Pulmonary alveolar microlithiasis (PAM) is a rare disease with unknown etiology and pathogenesis. It is characterized by diffuse, innumerable, and minute calculi, called microlithiasis in the alveoli. More than half of reported cases are asymptomatic at the time of diagnosis. We describe the first case of PAM in Korea. A 19-yr-old man without respiratory symptoms presented with interstitial thickening on the chest radiograph. His chest high resolution CT scan showed diffusely scattered, ill defined tiny micronodules and interstitial thickening. Open lung biopsy confirmed the diagnosis of PAM. He was followed up for 6 months without treatment, and no progression was noticed.
Humans ; Lithiasis/*diagnosis/pathology/radiography ; Lung Diseases/*diagnosis/pathology/radiography ; Male ; Pulmonary Alveoli/*pathology/radiography ; Republic of Korea ; Young Adult

Alveolar soft part sarcoma (ASPS) is a rare epithelial-like soft tissue sarcoma. The two main sites of its occurrence are the lower extremities in adults and the head and neck in children. Primary pulmonary involvement of this sarcoma, without evidence of soft tissue tumor elsewhere, is very exceptional. We present a case of primary ASPS of the lung in a 42-yr-old woman. A computed tomographic scan of the thorax demonstrated a well-circumscribed, solid tumor located in the right upper lobe. The mass was resected by right upper lobectomy. After 5 months, three metastatic lesions, involving lumbar vertebrae and occipital scalp, were found. Histologically, the tumor consisted of alveolar nests of large polygonal tumor cells, the cytoplasm of which frequently revealed periodic acid-Schiff-positive, diastase-resistant intracytoplasmic rod-like structures. On immunohistochemical staining, the tumor cells were positive only for vimentin and alpha-smooth muscle actin. Ultrastuctural study using electron microscopy revealed characteristic electron-dense, rhomboid intracytoplasmic crystals.
Soft Tissue Neoplasms/pathology/radiography ; Sarcoma/*pathology/*radiography ; Rare Diseases/pathology/radiography ; Pulmonary Alveoli/*pathology/*radiography ; Lung Neoplasms/*pathology/*radiography ; Humans ; Female ; Adult

OBJECTIVE: The aim of this study was to analyze the computed tomographic (CT) findings of atypical adenomatous hyperplasia (AAH) in the lung. MATERIALS AND METHODS: The CT findings of AAHs in eight patients were retrospectively reviewed. The CT findings of each AAH lesion were evaluated for multiplicity, location, shape, size and internal density of the lesion, the interface between the normal lung and the lesion, the internal features within the lesion and any change of the lesion on the follow-up CT scans (range: 33 to 540 days; average: 145.3 days). RESULTS: The eight patients consisted of three men and five women (age range: 43-71 years). Six of eight patients were asymptomatic. Four of them (50%) had synchronous malignancies in the lung: adenocarcinoma of the lung (n = 3), and metastatic squamous cell carcinoma from the uterus (n = 1). We could identify and evaluate eleven AAH nodules in seven patients on the CT scans. Three patients had multiple AAHs. Seven of the 11 lesions (64%) were located in the upper lobe. All the AAHs showed a well-defined oval or round shape and pure ground-glass opacity (GGO) without any solid component (size: 3.9x3 mm to 19x17 mm; internal attenuation: -467 to -785 HU). All the AAHs showed no change of their size and internal density on the follow-up CT scans. CONCLUSION: Atypical adenomatous hyperplasia is often associated with malignancy. This tumor is shown on CT as persistent well-defined oval or round nodular GGOs without solid components, and it does not change on the follow-up CT.
*Tomography, X-Ray Computed ; Retrospective Studies ; Pulmonary Alveoli/pathology ; Precancerous Conditions/pathology/*radiography ; Middle Aged ; Male ; Lung Neoplasms/epidemiology/radiography ; Lung/*pathology/*radiography ; Hyperplasia ; Humans ; Female ; Epithelial Cells/pathology ; Aged ; Adult ; Adenocarcinoma/pathology/radiography

We report a case of pulmonary fibrosis in a 32-year-old man, who had worked at a steel mill and who died of respiratory failure due to interstitial fibrosis despite vigorous treatment. He showed SLE-associated symptoms, such as pleural effusion, malar rashes, discoid rashes, arthritis, leukopenia, and positive antinuclear antibody and anti-histone antibody. However, he did not present anti-DNA antibody. A thoracoscopic lung biopsy showed interstitial fibrosis, chronic inflammation and a small non-caseating granuloma in lung tissues, which could be induced by external agents such as metals. The manganese concentration in the lung tissue was 4.64 microg/g compared to 0.42-0.7 microg/g in the controls. The levels of other metals, such as iron, nickel, cobalt and zinc in patient's lung tissue were higher than those in the controls. The patient was probably exposed to Si and various metal dusts, and the lung fibrosis was related to these exposures. Exposure to Si and metal dusts should be sought in the history of any patient with SLE, especially in a male with pulmonary signs, and if present, exposure should be stopped. In the meantime, steps should be taken to ensure that workers exposure to Si and metal dusts in all environments have adequate protection.
Adult ; Biopsy ; Case Report ; Fatal Outcome ; Human ; Male ; Occupational Diseases/radiography* ; Occupational Diseases/pathology ; Occupational Diseases/chemically induced* ; Occupational Exposure ; Pulmonary Alveoli/pathology ; Pulmonary Fibrosis/radiography* ; Pulmonary Fibrosis/pathology ; Pulmonary Fibrosis/chemically induced* ; Respiratory Insufficiency/radiography ; Respiratory Insufficiency/pathology ; Respiratory Insufficiency/chemically induced ; Steel/adverse effects*

Pulmonary alveolar microlithiasis (PAM) is a rare chronic disease with paucity of symptoms in contrast to the imaging findings. We present a case of a 24-year-old Malay man having an incidental abnormal pre-employment chest radiograph of dense micronodular opacities giving the classical "sandstorm" appearance. High-resolution computed tomography of the lungs showed microcalcifications with subpleural cystic changes. Open lung biopsy showed calcospherites within the alveolar spaces. The radiological and histopathological findings were characteristic of PAM.
Biopsy ; Calcinosis/*diagnosis/surgery ; Chronic Disease ; Genetic Diseases, Inborn/*diagnosis/surgery ; Humans ; *Incidental Findings ; Lung Diseases/*diagnosis/surgery ; Male ; Pulmonary Alveoli/pathology/radiography ; Thoracic Surgery, Video-Assisted/methods ; Tomography, X-Ray Computed/*methods ; Young Adult