam CT data were also reanalyzed with automatic feeder vessel detection (AFD) software that was developed later. RESULTS Cone-beam CT could depict all tumors, including eight that were first discovered during chemoembolization. Sixty-one tumors (89.7%) were detected on CT during arterial portography and during hepatic arteriography, and seven (10.3%) were detected with one or the other. Nonselective DSA depicted 49 tumors (72.1%). Among 100 tumor-feeding vessels, 81 were identified with MFD and 38 with nonselective DSA. Detectability of tumors with CT and tumor-feeding branches with MFD was significantly better than with nonselective DSA (both P<.001). Fifty-nine tumors (86.8%) were completely embolized and nine (13.2%) were adequately embolized. AFD identified 96 feeder vessels; 88 (88%) represented true-positive findings. CONCLUSIONS Transcatheter arterial chemoembolization guidance software with cone-beam CT technology has a sufficient performance level to detect small HCCs and their feeding branches.

Focal hepatic fatty infiltration in the posterior edge of the medial segment associated with aberrant gastric venous drainage: CT, US, and MR findings.

OBJECTIVE The purpose of this study is to demonstrate the relation between focal hepatic fatty infiltration and aberrant gastric venous drainage (AGVD) in the posterior edge of the medial segment (PEMS) of the liver. MATERIALS AND METHODS We present two cases of focal hepatic fatty infiltration with AGVD in the PEMS and discuss their imaging features. RESULTS In both cases the focal fatty infiltration areas were hyperechoic on sonography, hypodense on CT, and hyperintense on T1-weighted MRI. Computed tomography during arterial portography (CTAP) showed nodular perfusion defects corresponding to the areas in both cases, and early enhancement of the area was observed with dynamic MRI in one case. Although the findings on CTAP and dynamic MRI suggested a neoplastic nature for the lesions, focal fatty infiltration was confirmed with surgical resection in one case and with imaging follow-up in the other. Aberrant gastric venous drainage into the area was demonstrated on arteriography in both cases. CONCLUSION The variation in blood supply caused by AGVD may play an important role in fatty metabolism in the PEMS of the liver and may influence imaging features.

Covered Gianturco Stents for Malignant Biliary Obstruction: Preliminary Clinical Evaluation

PURPOSE To evaluate the effectiveness of covered Gianturco stents in patients with malignant biliary obstruction. MATERIALS AND METHODS Three types of partially polyurethane-covered stents were implanted in 19 patients with malignant biliary obstruction located distal to the hilar confluence. A transhepatic approach was employed in all but one patient, in whom the stent was placed through a T-tube tract. RESULTS Stent placement was possible in all patients. In 15 patients, the implanted stents were expanded to a mean of 81% of their original diameter. In the remaining four patients, the stents expanded to less than 40% of the original diameter, and balloon dilation and additional bare stent placement were required. All patients except one, who had impairment of liver function due to multiple liver metastases, showed relief of jaundice after stent placement. At follow-up, which ranged from 5 to 57 weeks (mean, 24.7 weeks), one stent (5%) was occluded after 26 weeks due to tumor growth above the upper stent edge, and required secondary intervention. Complications in three patients included stent migration in one (5%) and cholangitis in two (11%). CONCLUSION Preliminary results suggest that placement of covered Gianturco stents is feasible, the complication rate is acceptable, and short-term patency appears promising.

Malignant Esophageal Stricture and Fistula: Palliative Treatment with Polyurethane-covered Gianturco Stent

PURPOSE To evaluate the effectiveness of a polyurethane-covered Gianturco stent in the palliative treatment of malignant esophageal stricture and fistula. PATIENTS AND METHODS Twenty-seven patients with recurrent stricture (n = 24), fistula formation (n = 8), or both (n = 5) underwent palliative treatment for aphagia (n = 15) or dysphagia (n = 12). Eight patients had fistulas to the respiratory tract or mediastinum. A 15-F delivery sheath system was passed through the stricture; the inner dilator was removed, and the stent was compressed into the sheath and advanced with a pusher catheter. Follow-up included chest radiography for 3 days and monthly esophagography or endoscopy. RESULTS Covered stents occluded fistulas and opened strictures in 100% of patients. Food intake was upgraded to liquids in 7% of patients, to soft foods in 37% and to regular foods in 56%. New stricture or fistula occurred in 4% and 7% of patients, respectively. Stent migration occurred in 15% of patients. Twenty-one patients died after stent placement, and average life expectancy was 11 weeks. CONCLUSION A polyurethane-covered Gianturco stent is effective in the palliation of advanced malignant esophageal strictures and fistulas.