Idiopathic interstitial pneumonias are currently classified into four categories: usual interstitial pneumonia, nonspecific interstitial pneumonia with fibrosis, acute interstitial pneumonia and desquamative interstitial pneumonia. The fibrotic process in interstitial pneumonias appears to result from a complex interaction between fibroblasts, other lung parenchymal cells and macrophages. The complex relationship between the local release of growth-promoting cytokines by alveolar macrophages and resident fibroblasts represents a necessary step for fibrosis or remodeling after lung injury. Injury to the epithelium and basement membranes is likely necessary for the fibrotic process to occur. Usual interstitial pneumonia, most frequent among interstitial pneumonias and has a poor prognosis, appears on high-resolution CT as patchy subpleural areas of ground-glass attenuation, irregular linear opacity, and honeycombing. Nonspecific interstitial pneumonia with fibrosis, the second most frequent and has a better prognosis than usual interstitial pneumonia, appears as subpleural patchy areas of ground-glass attenuation with associated areas of irregular linear opacity on CT. Acute interstitial pneumonia with high mortality rate presents as extensive bilateral airspace consolidation and patchy or diffuse bilateral areas of ground-glass attenuation. Desquamative interstitial pneumonia with good prognosis presents as patchy subpleural areas of ground-glass attenuation in middle and lower lung zones.
Human ; Lung Diseases, Interstitial/radiography ; Lung Diseases, Interstitial/physiopathology ; Lung Diseases, Interstitial/pathology*

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

We report a case of huge pulmonary chondroid hamartoma with multilocular cysts in a 38-yr-old male patient. The lobectomy specimen of the left lower lobe showed a large multilocular cystic mass, 11.5 x 10 cm in size. The mass had thin-walled, variable-sized cysts and areas of solid cartilaginous small nodules in the interstitium, which occupied the superior segment and the upper portion of the basal segment. There was no connection with bronchus or vessel. Microscopically, the cysts and cleft-like spaces were lined by ciliated columnar epithelium and the solid components were composed of cartilage, myxohyalinized connective tissue, and adipose tissue. Also seen were foci of calcification within the sclerotic stroma.
Adult ; Cysts/*pathology ; Hamartoma/*pathology ; Humans ; Lung/radiography ; Lung Diseases/*pathology ; Male ; Tomography, X-Ray Computed

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

Biopsy ; Diagnosis, Differential ; Humans ; Lung ; diagnostic imaging ; pathology ; Lung Diseases, Interstitial ; classification ; diagnostic imaging ; pathology ; Radiography

OBJECTIVE: To evaluate the inter-group differences in growth and the pathological results of nodular ground-glass opacities (GGOs) according to their size and focal solid portions. MATERIALS AND METHODS: Ninety-six nodular GGOs in 55 individuals followed by CT for at least one month from an initial chest CT were included. Forty nodular GGOs in 30 individuals were pathologically confirmed to be: adenocarcinoma (n = 15), bronchioloalveolar carcinoma (BAC) (n = 11), atypical adenomatous hyperplasia (AAH) (n = 8), focal interstitial fibrosis (n = 5) and aspergillosis (n = 1). Lesions were categorized based on high-resolution CT findings: pure nodular GGO (PNGGO) < or = 10 mm, PNGGO > 10 mm, mixed nodular GGO (MNGGO) < or = 10 mm, and MNGGO > 10 mm. In each group, the change in size during the follow-up period, the pathological results and the rate of malignancy were evaluated. RESULTS: Three MNGGO lesions, and none of the PNGGO, grew during the follow-up period. Resected PNGGOs < or = 10 mm were AAH (n = 6), BAC (n = 5), and focal interstitial fibrosis (n = 1). Resected PNGGOs > 10 mm were focal interstitial fibrosis (n = 4), AAH (n = 2), BAC (n = 2), and adenocarcinoma (n = 2). Resected MNGGOs < or = 10 mm were adenocarcinoma (n = 2), and BAC (n = 1). Resected MNGGOs > 10 mm were adenocarcinoma (n = 11), BAC (n = 3), and aspergillosis (n = 1). CONCLUSION: Mixed nodular GGOs (MNGGOs) had the potential for growth; most were pathologically adenocarcinoma or BAC. By contrast, PNGGOs were stable for several months to years; most were AAH, BAC, or focal interstitial fibrosis.
Tomography, X-Ray Computed/*methods ; Middle Aged ; Male ; Lung Neoplasms/pathology/radiography ; Lung Diseases/pathology/*radiography ; Humans ; Female ; Aged, 80 and over ; Aged ; Adult ; Adenocarcinoma/pathology/radiography

Chronic active Epstein-Barr virus (CAEBV) infection is characterized by persistent infectious mononucleosis-like symptoms, an unusual pattern of Epstein-Barr virus (EBV) antibodies, detection of the EBV genome in affected tissues or peripheral blood, and chronic illness that cannot be attributed to any other known disease. This is the first reported Korean case of an immunocompetent adult with CAEBV-associated interstitial pneumonitis. A 28-year-old female was admitted with a fever that persisted for 3 weeks. She had multiple lymphadenopathy, hepatosplenomegaly, pancytopenia, and elevated serum aminotransferase levels. Serology for antibodies was positive and chest computed tomography showed diffuse ground glass opacities in both lungs. Histopathology of the lung tissue showed lymphocyte infiltration, and EBV DNA was detected in those lymphocytes using in situ hybridization with an EBV-encoded RNA probe. After 1 month of hospitalization, she improved without specific treatment.
Adult ; Chronic Disease ; Epstein-Barr Virus Infections/complications/*pathology/radiography ; Female ; *Herpesvirus 4, Human ; Humans ; Immunocompetence ; Lung Diseases/etiology/pathology ; Lung Diseases, Interstitial/etiology/*pathology/radiography

Congenital pulmonary lymphangiectasia (CPL) is a rare lymphatic pulmonary abnormality. CPL with respiratory distress has a poor prognosis, and is frequently fatal in neonates. We report a case of pneumonectomy for CPL in a newborn. An infant girl, born at 39 weeks' after an uncomplicated pregnancy, exhibited respiratory distress 1 hr after birth, which necessitated intubation and aggressive ventilator care. Right pneumonectomy was performed after her symptoms worsened. Histologic examination indicated CPL. She is currently 12 months old and developing normally. Pneumonectomy can be considered for treating respiratory symptoms for improving chances of survival in cases with unilateral CPL.
Female ; Gestational Age ; Humans ; Infant, Newborn ; Lung/pathology ; Lung Diseases/*congenital/diagnosis/pathology/radiography ; Lymphangiectasis/*congenital/diagnosis/pathology/radiography ; Lymphatic Vessels/pathology ; Tomography, X-Ray Computed

Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder characterized by lymphatic channel proliferation. It is mostly reported in children and young adults. Here, we report a case involving a 52-year-old asymptomatic woman who presented with increased interstitial markings, as seen on a chest radiograph. Diffuse interstitial septal thickening was found on a serial follow-up chest computed tomography scan, and lymphangitic metastasis was the primary radiologic differential diagnosis. However, histologic sections of wedge resected lung revealed diffuse pleural and interlobular septal lymphatic proliferation characteristic of DPL.
Diagnosis, Differential ; Female ; Humans ; Lung Neoplasms/pathology/*radiography ; Lymphangioma/pathology/*radiography ; Middle Aged ; Rare Diseases/pathology/*radiography ; Tomography, X-Ray Computed

This report describes the gross, histological, and immunohistochemical features of medullary thyroid carcinoma (MTC) with pulmonary metastases in a young dog. Sheets of pleomorphic cells supported by fibrous stroma characterized the primary mass, while metastatic nodules had a neuroendocrine pattern. Despite differing histologic features, all masses showed marked immunoreactivity against calcitonin and multiple neuroendocrine markers consistent with MTC. Although MTC is a well-recognized entity, it may be difficult to distinguish this mass from other thyroid neoplasms, necessitating immunohistochemical characterization.
Animals ; Carcinoma, Neuroendocrine/pathology/radiography/*veterinary ; Dog Diseases/*pathology/radiography ; Dogs ; Female ; Laryngeal Neoplasms/secondary/veterinary ; Lung Neoplasms/secondary/veterinary ; Neoplasm Metastasis ; Thyroid Neoplasms/pathology/radiography/*veterinary