[Mucosa-associated lymphoid tissue lymphoma in the thymus with multiple amyloid nodules in the both lungs].

Here, we present a case of mucosa-associated lymphoid tissue (MALT) lymphoma in the thymus with multiple amyloid nodules in both lung. A 66-year-old woman was incidentally found to have an abnormal shadow on mass-screening chest roentgenogram. A chest computed tomography (CT) demonstrated a mass of 50 mm in diameter with a smooth margin adjacent to the heart in the anterior mediastinum and multiple small nodules in both lung. As a differential diagnosis, thymic carcinoma with multiple lung metastases was firstly considered from these clinical informations. To make a definite diagnosis, the operation via a thoracoscopy was done. As a result, it turned out that pulmonary nodules were amyloidosis and the thymic tumor was MALT lymphoma. Postoperative course was uneventful and she was treated with chemoradiotherapy. In addition, she was diagnosed with Sjögren's syndrome 1 and half years later. Four years later the patient has been well without recurrence.

Heat stress induces alkaline phosphatase activity and heat shock protein 25 expression in cultured pulp cells.

AIM: To investigate the responses of cultured rat pulp cells to heat stress. METHODOLOGY: Pulp cells were obtained from rat incisors and cultured at 37 degrees C. The cells were cultured at 42 degrees C for 30 min and then cultured at 37 degrees C again. Morphology, alkaline phosphatase (ALP) activity and expression of heat shock protein 25 (HSP25) were investigated at 0, 1, 3, 5, 7, 10 and 14 days following stimulation. As a control, the cells were maintained at 37 degrees C. RESULTS: Although there were few cells of apoptosis immediately after heat stress, there were mitotic cells from day 1 after heat stress. ALP activity in the heat stress group significantly increased at days 7 and 14 compared with the control group (about 1.7-fold, P < 0.01, Friedman test). HSP25 expression increased in both groups, with HSP25 in the heat stress group being expressed earlier than in the control group, and nuclear localization of HSP25 was observed at days 0 and 1 in heat-stressed cells. CONCLUSION: These results suggest that heat stress not only induces HSP25 but also enhances ALP activity in pulp cells.

[New forceps for thoracoscopic needle biopsy].

INTRODUCTION: When the diagnosis cannot be established preoperatively but malignant lung tumor is suspected, we frequently perform thoracoscopic wedge resection in order to perform rapid histodiagnosis on the specimen. If the diagnosis is malignancy, we extend the surgery to lobectomy for complete resection in many cases. However, cartridges of linear endoscopic staplers used for wedge resection are useless in such cases. This economic loss is expensive. Thoracoscopic needle biopsy is economic, but the technique is difficult and there is a risk of damage to important blood vessels when the needle penetrates deeper than is needed. Therefore, we developed forceps for thoracoscopic needle biopsy. METHOD: We changed the tip shape of endoscopic grasping forceps, fixed a guide for inserting a biopsy needle and prevented the biopsy needle from going through the grasping extension for safety. We made 3 types of forceps, small, middle, and large sized forceps that could adapt the various sizes of tumors. RESULT: We used the small forceps for 23 cases: the middle forceps for 13 cases; the large forceps for 7 cases; for a total of 43 cases, and succeeded in diagnosing 35 cases. The reason for failure in 6 cases using the small forceps was the exceeding softness of the lesion in 1 case, failure of rapid histodiagnosis in 1 case, and mal-adaptation between the forceps and tumor size in the remaining cases. The reason for failure in 2 cases using middle forceps was failure of rapid histodiagnosis in both cases. There was no complication due to biopsy. All bleeding after the puncture was quickly stopped. There was no dissemination or recurrence in the thoracic lumen. CONCLUSION: During surgery for palpable visible lung tumors with an uncertain histological diagnosis, thoracoscopic needle biopsy is very easy and economic. It is also useful for avoiding unnecessary lung lobectomy, and is a minimally invasive method, contributing to medical economy.

[A case of chondrosarcoma originating from the rib and presenting as a posterior mediastinal tumor-like shadow].

A 66-year-old man was admitted to our hospital because of induction therapy of interferon for chronic hepatitis due to hepatitis C virus. On routine investigation, a mass-like lesion was detected at the level of the hilum of the left lung on the chest plain radiograph. On the thoracic CT, the heterogeneous tumor, including calcification, was 6 cm in diameter in the posterior mediastinal area, and the rib and vertebra had been damaged by it. The tumor extended to the descending aorta and the left main bronchus. A small nodule in the left S1 + 2 was noted on another slice section of this thoracic CT. The possible diagnoses, based on these radiological findings, for this tumor were primary lung cancer, posterior mediastinal tumor and malignant tumor originating from the chest wall. The diagnosis of chondrosarcoma was made based on the histological findings of the specimen obtained from the tumor. This tumor could not be resected in this case, because it had extensively damaged the rib and vertebra, and the nodular lesion in the left S1 + 2 was regarded as a metastasis from it. Although reports of chondrosarcoma originating from a rib and presenting as a posterior mediastinal tumor-like shadow are rare, chondrosarcoma should be considered as a possible diagnosis for such tumors-posterior mediastinal masses with characteristic findings such as a large tumor in contact with the chest wall, and containing calcification, with destruction of bone and dissection of the rib as shown in our CT findings.

[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.

Genetic classification of combined hepatocellular-cholangiocarcinoma.

Combined hepatocellular-cholangiocarcinoma (combined HCC/ CC) is a rare form of liver neoplasms showing both hepatocellular (HCC) and bile duct differentiation (CC). In an attempt to clarify the clonality and genetic/phenotypic relationships in the evolution of these neoplasms, we microdissected multiple HCC and CC foci and studied allelic status of chromosome arms 1p, 1q, 3p, 4q, 5q, 6q, 8p, 9p, 10q, 11q, 13q, 16q, 17p, 17q, 18q, and 22q. Overall, the highest frequency of loss of heterozygosity (LOH) was seen on 4q and 17p, followed by 8p and 16q. Of the 11 cases studied, 3 cases did not show any of the identical allelic losses between HCC and CC foci, indicating the biclonal nature. The remaining 8 cases showed multiple allelic losses shared between both components, strongly suggestive of a single clonal derivation. Moreover, 4 of the 8 cases showed additional or divergent allelic losses at more than 1 chromosomal locus only in HCC and/or CC foci. Thus, this heterogeneity was shown to affect the phenotypic diversity of the tumor. Summarizing the genetic patterns, combined HCC/CC could be classified into the following 3 possibilities: (1) collision tumor in which 2 independent neoplastic clones develop at close proximity; (2) single clonal tumor with homogeneous genetic background in both components--histological diversity is thus a manifestation of divergent differentiation potential of a single clone; (3) single clonal process in which genetic heterogeneity in the process of clonal evolution within the tumor parallels histologic diversity; therefore, the tumor in this category is mainly composed of mosaics of closely related subclones.

[Idiopathic plasmacytic lymphadenopathy with polyclonal hyperimmunoglobulinemia and pleural effusion].

A 56-year-old man was admitted for further evaluation of mediastinal lymphadenopathy and left pleural effusion. A blood test revealed polyclonal hypergammaglobulinemia with an elevated erythrocyte sedimentation rate and level of C-reactive protein. Chest computed tomography disclosed bilateral hilar and multiple mediastinal lymph-node swelling and bilateral pleural thickening accompanied by a moderate amount of left pleural effusion. Biopsy specimens from mediastinal lymph nodes and the right pleura were obtained under video-assisted thoracoscopy. Histology on microscopic examination demonstrated mature plasma cells and lymphocyte infiltration in the conserved lymph-node structure and the pleura, suggesting a diagnosis of idiopathic plasmacytic lymphadenopathy (IPL) with polyclonal hyperimmunoglobulinemia. Interestingly, IL-6 was elevated in the pleural effusion but normal in serum. This was a rare and instructive case of IPL accompanied by pleural effusion that was considered to be an important determinant of the clinical spectrum of the disease.

[Arteriovenous fistula associated with Staphylococcus aureus sepsis in a patient with Rendu-Osler-Weber syndrome].

A 58-year-old man with a history of cerebral infarction and bleeding due to duodenal ulcer was admitted with fever and arthralgia. Methicillin-sensitive Staphylococcus aureus (MSSA) was isolated from his peripheral blood. Bacteremia with MSSA was diagnosed, and antibiotic therapy was started. However, chest X-ray films and computed tomographic scans disclosed mass shadows in both lungs accompanied by dilated vascular markings. Pulmonary arteriography and magnetic resonance angiography revealed the existence of arteriovenous fistulas in both lungs. Ga scintigraphy disclosed a hot spot in the left lower lobe, consistent with the location of one fistula. This indicated that the fistula might be the focus of MSSA sepsis. Because the patient also had telangiectasia in his gastric mucosa, oral cavity, and nasal cavity, he was given a diagnosis of Rendu-Osler-Weber syndrome.

[Nontuberculous mycobacterial infection followed for 12 years].

A 67-year-old woman presented in September 1985 with productive cough, bloody sputum, and dyspnea on exertion. Productive cough and bloody sputum had developed when the patient was 55 years old. Sputum culture and radiologic findings yielded a diagnosis of nontuberculous mycobacteriosis (NTM). Antituberculous therapy with INH, RFP, and EB was initiated in November 1987 because of the development of a cavity in the right upper lobe, and led to resolution of the lesion and clinical symptoms. Despite progression of bronchiectatic changes in both lungs and a relapse of her clinical symptoms during the following 10 years, the patient retained enough pulmonary function to be able to maintain an active daily life until she died of advanced gastric cancer at the age of 79. Autopsy revealed cystic bronchiectasis accompanied by bronchial wall thickening in both lungs, with some granuloma and acid-fast-bacteria observed in lung tissue. In this report, we concluded that patients with NTM usually experience a gradual progression of symptoms and radiographic changes during their clinical course, and that their pulmonary function may be conserved well enough to maintain an active daily life.

[Marked airway constriction due to relapsing polychondritis].

A 40-year-old woman was admitted to our hospital presenting with dry cough, sore throat, and fever. Her right auricle was markedly swollen and her ocular conjunctiva were reddened, suggesting scleritis. A chest computed tomogram showed narrowing of the trachea due to enlarged mediastinal soft tissues. These clinical findings suggested the patient had relapsing polychondritis. A cartilage biopsy from her right auricle was taken to confirm the diagnosis. Pathological findings revealed loosening and dissolution of cartilage and infiltration of lymphocytes, which were consistent with relapsing polychondritis. Although prednisolone was given to the patient, her symptoms were not alleviated. Methylprednisolone pulse therapy and oral diaminodiphenylsulfone were added to the patient's treatment regimen. With this combination, her symptoms gradually subsided. Constriction of the airway has been cited as one of the prognostic factors in relapsing polychondritis. In view of the danger of sudden death caused by airway obstruction, close observation of the patient in this case was considered necessary.

Diffuse panbronchiolitis in rheumatoid arthritis.

The association of progressive obliterative bronchiolitis (OB) with rheumatoid arthritis (RA) is uncommon but has been reported previously. Diffuse panbronchiolitis (DPB) is a unique inflammation principally affecting the respiratory bronchioli and has been reported mainly in Japanese adults. Recently, DPB has also been noted in patients with RA in Japan. Therefore, there might be considerable overlap in clinical features between DPB and OB associated with RA in Japan. The aim of this study was to evaluate the clinicopathological characteristics of bronchiolitis in patients with RA. Three RA patients clinically diagnosed as having DPB were evaluated. All patients underwent chest radiographs, pulmonary function tests (PFT) and post mortem examination. Clinical features in all patients were a history of productive cough, exertional dyspnoea, wheezing and/or coarse crackles. Chest radiographs showed small nodular shadows up to 2 mm in diameter with bronchiolectasis throughout both lungs in all patients. The PFT revealed marked obstructive impairment in all patients. All patients died of progressive respiratory failure. Pathologically, two out of the three cases were confirmed as DPB, while the remaining one case was confirmed as OB, because the primary obstructive lesions were in the respiratory bronchioli in the former and in the membranous bronchioli and the proximal small bronchi in the latter. Thus, the clinical features of DPB and OB were strikingly similar, but the histopathological features revealed distinct differences. This study demonstrated that there was considerable overlap in clinical features between diffuse panbronchiolitis and obliterative bronchiolitis associated with rheumatoid arthritis, suggesting that diffuse panbronchiolitis might be a new manifestation of rheumatoid arthritis. The differentiation of these two disease entities is significant in making decisions on their therapeutic modality and is possible by analysing the precise histopathological findings of the lung.

[Pulmonary metastasis of axillary sweat gland carcinoma: a case report].

A case of axillary sweat gland carcinoma which metastasized to both lungs five years after resection of the primary lesion is described. After the resection of right lung metastasis, systemic chemotherapy was performed, but no response was achieved. The patient has no complaints now, but there are new and multiple metastases in both lungs. I propose that the complete remission can be achieved only by resections.

[Summer-type hypersensitivity pneumonitis with remarkable obstructive impairment of pulmonary function].

A 57-year-old-man was admitted because of persistent cough and progressive dyspnea over a period of 2 months. Examination revealed wheezing in both lungs, severe hypoxemia, and marked obstructive impairment of pulmonary function. A chest CT scan showed diffuse small nodular lesions with patchy air space opacifications. The patient was initially given a diagnosis of bronchiolitis of unknown cause. He showed marked improvement after treatment with oral prednisolone, but developed fever and dyspnea after returning home. This episode indicated hypersensitivity pneumonitis, a diagnosis supported by findings of increased CD 8 positive T-lymphocytes in bronchoalveolar lavage fluid, and a high titer of serum anti-Trichosporon antibody. Lung biopsy samples obtained under video-assisted thoracoscopy disclosed noncaseating granulomas in terminal and respiratory bronchioles, which resulted in marked narrowing of the lumen. The pathologic changes seemed to be consistent with obstructive impairment of pulmonary function in this patient.

[Allergic granulomatous angiitis associated with cerebral infarction, myo-pericarditis and acute respiratory failure due to eosinophilic pneumonia].

We encountered a 23-year-old woman with allergic granulomatous angiitis (AGA) associated with cerebral infarction, myo-pericarditis, and acute respiratory failure due to extended eosinophilic pneumonia. She underwent emergency treatment at our hospital because of right hemiparesis and impaired consciousness. AGA was suspected because the patient had a history of bronchial asthma accompanied by pulmonary infiltrations with eosinophilia, and presented with diffuse pulmonary infiltrates, pericardial effusion, diffuse hypokinesis of myocardium, cerebral infarction and marked peripheral eosinophlia. Pulmonary eosinophilia was confirmed by examination of broncho-alveolar lavage fluid. Myocardial tissue biopsy specimens revealed fibrous granulation indicative of myocarditis. The patient responded well to corticosteroid therapy.

Progressive interstitial pneumonia associated with myelodysplastic syndrome: implication of superoxide hyperproduction by neutrophils.

Interstitial pneumonia and aseptic neutrophilic infiltration in the lung are rare pulmonary manifestations of myelodysplastic syndrome (MDS). We report a patient with progressive interstitial pneumonia associated with MDS. Histological examination of the lung revealed infiltration of atypical haematopoietic cells associated with MDS and diffuse alveolitis with honeycombing. Neutrophils obtained from the patient showed superoxide hyperproduction after stimulation with phagocytosis and phorbol myristate acetate, which might be attributed to the pathogenesis of interstitial pneumonia.

[Pulmonary cavernous hemangioma--identification of its endothelial cell origin by immunohistological staining].

A 45-year-old woman was referred to our hospital because of a tumorous shadow in the S10 segment of the left lung. A chest computed tomography (CT) scan showed a nodular lesion with a slightly irregular margin and no contrast enhancement. CT-guided aspiration biopsy was tried but did not result in a histological diagnosis. The tumor was excised during video-assisted thoracoscopic surgery. Histological examination of the specimen revealed cavernous hemangioma. Most cells lining the lumen of the cavernous structure stained positively for von Willebrand factor antibody and negatively for anti-epithelial membrane antigen antibody, which suggests that the tumor was associated with endothelium. To the best of our knowledge, this is the first report of a case in which the diagnosis of pulmonary cavernous hemangioma was confirmed with an immunohistological study.

Diffuse progressive pulmonary interstitial and intra-alveolar cholesterol granulomas in childhood.

We describe an 18 year old male with pulmonary interstitial and intra-alveolar cholesterol granulomas (PICG), which developed to severe respiratory failure over 15 yrs. The histological diagnosis was made on the basis of open lung biopsy findings at the age of 3 yrs and autopsy at 18 yrs of age. Although the pathological features of the lung were similar to that of paediatric patients with lipoid pneumonia coexisting with pulmonary alveolar proteinosis (PAP), gastro-oesophageal reflux (GOR) and a diverse group of severe primary diseases, the patient lacked evidence for any of these. We believe the present case provides a new example of a diffuse-type of lipoid pneumonia coexisting with pulmonary alveolar proteinosis, which we call cholesterol granulomas.

[Pulmonary eosinophilic granuloma with relatively rapid progression and remission].

A 53-year-old woman with a smoking history of 20 pack-years was admitted to the hospital because of dry coughing and exertional dyspnea that had progressed over the previous 3 months. A chest X-ray film taken one month before the onset of symptoms was almost normal. Diffuse small nodular shadows with infiltration were evident one month after the onset of symptoms, and increased rapidly until admission to the hospital. Eosinophilic granuloma was diagnosed histologically after thoracoscopy. The symptoms, chest X-ray findings and pulmonary-function data improved spontaneously after admission to the hospital and the nodular shadows had a almost completely disappeared by 5 months later; the patient continued to smoke. The present case is unique and valuable because it shows the natural course of eosinophilic granuloma from the onset of symptoms to remission.