Bronchiolitis obliterans-organizing pneumonia: an Italian experience.

The purpose of this study was to describe the clinical features at onset and outcome and the diagnostic approach in subjects with bronchiolitis obliterans-organizing pneumonia (BOOP). Over a 7-year period we observed 78 cases of biopsy-proven bronchiolitis obliterans-organizing pneumonia, in which well documented clinical and radiographic data were available. The final diagnosis of BOOP was validated when patients presented: (i) negative microbiological analysis on BAL fluid; (ii) a well documented improvement either spontaneous, or after steroid treatment or (iii) cases with progressive respiratory failure and increasing radiographic shadows, an open lung biopsy or autopsy that excluded other entities. There were 42 males and 36 females; the mean age was 61+/-12 years (range 12-85 years). Forty-two (54%) patients were current smokers, 25 (32%) had never smoked and 11 (14%) were ex-smokers. The clinical pattern at presentation of BOOP was more frequently similar to classical acute or sub-acute infectious pneumonia. Fever (63%), dyspnoea (58%) and dry cough (53%) were the typical symptoms on admission. A flu-like syndrome preceeding BOOP was observed in 21 cases (27%). Inspiratory crackles (78%) were the most typical finding at physical examination. However, 13% of the patients were asymptomatic and an abnormal chest X-ray film was the reason for seeking medical attention. Radiographically the most frequent pattern of BOOP was a unilateral consolidation (44%) with lower field predominance. A migratory behaviour was present in 22% of the cases. High-resolution computed tomographic (HR-CT) scan when performed, was more sensitive in detecting ground glass infiltrates, sub-pleural or peri-bronchovascular distribution or the presence of nodules or cavitation. Most patients (68%) were classified as having idiopathic BOOP. However, the same clinical-roentgenological pattern was observed in patients after radiotherapy for ductal breast carcinoma (6%), in collagen-vascular diseases (6%), related to drugs (9%), to infections serologically documented (4%), and to graft vs. host disease (4%). Four patients (all of whom had idiopathic BOOP) presented a rapid progressive respiratory failure needing mechanical ventilation. In another two cases respiratory failure appeared after a long period during which patients experienced exertional dyspnoea and low grade fever. BAL profile was characterized by lymphocytosis with a reduction of the CD4/CD8 ratio, associated with a slight increase of neutrophils and eosinophils and scattered mast cells. However in two cases we had an increased CD4/CD8 ratio and in one case the presence of a significant 12% of polyclonal B cells. In a few cases atypical (cytokeratin-positive cells) epithelial cells were detected: these cells were constantly present in the BAL fluid of patients with rapidly progressive respiratory failure. From the diagnostic point of view this series documents that transbronchial lung biopsy (coupled with BAL) can be the first diagnostic step. However, therapy can be started on the basis of BAL data (when a characteristic morphological and phenotypical profile is evident) in cases in which the clinical presentation is suggestive and a biopsy cannot be made. Most patients showed a rapid and good response to steroid therapy. However, three patients died (4%) in spite of steroid therapy (two cases) and steroid and cyclophosphamide therapy (one case). In conclusion, although clinical findings, chest X-ray film and CT Scan findings usually suggest the diagnosis a definite confirmation requires transbronchial lung biopsy and BAL and, less frequently, open lung biopsy.

Bronchoalveolar lavage and transbronchial lung biopsy in alveolar and/or ground-glass opacification.

In order to assess the diagnostic yield of bronchoalveolar lavage (BAL) and transbronchial lung biopsy (TBB) in pulmonary diseases with a ground-glass and/or alveolar pattern on high-resolution computed tomography (HRCT) scan, a prospective study was undertaken. Thirty-six patients (17 males, 19 females), mean age 53 yrs, selected on the basis of the presence of an alveolar and/or ground-glass pattern on chest HRCT scan, were submitted to fibreoptic bronchoscopy. All patients underwent BAL. TBBs were performed in 33 cases. A specific diagnosis was achieved, taking into account data obtained by means of serology, microbiology, cytology and histopathology in appropriate clinical settings. Twelve (33%) patients only had the appearance of a ground-glass opacity, whereas 24 (67%) had associated areas of airspace consolidation. BAL was performed in all cases and gave a definitive diagnosis in 21 (58%). The diagnostic yield of BAL in patients with only ground-glass opacities was no different from that in those patients also showing areas of alveolar consolidation (58 versus 58%). In eight patients (six with ground-glass opacity and two with alveolar consolidation), BAL provided useful but not definitive information. In these patients, a definitive diagnosis was achieved by means of TBB in seven cases and by open lung biopsy in one case. TBB was performed in 33 out of 36 patients and gave positive results in 25 (76%). The diagnostic yield of TBB in patients showing areas of alveolar consolidation was significantly higher than in those with pure ground-glass opacity, i.e. 95% (21 of 22) and 36% (4 of 11) respectively (p < 0.001). BAL and TBB were performed during the same bronchoscopy in 33 patients, and an accurate diagnosis was achieved in 30 (91%). Overall, the diagnostic yield of TBB (76%) and BAL (56%) did not differ significantly in the whole patient group (p = 0.12), or in patients with a ground-glass opacification (58 versus 36%, p = 0.3). However, in patients with areas of alveolar consolidation, the diagnostic sensitivity of TBB (95%) was significantly greater than the diagnostic sensitivity of BAL (54%) (p = 0.03). In conclusion this study shows that high-resolution computed tomography can be helpful in predicting the diagnostic accuracy of bronchological procedures, in particular of bronchoalveolar lavage and transbronchial biopsy, and that alveolar and/or ground-glass are favourable patterns for these diagnostic tools.

Core needle biopsy is effective in the initial diagnosis of mediastinal lymphoma.

BACKGROUND AND OBJECTIVE: With the development and refinement of guidance modalities for percutaneous biopsies, many investigators have reported studies supporting the role of guided core needle biopsy in the diagnosis of mediastinal lymphoma. The aims of this report are to evaluate the efficacy of findings at core needle biopsy of mediastinal masses on patient care and define the key determinants of clinical success. DESIGN AND METHODS: Fluoroscopy-guided (in 75 patients) and computed tomography-guided (in 8 patients) core needle biopsies were performed in 83 patients with mediastinal lymphoma: all but one of the patients were at first diagnosis. All the biopsies were performed using a Menghini needle (from 1.2 mm to 1.8 mm). In the vast majority of cases the 1.8 mm gauge was employed. RESULTS: The overall sensitivity for the diagnosis of lymphoma was 81% (67/83 cases). In the remaining 16 patients the lymphoma diagnosis was reached either by mediastinoscopy (11 cases) or anterior mediastinotomy (3 cases) or core needle biopsy of the lung (1 case); one patient was treated directly after the needle biopsy had been unsuccessful because he needed rapid therapy. In 77/82 (93%) patients it was possible to assess the specific histotype. There was no operative mortality; all the biopsies were performed on an outpatient basis. INTERPRETATION AND CONCLUSIONS: Our data indicate that core needle biopsy should be considered as an effective and safe procedure in the diagnosis of patients with mediastinal lymphoma with the possibility of determining the tumor subtype and subsequent specific treatment.

Epithelioid haemangioendothelioma of the lung imitating clinical features of pulmonary histiocytosis X.

A young male who was a heavy smoker presented with spontaneous right pneumothorax. A high resolution computed tomography scan showed disseminated nodules up to 1 cm in diameter; the greatest majority of which were sited in the centrilobular zone, though some abutted on the pleural surface. Surgical lung biopsies allowed a diagnosis of epithelioid haemangioendothelioma. The neoplastic tissue infiltrated the wall of bronchioles, partially obliterating them and the visceral pleura. These two histological aspects could be considered as concomitant mechanisms for the appearance of spontaneous pneumothorax. Epithelioid haemangioendothelioma should be added to the list of lung diseases in young heavy smokers that can begin with a spontaneous pneumothorax.

Bronchoalveolar lavage, histological and immunohistochemical features in cryptogenic organizing pneumonia.

This study describes bronchoalveolar lavage (BAL), histological and immunohistochemical features in a series of 10 patients with cryptogenic organizing pneumonia (COP). The histological diagnosis was performed by transbronchial biopsy in seven cases and by open lung biopsy in three cases. All patients showed a marked increase in lymphocytes and a mild increase in neutrophils and eosinophils in BAL fluid. The number of T-lymphocytes expressing human leucocyte antigen-DR (HLA-DR) surface antigen was increased (p < 0.002). The majority of lymphocytes expressed the CD8 phenotype, so that the CD4/CD8 ratio was markedly decreased. Masson bodies were present in the lung specimens of all patients. Most of the epithelial cells surrounding the Masson bodies were immunoreactive with an anti-granulocyte/macrophage colony-stimulating factor (GM-CSF) monoclonal antibody. The great majority of mononuclear cells in the lung specimens showed immunoreactivity with anti-CD3, anti-CD8 and anti-CD45R0 monoclonal antibodies. In the Masson bodies, spindle cells were immunoreactive with anti-alpha smooth muscle (alpha-sm) actin monoclonal antibody. Glucocorticoid treatment (the therapy of choice in COP) downregulated GM-CSF messenger ribonucleic acid (mRNA) expression in lung epithelial cell lines. These findings indicate that the combination of bronchoalveolar lavage cell profile with histological evidence is a valuable means of corroborating a clinical diagnosis of cryptogenic organizing pneumonia, and that granulocyte/macrophage colony-stimulating factor may be one of the cytokines involved in the pathogenesis.

Bronchoalveolar lavage in the diagnosis of low-grade, MALT type, B-cell lymphoma in the lung.

Low-grade B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) type represents one of the most frequent primary lymphomas in the lung. This study demonstrates the value of bronchoalveolar lavage (BAL) in the diagnosis of this entity. Three patients with biopsy proven MALT lymphoma in the lung were submitted to bronchoscopy. BAL fluid analysis showed an increased cellularity and a striking lymphocytosis. Cytological features consistent with a low-grade malignant lymphoma were present in two cases (medium sized lymphoid cells with an evident lymphoplasmocytoid differentiation, and irregular nuclear borders). In all cases, flow cytometry analysis showed a high percentage of cells expressing a B phenotype (52, 40 and 28%, respectively). In two cases, there was a striking monotypic expression of surface light-chain immunoglobulin (Kappa/Lambda ratio = 38 and 0.04, respectively). Bronchoalveolar lavage appears to be a valuable procedure in the diagnosis of low-grade B-cell lymphoma of MALT type in the lung.

Gastric carcinoids and their precursor lesions. A histologic and immunohistochemical study of 23 cases.

A histologic and immunohistochemical study was carried out in 23 unselected nonantral gastric carcinoids and their precursor lesions classified according to Solcia et al. None of the patients showed Zollinger-Ellison syndrome. Two variants of carcinoids showing distinctive pathologic and pathogenetic characteristics were identified on the basis of presence or absence of associated chronic atrophic gastritis type A (A-CAG). Chronic atrophic gastritis type A was found in 19 cases showing either single or multiple neoplasms, tumor extension limited to the mucosa or submucosa, consistent endocrine cell precursor changes in extratumoral mucosa, and consistent hypergastrinemia and/or G cell hyperplasia. Associated precursor lesions were only hyperplastic in all but two cases with single carcinoids whereas they were also dysplastic in all but one case with multiple carcinoids. The four tumors arising in nonatrophic mucosa were all single, more aggressive, and not associated with extratumoral endocrine cell proliferations or with signs of gastrin hypersecretion. Tumor cells were diffusely immunoreactive for chromogranin A and synaptophysin but usually negative for chromogranin B or HISL-19. Scattered serotonin cells were found in ten carcinoids. They were more frequent in infiltrating than in intramucosal tumors as were the less represented pancreatic polypeptide cells whereas the reverse was found for alpha-subunit-containing cells. These results are of relevance for tumor pathogenesis and may provide the rationale for a less aggressive therapeutic approach in the patients.

Inflammatory pseudotumor of the liver: report of two cases.

Inflammatory pseudotumor of the liver is a rare benign lesion which clinically and grossly is often interpreted as a neoplastic condition. It usually arises in children or young adults. Only 11 cases have been described in the literature, and its etiology is still uncertain. Two new cases of inflammatory pseudotumor in adult male patients are reported here. Morphologically, these cases summarize all the features seen in the other cases described previously. They showed fibrous bands delimiting microabscesses made up of granulocytes, mononuclear elements and foamy macrophages. These lesions have in common the symptomatology which is amenable to infectious disease. The purpose of this paper is to draw attention to the existence of a lesion that clinically simulates a neoplastic condition of the liver. This lesion should be histologically recognized to avoid excessive surgical treatment.