[Pulmonary Carcinosarcoma of Right Middle Lobe Extending to the Left Main Bronchus].

A 72-year-old man with a lung tumor admitted to our hospital for surgical treatment. Computed tomography (CT) revealed a mass in the right middle lobe extending to the truncus intermedius and the left main bronchus. The enucleation of the tumor in the left main bronchus was performed with a bronchoscope followed by right middle and lower lobectomy. From the bronchial stump, the residual tumor was observed in the bronchus and was able to be pulled out since there was no invasion of the tumor into the bronchial wall. Pathological diagnosis was pulmonary carcinosarcoma.

An inflammatory myofibroblastic tumor exhibiting immunoreactivity to KIT: a case report focusing on a diagnostic pitfall.

Inflammatory myofibroblastic tumors (IMTs) and gastrointestinal stromal tumors (GISTs) are both spindle cell tumors, and occur rarely in the wall of the urinary bladder. In general, immunostaining allows differentiation of IMTs and GISTs. Most IMTs are positive for anaplastic lymphoma kinase (ALK) and negative for KIT, whereas most GISTs are ALK-negative and KIT-positive. Here, we describe a case of a spindle cell tumor in the wall of the urinary bladder. The spindle cells were positive for both ALK and KIT, and it was thus difficult to determine whether the tumor was an IMT or a GIST. We eventually diagnosed an IMT, because ALK gene rearrangement was confirmed by fluorescent in-situ hybridization. Cytoplasmic staining for KIT and the absence of other GIST markers, including DOG1 and platelet-derived growth factor α, indicated that the tumor was not a GIST. Therefore, IMTs should be included in the differential diagnosis of spindle cell tumors, even those that are KIT-positive.

[Three autopsy cases of chronic necrotizing pulmonary aspergillosis].

Chronic necrotizing pulmonary aspergillosis (CNPA), also called semi-invasive pulmonary aspergillosis, was first described in the early 1980s as a distinct type of pulmonary aspergillosis. CNPA was an indolent, cavitary, infectious process of the lung parenchyma secondary to local invasion by Aspergillus species. Diagnosis is confirmed by pathological evidence of lung tissue invasion by the fungus. Clinical diagnosis by combined clinical, radiological and laboratory findings is needed because histopathological confirmation cannot always be obtained in the clinical setting. CNPA is recognized as a clinical syndrome in Japan, and has been poorly defined histologically. We report three autopsy cases of CNPA evaluated histopathologically. Subjects were middle-aged to older men with a medical history of pulmonary mycobacterial infection who presented with pulmonary or systemic symptoms. Radiologically, progressive upper lobe cavitary infiltrates were seen with mycetomas and infiltration in lower lung fields. Clinically, CNPA was diagnosed based on 2007 Japanese guidelines for the diagnosis and treatment of deep fungal infection. Subjects died of respiratory failure within one month to three years of diagnosis despite antifungal therapy including micafungin, voriconazole, or itraconazole combined with broad spectrum antibiotics. Autopsy findings showed cavities containing the fungus but no fungal invasion of viable lung tissue. The area of progressive infiltration revealed bacterial pneumonia, organizing pneumonia or organizing diffuse alveolar damage without Aspergillus. In conclusion, CNPA is a chronic progressive clinical form of pulmonary aspergillosis with significant morbidity and mortality.

Diffuse Pulmonary Ossification with Connective Tissue Weakness Potentially Due to Vascular Ehlers-Danlos Syndrome.

A 30-year-old non-smoking man was referred to our hospital for the further examination of abnormal shadows revealed by chest X-ray. He had mild shortness of breath. Chest computed tomography revealed a fine-grained dendritic shadow with diffuse calcification in both lungs and as well as emphysematous changes in the upper lung lobes. A surgical lung biopsy histology revealed diffuse pulmonary ossification complicated with lung laceration, vascular disruption, hemosiderosis, and emphysema, suggesting vascular Ehlers-Danlos syndrome (vEDS). However, the patient had no external physical signs or family history of vEDS and no COL3A1 gene mutations. We are closely monitoring this patient in the clinic.

Asymmetrical Interstitial Lung Disease Suggested to Be Due to Hypoplasia of the Unilateral Pulmonary Artery: A Case Report with a 20-year Follow-up.

We herein report a case of asymmetrical interstitial lung disease (ILD) that remained almost completely asymmetrical over time on chest computed tomography (CT). An open lung biopsy from the right lung showed severe pleural adhesion, obstruction of the pulmonary artery, and dilated systemic arteries in addition to the usual interstitial pneumonia pattern. Three-dimensional CT angiography showed partial defects of pulmonary arteries on the affected side. After excluding other known causes of ILD and gastroesophageal reflux, we suspected that decreased pulmonary artery perfusion in the present case may have been responsible for the observed asymmetrical unilateral fibrosis.

Simultaneous presence of lung adenocarcinoma and malignant pleural mesothelioma: A case report.

The co-presence of malignant pleural mesothelioma (MPM) and lung cancer is rare. We report a 70-year-old male with exposure to asbestos. Chest computed tomography revealed a right mediastinal mass combined with an enlarged ipsilateral lymph node and left pleural effusion. Transbronchial lung biopsy revealed lung adenocarcinoma. Thoracoscopic examination revealed multiple left pleural nodules, leading to the diagnosis of MPM. Despite aggressive anticancer drug therapy, he expired due to disease progression 2.5 years after diagnosis. Autopsy confirmed an epithelioid MPM in the left pleura. MPM comorbidity in patients diagnosed with lung cancer should be considered, especially in those exposed to asbestos.

[Value of 18F-fluorodeoxyglucose-PET in the diagonosis and monitoring of pulmonary sarcoidosis: a case report].

A 46-year-old woman was referred to our hospital because of abnormal findings of 18F-fluorodeoxyglucose-PET (FDG-PET) scan at a medical check-up. Her chest radiography and CT showed nodular shadows distributed along the broncho-vascular bundle. The 1st FDG-PET showed a high FDG uptake in the pulmonary nodules, and the hilar, mediastinal and supraclavicular lymph nodes. Although sarcoidosis was suspected, diagnosis was not obtained by bronchoscopy. Thoracoscopic lung biopsy was performed in order to exclude malignancy, resulting in a definite diagnosis of sarcoidosis. No treatment was given and subsequently spontaneous regression was obtained. The follow-up FDG-PET showed no abnormal uptake at all. FDG-PET has been widely used in the assessment of malignancy, however recent reports have indicated that sarcoid lesions could have increased FDG activity. FDG-PET may be useful for diagnosis and monitoring in sarcoidosis.

Angiosarcoma of the breast - specific findings of MRI.

We present a case of low-grade angiosarcoma of the breast. A 26-year old woman presented with a lump in the left breast. An elastic hard and ill-defined tumor, 80 x 50 mm in size, was palpated in the upper region of her left breast. Mammography showed a dense lesion with poorly defined border. Ultrasonography showed a hyper-and hypo-echoic lesion with an unclear border, but no definite tumor. Fine needle aspiration cytology showed no evidence of malignancy. Therefore, she was followed with a diagnosis of mastopathy. Six months later, the lump got enlarged. A contrast-enhanced MRI of the breast was performed. It showed a 100 x 60 mm enhancing vascular mass. Most parts of the tumor enhanced remarkably at the early phase, and prolonged enhancement was recognized at the late phase. Core needle biopsy was performed, and a possible angiosarcoma was diagnosed. It is not easy to diagnose the mammary angiosarcoma. MRI may contribute to the accurate diagnosis and play an important role regarding this entity.

Elemental analysis of occupational granulomatous lung disease by electron probe microanalyzer with wavelength dispersive spectrometer: Two case reports.

The parenchymal lung diseases caused by metal inhalation include interstitial fibrosis, giant cell interstitial pneumonitis, chemical pneumonitis, and granulomatous disease, among others. We reported two cases of granulomatous lung disease with occupational exposure to metal dusts other than beryllium. They had worked in the battery manufacturing industry for 7 years and in an aluminum-processing factory for 6 years, respectively. Chest high-resolution computed tomography showed diffuse micronodules, and histology of video-assisted lung biopsy specimens revealed granulomatous lesions in the pulmonary interstitium. Results of microscopic examination of the tissue with special stains for mycobacteria and fungi were negative. Analysis by an electron probe microanalyzer with a wavelength-dispersive spectrometer (EPMA-WDS) confirmed the presence of silicon, iron, aluminum, and titanium in the granulomas. In particular, aluminum was distributed in a relatively high concentration in the granulomatous lesions. Although chronic beryllium disease is well known as an occupational granulomatous lung disease, much less is known about the other metals that cause granulomatous reactions in humans. Our report pointed out manifestations similar to beryllium disease after other metal dust exposures, in particular aluminum exposure. To our knowledge, this is the first report showing two-dimensional images of elemental mapping in granulomatous lesions associated with metal inhalation using EPMA-WDS.

IgG4-related Kidney Disease in Which the Urinalysis, Kidney Function and Imaging Findings Were Normal.

IgG4-related kidney disease (IgG4RKD) is recognized as a fibroinflammatory disease characterized by storiform fibrosis, lymphoplasmacytic infiltration and a high serum IgG4 level. A renal biopsy is necessary to diagnose IgG4RKD in patients without any lesions in other organs. Nephrologists typically perform renal biopsies in patients with abnormal urinalysis, such as proteinuria or hematuria, or renal failure. However, we experienced a patient with IgG4RKD without abnormalities in the urinalysis, renal function or imaging, who had severe interstitial lesions. We therefore propose that renal biopsies should be considered if patients do not show abnormal urinalysis findings and are suspected to have IgG4RKD.

Late-onset familial amyloid polyneuropathy: an autopsy study of two Japanese brothers.

We report an autopsy study of late-onset familial amyloid polyneuropathy with a variant transthyretin Val30Met in 2 brothers living in Kyoto, Japan. The disease onsets were at 64 and 59 years, and they died at 71 and 74 years old, respectively. They exhibited almost the same postmortem findings. Amyloid deposition was remarkable in the hearts, but was not seen in the renal glomeruli. In the peripheral nervous system, amyloid deposition was most prominent in the nerves immediately caudal to ganglia, moderate in the dorsal and sympathetic ganglia, and mild in the spinal roots, sciatic nerves, and distal nerves. The difference between the amyloid deposition in the proximal portion and distal portion of the extremity nerves appeared to be greater in the late-onset type than in the ordinary type, and this proximal deposition of amyloid may have induced severe distal nerve fiber degeneration.

Reversed halo sign in pulmonary infarction with tumor emboli: a case report.

We describe the case of a 79-year-old woman with pulmonary infarction due to tumor emboli whose high-resolution CT (HRCT) scan demonstrated the reversed halo sign. The patient had gastric cancer and died because of cancer-related cerebral infarction. On autopsy, the central ground-glass area of the reversed halo sign on HRCT corresponded to pulmonary edema associated with alveolar septal capillary metastasis, whereas the peripheral ring-like consolidation consisted of a hemorrhagic infarct with tumor emboli. The present case is important because a detailed pathologic correlation with this unique HRCT appearance was revealed.

AP-VAS 2012 case report: anti-glomerular basement membrane disease with high titer of myeloperoxidase anti-neutrophil cytoplasmic antibody-an autopsy case report.

It has been reported that patients who are positive for both myeloperoxidase anti-neutrophil cytoplasmic antibody (MPO-ANCA) and anti-glomerular basement membrane (GBM) antibody have a poor prognosis. We present an autopsy case of anti-GBM disease with a high titer of MPO-ANCA. The patient was a 77-year-old woman with a medical history of idiopathic interstitial pneumonia. After being treated for bacterial pneumonia, she was referred to our hospital for evaluation of non-nephrotic range proteinuria, hematuria, and a course of rapidly progressive glomerulonephritis. Results of urinalysis were 2+ for protein and 3+ for blood, with many dysmorphic red blood cells observed in the urinary sediment. A sample of a 24-h urine collection contained 0.3 g protein. The serum creatinine concentration was 5.0 mg/dl on admission. The patient tested positive for MPO-ANCA at a titer of >640 EU and for anti-GBM antibody at a titer of 14 EU. Renal biopsy revealed glomerulonephritis with crescent formation, and immunofluorescence studies showed that the glomeruli had a generalized linear fluorescence and anti-immunoglobulin G (IgG) and C3 along the peripheral glomerular capillaries. She was diagnosed with anti-GBM disease. Treatment was started with intravenous prednisolone and oral cyclophosphamide, followed by plasma exchange. Despite improved renal function, she died of pulmonary hemorrhage. Autopsy revealed deposits of IgG and C3 in the basement membranes of lung alveoli.

Case of interstitial lung disease with anti-EJ and anti-CCP antibodies preceding rheumatoid arthritis.

Autoantibodies against aminoacyl-tRNA synthetases (ARSs) are highly specific for myositis and/or interstitial lung disease. We report a rare case of double positive antibodies (anti-EJ antibody, the least common among anti-aminoacyl-tRNA synthetase antibodies, and anti-cyclic citrullinated peptide antibody, reported to be specific for rheumatoid arthritis) in a patient who presented with interstitial lung disease and later developed rheumatoid arthritis. The patient did not have clinically apparent myositis over a period of careful follow-up of several years. The initial pulmonary pathologic findings showed a nonspecific interstitial pneumonia pattern, with the formation of lymphoid follicles, which should be recognized as the first manifestation of rheumatoid arthritis.

Comparison between mucinous cystic neoplasm and intraductal papillary mucinous neoplasm of the branch duct type of the pancreas with respect to expression of CD10 and cytokeratin 20.

OBJECTIVE: Mucinous cystic neoplasm (MCN) and intraductal papillary mucinous neoplasm of the branch duct type (IPMN-BD) differ in biological and clinical behaviors, but MCN is often misdiagnosed as IPMN-BD. The purpose of this study was to find useful markers for the differential diagnosis of MCN and IPMN-BD. METHODS: Immunohistochemically, the expression of the 2 types of mucin (MUC) 1 (MUC1/DF3 and MUC1/CORE), MUC2, MUC5AC, MUC6, human gastric mucin (HGM), caudal-related homeobox transcription factor 2 (CDX2), CD10, cytokeratin (CK) 7, and CK20 was examined in 7 cases of MCN and 16 cases of IPMN-BD. RESULTS: Expression frequencies in MCN and IPMN-BD were 100% versus 44% for MUC1/DF3, 86% versus 31% for MUC1/CORE, 57% versus 19% for MUC2, 86% versus 100% for MUC5AC, 57% versus 88% for MUC6, 86% versus 100% for HGM, 57% versus 0% for CDX2, 71% versus 0% for CD10, 100% versus 69% for CK7, and 86% versus 6% for CK20. CONCLUSIONS: Mucin 1/DF3, MUC1/CORE, CDX2, CD10, and CK20 were expressed significantly more frequently in MCN than in IPMN-BD. In particular, CD10 and CK20 showed marked differences in immunohistochemical sensitivity and specificity between MCN and IPMN-BD. It is therefore proposed that CD10 and CK20 may be used for the differential diagnosis of MCN and IPMN-BD.