Nonspecific interstitial pneumonia-nobody said it's perfect.
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Diagnosis, Differential↗
Biomedical subjects
A G Nicholson
Publications and source records attributed to A G Nicholson.
Adenocarcinoma arising in a retained esophageal remnant.
Fistulation between the trachea and an infected mucocele arising from an excluded retained esophageal segment is an interesting and unusual complication of esophageal bypass surgery. We present such a case in which there was also malignant transformation in the cyst wall, manifested by invasive adenocarcinoma and widespread high-grade glandular dysplasia.
Abscess↗
Thoracic metastasectomy in thyroid malignancies.
BACKGROUND: Relatively little evidence exists to guide the decision pathway regarding thoracic metastasectomy for thyroid malignancy. METHODS: Single-institution 10-year review. RESULTS: Sixteen patients had surgical treatment for intrathoracic metastatic thyroid malignancy: 12 men and 4 women, mean age 43.7 years (range 19 to 77). Histopathologic type was papillary in 6 cases, follicular in 4, Hurthle cell in 3, and medullary in 3. Indication was either "bulky" disease (8 patients) or poor response to radiotherapy (8 patients). We performed 11 sternotomies and five thoracotomies. Operative mortality was 6.25%. Operative morbidity was 6.25%. Mean survival was 39.5 months (0 to 144). Nine patients died during follow-up (mean survival of 41.2 months). Six patients survived, 4 free of disease (mean survival 70 months) and 2 with further relapse (mean survival 17 months). Five-year survival was 32.5%. CONCLUSIONS: The cohort studied is one of the largest in the literature on the topic. Surgical treatment achieved a reasonable survival in a small subgroup of patients where radiotherapy had failed or was deemed inappropriate because of the size or location of the tumor. Further follow-up and more observations will be required for evaluating these preliminary findings.
Adult↗
Concurrent cysts of the mediastinum, pleura and neck.
A 14-year-old male was found to have a mediastinal mass on chest radiograph. Chest computed tomography scans showed a cystic lesion behind the left main bronchus. Magnetic resonance imaging revealed additional cystic lesions in the left chest and root of the neck. He underwent excision of mediastinal mass and a pleural cyst. The neck lesion was presumed to be a cystic hygroma. Histological examination of the two lesions resected showed them to be a foregut cyst and a benign mesothelial cyst. We know of no other report of concurrent multicystic lesions in the chest and neck and hypothesize that these cysts may have a common embryonic origin.
Adolescent↗
Rupture of pulmonary aneurysms in association with long-standing Waterston shunts.
Anastomosis of the ascending aorta to the right pulmonary artery, the so-called Waterston shunt, was undertaken as a palliative procedure for children with cyanotic congenital heart disease due to obstruction of the pulmonary outflow tract with reduced pulmonary blood flow. We present the clinicopathological correlations in two patients who underwent construction of Waterston shunts as neonates, and subsequently died of ruptured pulmonary aneurysms in adult life. Rupture should, therefore, be recognized as a late complication of this procedure, and be considered in the long-term follow-up of such patients, especially when the shunted lung is hypertensive.
Anastomosis, Surgical↗
Reproducibility of the WHO/IASLC grading system for pre-invasive squamous lesions of the bronchus: a study of inter-observer and intra-observer variation.
AIMS: Although many workers have graded pre-invasive squamous lesions arising in the bronchus, there has been no consensus classification system until the latest edition of the WHO/IASLC histological classification of pulmonary and pleural tumours. Because the value of any such system is dependent on its reproducibility, we have circulated a series of such lesions to a panel of histopathologists to assess interobserver and intra-observer variation when the WHO/IASLC classification was applied. METHODS AND RESULTS: Colour transparencies of 28 pre-invasive squamous lesions were assessed by six histopathologists (two with a special interest in pulmonary pathology, two generalists and two trainees) on three separate occasions over a period of 3 months, using the criteria of the WHO/IASLC (mild, moderate and severe dysplasia, and in-situ carcinoma). An additional category of metaplasia was added for those cases that showed no dysplasia. Weighted kappa coefficents of agreement (K(w)) were used to evaluate paired observations with a standard quadratic weighting being employed, such that kappa coefficients corresponded to intra-class correlation coefficients. Wilcoxon's sign-ranked test was used to measure the statistical significance of group trends, when comparing kappa values for the three grading systems. Various 3-point systems were also assessed, through combination of the above groups. Intra-observer agreement was substantially better than interobserver variation (mean: 0.71 vs. 0.55). Between the various pathologist groups, inter-observer variation was relatively minor, although intra-observer variation was higher within the trainee pathologist group. Using weighted kappa values, there was no significant difference in either inter-observer or intra-observer agreement between the five point grading system and a 3-point system of metaplasia/mild, moderate and severe/in-situ grades. However, there was a significant increase in variation when a 3-point system of metaplasia/mild, moderate/severe and in-situ carcinoma was used. CONCLUSION: This study shows levels of interobserver and intra-observer variation similar to those found in other grading systems in histopathology, with no significant decrease in variability found by abridging the system. The WHO/IASLC system is therefore recommended for future use in both clinical and research fields.
Bronchial Neoplasms↗
Desquamative interstitial pneumonia and respiratory bronchiolitis-associated interstitial lung disease.
Our understanding of the various types and patterns of diffuse lung disease that might result in fibrosis has evolved considerably over the last 50 years. Many entities now regarded as distinct had been previously "lumped'' together as a single disease, "lung fibrosis,'' and more recently misdiagnosed as idiopathic pulmonary fibrosis (IPF, synonymous with cryptogenic fibrosing alveolitis). In 1965 desquamative interstitial pneumonia (DIP) was first described, and later it was clearly demonstrated that the clinical and pathological features of DIP and IPF were different, particularly in terms of survival and response to therapy. They are not part of the same disease spectrum nor does DIP evolve into usual interstitial pneumonia (UIP). Later, in the mid-1980s, RBILD was described as a distinct clinicopathologic syndrome with features consistent with an interstitial lung disease among current or former smokers. In the recent histopathological classification of idiopathic interstitial pneumonia (IIP), DIP and RBILD have been included as separate entities, although there is some evidence that suggests they may lie at the two ends of a single spectrum. The debate bears similarities with the debate about DIP and UIP and is as yet unresolved. This article will give a broad and current overview of these two rarer forms of IIP, including issues that relate to diagnosis, imaging, histopathology, treatment, and prognosis.
Journal Article↗
Lymphocytic interstitial pneumonia and other lymphoproliferative disorders in the lung.
Lymphocytic interstitial pneumonia (LIP) is a clinicopathologic term that relates histologically to a dense interstitial infiltrate of mainly T cells, plasma cells, and histiocytes, with germinal centers often identified. Its precise etiology is unknown, but there are strong clinical associations with several autoimmune disorders, as well as both congenital and acquired immunodeficiency syndromes. It may overlap histologically with both extrinsic allergic alveolitis and nonspecific interstitial pneumonia, and therefore close clinical/radiological association is essential for diagnosis. LIP also overlaps clinically and histologically with follicular bronchitis/bronchiolitis, the latter showing reactive lymphoid hyperplasia with a peribronchiolar distribution predominantly comprising lymphoid follicles. LIP may also be histologically indistinguishable from nodular lymphoid hyperplasia and lymphomas arising from mucosa-associated lymphoid tissue (MALT) but can usually be differentiated via analysis of clinical and imaging data plus assessment of immunohistochemistry and gene rearrangement studies. Other entities include lymphomatoid granulomatosis, intravascular lymphomatosis, Castleman's disease, primary pleural lymphomas, primary effusion lymphomas, plasmacytomas, and secondary involvement by lymphoma, but these should all be readily distinguishable from lymphocytic interstitial pneumonia if all clinical, imaging, and histological data are apparent.
Journal Article↗
Epithelial-myoepithelial carcinomas of the bronchus.
Epithelial-myoepithelial carcinomas are very rare in the lung, and little is known about the relationship of their histologic features to prognosis. We describe five primary pulmonary epithelial-myoepithelial carcinomas with details on clinical presentation, histology, and immunohistochemical profiles. We also reviewed the literature to detail further their prognosis. The patients' ages ranged from 33 to 57 years (average 51 years). The tumors were all endobronchial and the patients presented with symptoms or imaging features of airway obstruction. The tumors were completely resected; none showed nodal involvement. All five patients are alive and free of disease 4 months to 8 years (average 4.2 years) after surgery. Four tumors showed a mixed pattern of glands lined by a dual layer of cells and solid sheets of either spindle cells or clear cells, the glandular and solid components being present in variable proportions. The fifth tumor comprised purely spindle cells. The mitotic rate was <1/20 high power fields in both the glandular and spindle/clear cell components. In one case there was focal nuclear pleomorphism. The inner layer of the glands stained for cytokeratins and epithelial membrane antigen, and the outer layer for S-100 and smooth muscle actin. In one case the spindle cells stained for CD34. A review of published cases shows the majority of tumors behave in an indolent fashion, the rare aggressive tumors being predominantly myoepitheliomatous. Nevertheless, the term epithelial-myoepithelial carcinoma is preferred because of their malignant potential. A high mitotic rate, tumoral necrosis, and nuclear pleomorphism appear to be adverse prognostic factors.
Actins↗
Lung fibrosis: new classifications and therapy.
The recent American Thoracic Society/European Respiratory Society consensus classification of idiopathic interstitial pneumonia is equally applicable to pulmonary fibrosis associated with connective tissue disease. The most frequent histopathologic entities are usual interstitial pneumonia (UIP) and nonspecific interstitial pneumonia (NSIP), which is more prevalent than UIP in systemic sclerosis. The prognostic significance of NSIP is unknown in connective tissue disease, although NSIP has a better prognosis than UIP in idiopathic interstitial pneumonia. The use of computed tomography to distinguish between UIP and NSIP requires further refinement. Recent therapeutic studies have reinforced disenchantment amongst clinicians with corticosteroid and immunosuppressive regimens in UIP. UIP is increasingly regarded an "epithelial-fibrotic" disease rather than a primarily inflammatory disorder, accounting for recent widespread interest in antifibrotic agents. This conclusion should not be extrapolated to NSIP, especially in connective tissue disease. Strong circumstantial evidence of a therapeutic benefit justifies the continued use of cyclophosphamide in progressive lung fibrosis in systemic sclerosis.
Connective Tissue Diseases↗
Methodology for assessing patterns of interstitial pneumonia in children.
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Biopsy↗
Intramural neurofibroma of the trachea treated by multiple stents.
The case history is presented of a patient in whom an intramural tracheal neurofibroma developed, causing severe airway stenosis. The patient was treated with multiple stents over a period of 5 years because of progression of the disease and associated airflow limitation. Clinicians should be aware of this rare complication of neurofibromatosis.
Bronchoscopy↗
Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparative appearances at and diagnostic accuracy of thin-section CT.
PURPOSE: To compare the morphologic abnormalities on thin-section computed tomographic (CT) images in a group of patients with histopathologically confirmed nonspecific interstitial pneumonia (NSIP) or usual interstitial pneumonia (UIP) and a clinical presentation of idiopathic pulmonary fibrosis. MATERIALS AND METHODS: Thin-section CT imaging patterns and distribution of disease in 53 patients with histologic diagnoses of NSIP (n = 21) or UIP (n = 32) were quantified retrospectively and independently by four observers. The appearances of NSIP and UIP at CT were compared with univariate and multivariate techniques. RESULTS: The use of thin-section CT proved to have moderate sensitivity (70%), specificity (63%), and accuracy (66%) in the diagnosis of NSIP. An increased proportion of ground-glass attenuation was the cardinal feature of NSIP at CT (odds ratio: 1.04 for each 1% increase in the proportion of ground-glass attenuation). A histologic diagnosis of NSIP was most frequent (in 24 of 35 observations [69%]) when ground-glass attenuation predominated, and was more frequent with mixed (35 of 79 observations [44%]) than with predominantly reticular disease (25 of 98 [26%] observations, P < .005). Logistic regression analysis of the data indicated that misdiagnosis of UIP in patients with NSIP was associated with less ground-glass attenuation (P < .005) at CT and a subpleural disease distribution (P = .02), with the converse being true for UIP cases misdiagnosed as NSIP. CONCLUSION: In patients with a clinical presentation of idiopathic pulmonary fibrosis, the accuracy of thin-section CT in identifying NSIP is considerably higher than previously reported. At CT, NSIP is characterized by more ground-glass attenuation and a finer reticular pattern than is UIP. Nevertheless, considerable overlap in thin-section CT patterns exists between NSIP and UIP.
Biopsy↗
Pulmonary MALT lymphoma: imaging findings in 24 cases.
The aim of this study was to describe the imaging features of pulmonary mucosa-associated lymphoid tissue (MALT) lymphoma. The chest radiographs (n = 18) and CT scans (n = 17) of 24 patients (18 men and 6 women) aged 27-78 years (mean = 56 years), with a known diagnosis of pulmonary MALT lymphoma, were retrospectively reviewed by two radiologists and the imaging findings are described. Six of the 24 patients had a history of an autoimmune disorder and 1 patient had acquired immune deficiency syndrome. Multiple pulmonary lesions were identified in 19 of 24 patients (79%) and solitary lesions in 4 of 24 patients (17%). Diffuse pulmonary infiltration was present in 1 patient. Lesions included masses or mass-like areas of consolidation (n = 21) and pulmonary nodules (n = 18). Associated findings were air bronchograms, airway dilatation, a positive angiogram sign and a halo of ground-glass shadowing at lesion margins. Peribronchovascular thickening was also observed, as were hilar or mediastinal lymph node enlargement and pleural effusions or thickening. Although rare, the diagnosis of pulmonary MALT lymphoma should be considered in patients with the imaging features described, particularly when in association with an indolent clinical course or a history of autoimmune disease.
Adult↗
Extramedullary plasmacytoma presenting with myasthenia gravis and mediastinal mass.
A 68-year-old woman presented with myasthenia gravis and an anterior mediastinal mass, which proved to be an extramedullary plasmacytoma in the thymus with associated extracellular light chain deposition. Further specimens revealed plasma cell proliferation in an internal thoracic lymph node and in a subsequent colectomy specimen, indicating systemic disease. This case demonstrates the rare association between a plasmacytoma in the thymus and myasthenia gravis.
Aged↗
The feasibility of conservative resection for carcinoid tumours: is pneumonectomy ever necessary for uncomplicated cases?
OBJECTIVE: To assess the feasibility of a policy of conservative resection for carcinoid tumours, to validate this by a study of recurrence rates and survival, and to assess those factors that might frustrate such a policy. METHODS: Over 20 years, 95 patients with a final histological diagnosis of carcinoid tumour were assessed for surgery. During this time we had a policy of conservative resection for such tumours, with preservation of functional lung parenchyma wherever possible. RESULTS: The mean age at presentation was 51 years (range 14-81). Symptoms were present in 62 patients (65.3%). Fifty-eight tumours (61.1%) were central in position. Surgical resection was performed in 92 patients. Three patients (3.3%) underwent pneumonectomy: in two because the situation was complicated by destruction or severe damage to the distal lung parenchyma, and in one the initial biopsy was interpreted as lung cancer. Less than 50% of patients were referred with the correct histological diagnosis. In 18% preoperative biopsies were interpreted as non-small cell lung cancer (NSCLC). At thoracotomy similar confusion remained with 26% of frozen section reports suggesting NSCLC. In those patients coming to thoracotomy, lymph node involvement was present in 15 patients (16.3%) (N1 in 13 patients, N2 in two patients) being found in 11 of 81 (13.6%) patients whose tumours showed typical histological features, and four of the 14 patients whose tumours (28.5%) displayed atypical features. During follow-up from 6 months to 12 years (mean 3.9 years) four of the 92 operated patients were found to have local recurrence and underwent further surgery and three others developed distant metastases (3.2%), two of whom have died. CONCLUSIONS: Bronchial carcinoid is now considered to be a low-grade, but malignant tumour. Despite this we have found over the last 20 years that a policy of conservative resection is feasible and safe whenever the true histology is known and the distal lung parenchyma is functional. This is not affected by the presence of nodal involvement or atypical features and the long-term results of conservative resection are not affected by the presence of nodal disease. These factors should not influence the extent of surgical resection.
Adolescent↗
Value of the mesothelium-associated antibodies thrombomodulin, cytokeratin 5/6, calretinin, and CD44H in distinguishing epithelioid pleural mesothelioma from adenocarcinoma metastatic to the pleura.
Until recently, the standard approach of most laboratories in distinguishing epithelioid pleural mesothelioma from metastatic adenocarcinoma has been a negative result from a panel of adenocarcinoma-associated antibodies. However, several "mesothelium-associated" antibodies have been proposed as useful in this situation, and we have applied four of these putative mesothelioma markers--thrombomodulin, cytokeratin 5/6, calretinin, and CD44H--to a series of 61 epithelioid pleural mesotheliomas and 63 metastatic adenocarcinomas with known primary sites (lung = 19; breast = 21; ovary = 6; colon = 10; kidney = 4; uterus, epididymis, pancreas = 1 case each). Of the mesotheliomas, 55 of 61 (90%) stained for thrombomodulin, 56 of 61 (92%) for cytokeratin 5/6, 47 of 51 cases (92%) were positive for calretinin, and 39 of 43 (91%) were positive for CD44H. Of the metastatic adenocarcinomas, 12 of 63 (19%) cases were positive for thrombomodulin, 9 of 63 (14%) were positive for CK5/6, and 27 of 60 (45%) were positive for CD44H. With calretinin, only 1 case of 59 (2%) showed positive nuclear staining. All four antibodies stained reactive mesothelium; thrombomodulin also stained endothelium; and CD44H variably stained lymphocytes, macrophages, and fibroblasts. We conclude that all four antibodies show high sensitivity for epithelioid mesothelioma, but only calretinin (98%), cytokeratin 5/6 (86%), and thrombomodulin (81%) show sufficient specificity for practical use in this situation.
Adenocarcinoma↗
Calcifying fibrous pseudotumour arising within an inflammatory pseudotumour: evidence of progression from one lesion to the other?
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Actins↗