Multiple hemangiomas (hepatic small vessel neoplasia) in the liver with Budd-Chiari syndrome.

Hepatic small vessel neoplasia (HSVN) is a recently recognized hemangioma of the liver with uncertain malignant potential. Almost all the patients are asymptomatic. Budd-Chiari syndrome (BCS) is a rare disorder characterized by noncardiogenic hepatic venous outflow obstruction. Benign hepatocellular nodules have been acknowledged for a long time in the liver with the chronic BCS. However, there has been no case report of BCS associated with HSVN. The patient was diagnosed with BCS 13 years ago. The imaging test initially displayed multiple hepatic nodules that were suspected of benign hepatocellular nodules. They gradually increased in size and number in the course of the disease. At an autopsy, these nodules were confirmed to be multifocal HSVN. The tumor of the present case could not be proved to have GNAQ and GNQ14 mutations. We describe the case focusing on the chronological imaging changes and discuss on the relationship between BCS and HSVN.

Synchronous Occurrence of Advanced Gastric Carcinoma with Retroperitoneal Liposarcoma: A Case Report.

BACKGROUND Gastric carcinoma (GC) remains one of the most common and deadly neoplasms in the world. Liposarcoma (LPS) is the most common sarcoma of adults. However, synchronous or metachronous occurrence of GC with LPS seems to be very rare. Tumor staging and differential diagnosis with these cases are extremely difficult. CASE REPORT The patient was a man in his 70s, who reported anorexia and weight loss of 4 kg over 2 months. Gastroscopy demonstrated a large tumor of Borrmann type 3, of which histology was moderately to poorly differentiated adenocarcinoma. The clinical stage was initially defined as IVb due to a 11×6 cm retroperitoneal (RP) tumor. Despite chemotherapy for GC, the RP tumor rapidly enlarged. Endoscopic ultrasound-guided fine-needle aspiration biopsy showed that it was an undifferentiated sarcoma. He died of hepatorenal failure secondary to severe jaundice. The autopsy revealed a synchronous occurrence of GC and RP sarcoma. GC had no areas admixed with sarcoma. Histology of RP sarcoma showed that it mainly consisted of undifferentiated sarcoma and focally of well-differentiated LPS characterized by well-differentiated adipocytes admixed with scattered atypical stromal cells. The tumor cells in both areas were positive for MDM2 and CDK4 by immunohistochemistry. The diagnosis of the RP sarcoma was revised to dedifferentiated LPS. CONCLUSIONS There were no previous case reports of synchronous occurrence of GC with LPS in the English and Japanese literature. GC and LPS pose challenging problems in their diagnoses, staging, and treatments when they occur synchronously or metachronously.

An Autopsy Case of an Elderly Patient with Classic Hodgkin Lymphoma Presenting with a Plethora of Clinical Symptoms and Signs.

BACKGROUND Hodgkin lymphoma (HL) is a potentially curable disease with favorable outcomes. However, elderly patients with HL usually have more adverse prognostic factors and hence a much worse prognosis than younger patients. CASE REPORT The patient was a woman in her 80s. She reported high fever, anorexia, and a weight loss of 8 kg within 5 months. She had been on treatment for diabetes mellitus and hypertension. She had undergone percutaneous coronary intervention and pacemaker implantation to treat acute coronary syndrome and sinus arrhythmia, respectively. Blood tests showed elevation of alkaline phosphatase, C-reactive protein, leukocyte count, CA 19-9, and carcinoembryonic antigen. Computed tomography did not show tumors in the liver, and cholangitis and sepsis were suspected. Aspartate transaminase, alanine aminotransferase, and total bilirubin gradually increased through the course of the patient's hospital stay. Despite treatment, her condition deteriorated and she died 22 days after hospital admission. At autopsy, we found stage IV HL with lymph node swelling on both sides of the diaphragm, as well as diffusely disseminated nodules in the liver and spleen. CONCLUSIONS Our patient had several poor prognostic factors including B symptoms, comorbidity, advanced stage, Epstein-Barr virus infection, and expression of programmed death-ligand 1 and interleukin-6, all of which were closely connected with her advanced age. Her age and comorbidities may have been the most adverse prognostic factors for her illness. An effective HL screening method for elderly individuals should be developed to ameliorate poor prognosis and adverse outcomes.

An Autopsy Case of Pulmonary Capillary Hemangiomatosis with an Electron Microscopy Study.

BACKGROUND Pulmonary capillary hemangiomatosis (PCH) and pulmonary veno-occlusive disease (PVOD) are rare diseases that share clinical, X-ray, and histological features. Most patients have poor prognosis due to severe respiratory impairment. Recently, EIF2AK4 mutations were found in some patients with PCH and PVOD, but the role of this mutation is still unknown. We report an autopsy case of PCH and discuss a mechanism of respiratory dysfunction based on an electron microscopy study. CASE REPORT The patient was a Japanese man in his sixties. He suffered from acute exacerbation of dyspnea during treatment of COPD. Respiratory function testing revealed DLCO' 32.1% and DLCO'/VA 23.6%. Echocardiography demonstrated findings consistent with pulmonary hypertension. A CT scan showed mild emphysema and small ground-glass opacity in the lungs. However, we could not find the exact cause of his respiratory failure and he died 28 days after admission. At autopsy, the histology showed multilayering capillary proliferation within the alveolar walls. Electron microscopy examination revealed prominent widening of the air-blood barrier, scarce fusion of the epithelial and capillary basement membranes, and frequent narrowing of the capillary lumen. CONCLUSIONS We reported an autopsy case with PCH with no histological findings of PVOD. Whether PCH and PVOD are 2 different histological patterns of the same disease remains to be verified. The changes in the air-blood barrier detected by electron microscopy may explain the respiratory impairment and pulmonary arterial hypertension.

An Autopsy Case of Mesenteric Panniculitis with Massive Pleural Effusions.

BACKGROUND Mesenteric panniculitis (MP) is an idiopathic chronic inflammatory condition of the mesentery. The main symptoms include abdominal pain, abdominal distention, weight loss, fever, nausea, and vomiting. The patients also present with chylous ascites in 14% of the cases and chylous pleural effusion (CPE) in very rare occasions. Despite the previous view of excellent prognosis of MP, two recent papers reported several fatal cases. However, there are still only a few autopsy case reports that describe the macroscopic and histological details of MP cases. CASE REPORT The patient was an 81-year-old Japanese woman. She complained of edema of her lower legs and face, general fatigue, and dyspnea. She was overweight and had type 2 diabetes (T2D). Computerized tomography (CT) demonstrated massive bilateral pleural effusions, with mild pericardial effusion and mild ascites. There was no pulmonary, cardiac or hepatic condition to explain the effusions. However, MP was suspected based on her CT. She gradually deteriorated into respiratory failure. The autopsy revealed CPEs (left 1,300 mL, right 1,400 mL) and MP in the mesentery of the small intestine. Neither neoplasia nor inflammatory conditions other than MP were detected. CONCLUSIONS In rare occasions, patients with MP present with CPE or chylothorax. We thought that a possible mechanism of the CPEs was a diaphragmatic defect. We suspected that being overweight and T2D had an etiological relationship with MP in our patient's case. Adipose tissue of the mesentery is the main focus of MP. We believed that MP would be the best umbrella term of the many synonyms.

Comparison of clinical and pathological features of lung lesions of systemic IgG4-related disease and idiopathic multicentric Castleman's disease.

AIMS: The lung lesion [immunoglobulin (Ig)G4-L] of IgG4-related disease (IgG4-RD) is a condition that occurs together with IgG4-RD and often mimics the lung lesion [idiopathic multicentric Castleman's disease (iMCD-L)] of idiopathic multicentric Castleman's disease (iMCD). Because no clinical and pathological studies had previously compared features of these diseases, we undertook this comparison with clinical and histological data. METHODS AND RESULTS: Nine patients had IgG4-L (high levels of serum IgG4 and of IgG4+ cells in lung specimens; typical extrapulmonary manifestations). Fifteen patients had iMCD-L (polyclonal hyperimmunoglobulinaemia, elevated serum interleukin-6 levels and polylymphadenopathy with typical lymphadenopathic lesions). Mean values for age, serum haemoglobin levels and IgG4/IgG ratios were higher in the IgG4-L group and C-reactive protein levels were higher in the iMCD-L group. All IgG4-RD lung lesions showed myxomatous granulation-like fibrosis (active fibrosis), with infiltration of lymphoplasmacytes and scattered eosinophils within the perilymphatic stromal area, such as interlobular septa and pleura with obstructive vasculitis. All 15 lung lesions of iMCD, however, had marked accumulation of polyclonal lymphoplasmacytes in lesions with lymphoid follicles and dense fibrosis, mainly in the alveolar area adjacent to interlobular septa and pleura without obstructive vasculitis. CONCLUSIONS: Although both lesions had lymphoplasmacytic infiltration, lung lesions of IgG4-RD were characterized by active fibrosis with eosinophilic infiltration within the perilymphatic stromal area with obstructive vasculitis, whereas lung lesions of iMCD had lymphoplasmacyte proliferating lesions mainly in the alveolar area adjacent to the perilymphatic stromal area. These clinicopathological features may help to differentiate the two diseases.

A case of primary angiosarcoma of the lung presenting as a hemorrhagic bronchial tumor.

Pulmonary angiosarcomas are usually secondary tumors, and only a few primary cases have been reported. Effective strategies for treating this tumor have not been established, and the prognosis of affected individuals is generally very poor. We report a case of primary angiosarcoma presenting as a hemorrhagic solitary nodule at the bifurcation of the left main bronchus, followed for two years before surgery. Bronchial arteriography revealed a tumor stain sign, and racemose hemangioma of the bronchial artery was excluded. The hemoptysis was not controlled by repeated bronchial artery embolization, and the patient underwent left pneumonectomy with routine mediastinal lymph node dissection. Histopathologically, the excised tissue revealed a highly-cellular growth of atypical spindle cells with a storiform pattern. These atypical cells showed relatively low mitotic activity; the MIB-1 index was 10%. The tumor was diagnosed as a primary angiosarcoma of the lung by the following immunohistological findings: positivity for factor VIII-related antigen and CD31. One year after resection, the patient remains well without signs of recurrence.

Radiographic and CT features of radiation-induced organizing pneumonia syndrome after breast-conserving therapy.

PURPOSE: To investigate radiographic and computed tomography features of radiation-induced organizing pneumonia syndrome after breast-conserving surgery. MATERIALS AND METHODS: The appearances and distribution of lung parenchymal abnormalities were retrospectively analyzed on chest radiographs (n = 12) and computed tomography scan images (n = 10) of 12 women (range 37-78 years, mean 55.8 years) with radiation-induced organizing pneumonia syndrome after breast-conserving surgery. RESULTS: The principal radiographic feature was an airspace filling pattern in all patients that involved the middle and lower lung zones in 10 of the 12 patients. Multi-focal lesions manifesting airspace consolidation surrounded by ground-glass opacities were the predominant CT finding in all 10 of these patients. The main lesion was predominantly located in the lung periphery in nine patients and contiguously extended to the central portion of the lung in seven patients. Frequent ancillary findings were airway dilation within the consolidation and lobar volume loss in nine and eight patients, respectively. All had solitary (6/10) or multifocal (4/10) distant lung opacities, mostly consistent with the finding of ground-glass opacities (9/10). Migration of the lung disease was observed in ten patients on subsequent radiographs. CONCLUSION: The cardinal radiologic feature of this syndrome is airspace consolidation surrounded by ground-glass opacification with airway dilation and volume loss, involving primarily the irradiated, subpleural area, along with distant opacities.

[High-grade fetal adenocarcinoma of the lung; report of a case].

82-year-old man was admitted with an abnormal shadow on the chest roentgenogram. Computed tomography showed a 2.8 x 2.4 cm solid tumor in S3 of the left lung. Transbronchial lung biopsy revealed adenocarcinoma and a left upper lobectomy (ND2a-1) was performed. The tumor consisted mainly of tall columnar clear cells, and no morules were found. Immunohistochemically, the tumor was positive for alpha-fetoprotein (AFP) and p53. Accordingly, we made the histological diagnosis of high-grade fetal adenocarcinoma of the lung, pT2N0M0, stage IB. The patient was not received adjuvant therapy and has been doing well without any tumor recurrence for 3 months postoperatively.

[A case of Mycobacterium avium pleuritis and pneumothorax in a rheumatoid arthritis patient treated with a TNF-alpha antagonist].

A 70-year-old woman with rheumatoid arthritis received treatment with corticosteroids and methotrexate for 4 years, followed by an additional TNF-alpha antagonist (infliximab) for about 3 years. She presented with a several-week history of persistent cough, and CT images of the lung showed a thin-walled cavitary lesion abutting the pleural surface of the left upper lobe. While we investigated the cause of this lesion, we admitted her because of acute chest pain. Chest radiography demonstrated moderate left-sided pneumothorax with pleural effusion. After further investigation, we suspected that her pneumothorax and pleuritis had been caused by a ruptured cavitary lesion arising from a Mycobacterium avium infection. Despite multi-drug therapy, chest tube drainage and surgical pulmorrhaphy her pleural complications were intractable. This is a rare case of pneumothorax and pleuritis caused by Mycobacterium avium infection induced by a TNF-alpha antagonist. Physicians should be aware of nontuberculous mycobacterial infections in patients treated with TNF-alpha antagonists.

Pulmonary arterial thrombosis in eisenmenger syndrome is associated with biventricular dysfunction and decreased pulmonary flow velocity.

OBJECTIVES: This study sought to determine what factors are associated with pulmonary artery thrombi in Eisenmenger patients. BACKGROUND: Pulmonary artery thrombosis is common in Eisenmenger syndrome, although its underlying pathophysiology is poorly understood. METHODS: Adult patients with Eisenmenger syndrome underwent computed tomography pulmonary angiography, cardiac magnetic resonance imaging, and echocardiography. Measurement of ventricular function, pulmonary artery size, and pulmonary artery blood flow were obtained. Hypercoagulability screening and platelet function assays were performed. RESULTS: Of 55 consecutive patients, 11 (20%) had a detectable thrombus. These patients were older (p = 0.032), but did not differ in oxygen saturation, hemoglobin, or hematocrit from those without thrombus. Right ventricular ejection fraction by magnetic resonance imaging was lower in those with thrombus (0.41 +/- 0.15 vs. 0.53 +/- 0.13, p = 0.017), as was left ventricular ejection fraction (0.48 +/- 0.12 vs. 0.60 +/- 0.09, p = 0.002), a finding corroborated by tissue Doppler and increased brain natriuretic peptide. Those with thrombus also had a larger main pulmonary artery diameter (48 +/- 14 mm vs. 38 +/- 9 mm, p = 0.007) and a lower peak systolic velocity in the pulmonary artery (p = 0.003). There were no differences in clotting factors, platelet function, or bronchial arteries between groups. Logistic regression showed pulmonary artery velocity to be independently associated with thrombosis. CONCLUSIONS: Pulmonary arterial thrombosis among adults with Eisenmenger syndrome is common and relates to older age, biventricular dysfunction, and slow pulmonary artery blood flow rather than degree of cyanosis or coagulation abnormalities. Further work to define treatment efficacy is needed.

Interlobular septal thickening in idiopathic bronchiectasis: a thin-section CT study of 94 patients.

PURPOSE: To retrospectively establish the prevalence of interlobular septal thickening at thin-section computed tomography (CT) in patients with bronchiectasis and to retrospectively examine the relationship between septal thickening and various CT features of bronchiectasis and functional parameters. MATERIALS AND METHODS: Institutional review board approval or informed consent are not required for such a retrospective study. Thin-section CT scans of 94 patients (29 men, 65 women; age range, 18-82 years) with idiopathic bronchiectasis confirmed at thin-section CT and without any history or condition that causes bronchiectasis were assessed independently by two observers. The presence and signs of bronchiectasis, extent and distribution of septal thickening at global and lobar levels, and presence of emphysema were recorded. Univariate and multiple regression analyses were used to identify relationships between individual parameters of bronchiectasis, the extent of septal thickening, and functional indices. RESULTS: Interlobular septal thickening was present in 56 (60%) of 94 patients with idiopathic bronchiectasis, excluding those with trivial septal thickening (34 of 94, 36%). At a lobar level, 69% (514 of 748) of lobes with bronchiectasis had septal thickening. There was strong correlation between the extent of septal thickening and the extent (r = 0.61, P < .001) and severity (r = 0.54, P < .001) of bronchiectasis. Stepwise regression analysis showed a relationship between the extent of septal thickening and the extent of bronchiectasis (P < .001). Septal thickening was not linked to functional indices of obstruction or restriction. CONCLUSION: At thin-section CT, interlobular septal thickening is a frequent finding in patients with idiopathic bronchiectasis.

Organizing pneumonia: perilobular pattern at thin-section CT.

PURPOSE: To describe the appearance and frequency of a perilobular pattern at thin-section computed tomography (CT) in patients with organizing pneumonia. MATERIALS AND METHODS: Thin-section CT scans of 21 consecutive patients with cryptogenic organizing pneumonia were retrospectively reviewed. Two thoracic radiologists in consensus recorded the presence and distribution of the CT abnormalities (consolidation, ground-glass opacification, nodules, bandlike opacities, interlobular septal thickening, and findings of fibrosis), with a particular focus on the presence and predominant location of the perilobular pattern, that is, a poorly defined arcadelike or polygonal appearance. RESULTS: The perilobular pattern was present in 12 (57%) of 21 patients, 10 of whom had five or more perilobular opacities. Other CT features were consolidation (20 patients, 95%), which was predominantly a subpleural and/or peribronchial distribution in 17 patients, and ground-glass opacification (18 patients, 86%). Bandlike opacities and interlobular septal thickening were observed in four patients and one patient, respectively. The perilobular pattern abutted the pleural surface in 10 of 12 patients and was surrounded by aerated lung parenchyma in 11 of 12 patients. There was no obvious relationship between perilobular opacities and CT findings indicative of established fibrosis. CONCLUSION: A perilobular pattern was present in more than half of the patients, along with the expected thin-section CT features of organizing pneumonia.