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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 lymphangiomatosis is a rare clinical and pathological entity which is distinct from lymphangiomyomatosis and from pulmonary lymphangiectasis. We report a case of a 20 year old man with diffuse lymphangiomatosis involving the mediastinum, lungs and retroperitoneum. The patient's intrathoracic lymphangiomatosis produced restrictive and obstructive impairment of his pulmonary function, but did not result in chylothorax or chylopericardium, which are two common manifestations of the disease. The patient's clinical course was remarkable for the concomitant development of chronic disseminated intravascular coagulation (DIC). Lymphangiomatosis involving both the mediastinum and pulmonary parenchyma is rare, and DIC is a very unusual complication of lymphangiomatosis.

Polyarteritis nodosa (PAN) is characterized by necrotizing arteritis of medium-sized and small arteries in various organs. Pulmonary artery involvement in PAN has been considered rare. Previously, it also has been thought that patients with PAN do not have interstitial pneumonitis and fibrosis. A detailed pathologic analysis of pulmonary diseases associated with PAN was made in 10 autopsy cases of PAN. Arteritis affecting bronchial arteries was present in seven patients (70%). The data obtained suggest that arteritis in the lung in patients with PAN is more common than has been recognized previously. Diffuse alveolar damage (DAD) involving all lobes bilaterally was present in five patients; it was acute in two patients and organizing in three. In the patients with organizing DAD the degree of fibrosis in the interstitium differed among the lobes, and the fibrosis was more severe in the lower lobe than in the other lobes. Two patients presented with interstitial fibrosis with honeycomb lung of the posterior and lateral basal segments of the lower lobes of both lungs; in one of these patients interstitial fibrosis was present in an area of organizing DAD. Five patients died of respiratory failure resulting from DAD. In conclusion, it is important to consider DAD and interstitial fibrosis as complications of PAN.

Behcet's disease associated with large vascular lesions is called vasculo-behcet's disease. The pathologic features of eight autopsy cases of vasculo-Behcet's disease were studied. The patients' ages ranged from 31 to 56 years; there were five men and three women. Of these patients, large vascular lesions were as follows: aneurysm (three cases), aneurysm and arterial occlusion (one case), aneurysm with arterial and venous occlusion (one case), and venous occlusion (two cases). Aneurysms were of the saccular or dissecting type. The aneurysm formation most commonly occurred in the aorta (two cases). Histologically, aortitis was seen in six patients. Aortitis was divided into active and scar stages. In one case with active stage aortitis, granulomatous aortitis similar to Takayasu's arteritis was seen. In conclusion, we believe that the large arterial lesion in vasculo-Behcet's disease represents inflammation occurring in the media and adventitia. In the affected arteries, active arteritis occurs initially, followed by destruction of the media and fibrosis. Saccular aneurysms are probably produced by severe destruction of the media by intense active inflammation. The large venous lesions represent thrombophlebitis.

A 24-year-old woman was admitted due to dyspnea on exertion. A chest roentgenogram revealed a massive tumor originating from the right anterior chest wall when pleural effusion was drained. Diagnosis of Askin tumor was made based on light microscopic findings characterized by composing with small round cells, immunocytochemical findings suggestive of neuroectodermal origin, and cytogenetic analysis demonstrating the chromosomal translocation (11; 22). After intensive combined modality therapy, including chemotherapy, irradiation, and additional surgery, she was followed up as an outpatient and has remained disease-free for 16 months after initial diagnosis.

The age-related incidence of spontaneously occuring neoplastic and non-neoplastic lesions in untreated F-344/Jcl rats, used as controls in carcinogenicity testing, were studied from the histological examination of tissues from 469 males and 354 females. The incidence of spontaneous tumors was 83.2% in the males and 71.2% in the females. The most common neoplasms were leukemia (males : 24.3%, females : 24.0%), pituitary adenoma (males : 16.0%, females : 45.2%), pheochromocytoma (males : 14.7% females : 7.3%, testicular interstitial cell tumor (males : 79.1%), and uterine endometrial stromal polyp(females : 16.4%). The incidence of other tumors of almost all the organs and/or tissues was low. Non-neoplastic lesions generally increased with advancing age of the animals.

Autopsy was performed on a 52 year old man with a 20-year history of neurological symptoms. At autopsy, both a brain stem tumor and a spinal cord tumor were found. These showed the features of pilocytic astrocytoma histologically. A pilocytic astrocytoma was also found in the optic chiasm upon microscopical examination. These three tumors were thought to be multicentric astrocytomas, because there was no continuity among them and no evidence of dissemination or metastasis by any pathway. From a review of the literature, the present case is considered to be an exceedingly rare one because of the multicentric sites of occurrence. Acta Pathol Jpn 39: 664 669, 1989.

正常対照,急性肝炎,慢性非活動性肝炎,慢性活動性肝炎,肝硬変の肝内ケラチンをポリクローナル抗体,4種のモノクローナル抗体(KL-1, PKK-1, 2, 3)を用いて免疫組織化学的に検討した.肝硬変では,正常対照に比べ,ケラチン陽性肝細胞および細胞質全体にケラチン陽性が見られる肝細胞(びまん型肝細飽)が増加した.ケラチン陽性肝細胞,びまん型肝細胞は小葉または再生結節の周辺部,広節な線維化域内及びその周囲に主として存在し,小結節性の再生結節では特に多かった.また,びまん型肝細胞は変性肝細胞,偽腺房様構造を取る肝細胞で頻繁に見られた.これらの結果は,肝細胞の変性,再生性変化に伴い細胞内ケラチンの増加,細胞質全体への分布が起こる事を示し,また肝硬変の再生結節を構成する肝細胞の多くは正常肝細胞とは細胞内ケラチンの量,局在状態は異なる事も示した.

A 73-year-old male metalworker was admitted to our hospital with a 3-year history of progressive dry cough. Chest high-resolution computed tomography revealed emphysematous changes and reticular lesions, which is referred to as combined pulmonary fibrosis and emphysema (CPFE). Surgical lung biopsy specimens revealed unclassified interstitial pneumonia, including a nonspecific interstitial pneumonia pattern and usual interstitial pneumonia pattern. Two years after his first admission he developed rapid progressive renal dysfunction with an elevated level of myeloperoxidase-antineutrophil cytoplasmic antibody (428 EU). A renal biopsy specimen revealed interstitial nephritis and glomerulonephritis. Consequently, microscopic polyangiitis preceded by CPFE was diagnosed. Despite transient exacerbation of renal involvement, his general condition remained mostly stable during a 2-year period of corticosteroid treatment. He ultimately died from severe pneumococcal pneumonia associated with acute lung injury.

Erdheim-Chester disease (ECD) is a rare non-Langerhan’s cell histiocytosis of unknown origin, involving multiple organs. The patient with ECD described here is a 38-year-old man who was admitted to the hospital with dyspnea on exertion. His chest radiograph revealed a diffuse reticulonodular shadow. After the video-assisted thoracoscopic surgery was performed, he was diagnosed as having ECD. A brown eruption on his left temple, when tested by skin biopsy, proved to be ECD. No lesions other than these on the lung and skin were identified, and oral administration of predonisolone successfully treated both of them. Although recovery has followed the administration of predonisolone and chemotherapy for several patients with pulmonary ECD, this is the first report that predonisolone alone provided clinical and objective recovery from pulmonary ECD. This outcome indicates that, of all the many treatments tried for ECD, steroids may become the first-line therapy for pulmonary involvement.

症例は48歳の男性で, 黄疸, 上腹部痛を主訴として入院した.諸検査の結果, 膵頭部癌が疑われ, 胆汁浮遊細胞診, 胆道擦過細胞診が施行された.何度も行った胆汁浮遊細胞診では癌細胞はみられなかったが, 初回胆道擦過細胞診で腺癌細胞が多数みられ, 膵頭部癌と診断された.その後, 全身状態悪化し, 死亡, 剖検となった.剖検所見の検討の結果, 胆道擦過細胞診は胆管の粘膜上皮下に癌細胞が存在する場合でも正診できる検索方法であることが明らかになった.

Introduction Mediastinal schwannomas are typically benign and asymptomatic, and generally present no immediate risks. We encountered a rare case of a giant benign posterior mediastinal schwannoma, complicated by life-threatening cardiac tamponade. Case presentation We report the case of a 72-year-old Japanese woman, who presented with cardiogenic shock. Computed tomography of the chest revealed a posterior mediastinal mass 150 cm in diameter, with pericardial effusion. The cardiac tamponade was treated with prompt pericardial fluid drainage. A biopsy was taken from the mass, and after histological examination, it was diagnosed as a benign schwannoma, a well-encapsulated non-infiltrating tumor, originating from the intrathoracic vagus nerve. It was successfully excised, restoring normal cardiac function. Conclusion Our case suggests that giant mediastinal schwannomas, although generally benign and asymptomatic, should be excised upon discovery to prevent the development of life-threatening cardiopulmonary complications.