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Presented by Fred Askin, M.D. and prepared by Carol Allan, M.D.
Case 6: The patient was a 51 year old woman with a history of increasing dyspnea and lower lung zone radiographic infiltrates.
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1. Question
Week 54: Case 6
The patient was a 51 year old woman with a history of increasing dyspnea and lower lung zone radiographic infiltrates./images/fa6a.jpg
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/images/fa6d.jpgCorrect
Answer: Metastatic carcinoma
Histology: See “Discussion” section below.
Discussion: This is a very unusual case but one that illustrates several important features:
(1) the necessity of distinguishing between primary and secondary bronchiolitis obliterans organizing pneumonia, and
(2) that not all fibrotic interstitial lung disease is usual interstitial pneumonitis.
Histologic variability of the processes in this biopsy are perhaps the best clue to the diagnosis of something unusual. There are clear-cut areas of bronchiolitis obliterans organizing pneumonia and in other areas there are clear-cut areas of interstitial fibrosis, often with a subpleural accentuation. Although its not particularly unusual to find combinations of interstitial pneumonitis and fibrosis with BOOP, the inability to specifically place this lesion in one category or another should be a warning to look for other, less usual, diagnoses. Careful inspection of the interstitium and the perivascular areas show lymphangitic and interstitial collections of large atypical cells. Although not shown, abnormal mitotic figures were also seen. These cells showed positive staining with several immunocytochemical cytokeratin cocktails.
In making the diagnosis of BOOP, one should always be alert to the possibility of BOOP as a secondary or reactive component of some other specific disorder. In this case, the BOOP is associated with metastatic carcinoma. Other disorders in which BOOP can be a prominent component include extrinsic allergic alveolitis and pulmonary eosinophilic granuloma (Langerhans’ cell histiocytosis) among many others. It is, therefore, risky to make a specific diagnosis of BOOP on the basis of a transbronchial biopsy. The combination of BOOP and interstitial inflammation and fibrosis may be seen in a non-specific interstitial pneumonitis as described by Katzenstein, however, the lack of an inflammatory interstitial component and the presence of relatively “young” collagen in this case argues against that diagnosis. On the other hand, the presence of a relatively immature collagen in the interstitium should suggest the diagnosis of organizing diffuse alveolar damage, however, the absence of hyaline membranes or intraalveolar fibrin or of a more prominent intraalveolar fibroblastic component argues against that diagnosis. Basically the unusual histologic picture here is a very fibroblastic form of lymphangytic pulmonary carcinomatosis.
Incorrect
Answer: Metastatic carcinoma
Histology: See “Discussion” section below.
Discussion: This is a very unusual case but one that illustrates several important features:
(1) the necessity of distinguishing between primary and secondary bronchiolitis obliterans organizing pneumonia, and
(2) that not all fibrotic interstitial lung disease is usual interstitial pneumonitis.
Histologic variability of the processes in this biopsy are perhaps the best clue to the diagnosis of something unusual. There are clear-cut areas of bronchiolitis obliterans organizing pneumonia and in other areas there are clear-cut areas of interstitial fibrosis, often with a subpleural accentuation. Although its not particularly unusual to find combinations of interstitial pneumonitis and fibrosis with BOOP, the inability to specifically place this lesion in one category or another should be a warning to look for other, less usual, diagnoses. Careful inspection of the interstitium and the perivascular areas show lymphangitic and interstitial collections of large atypical cells. Although not shown, abnormal mitotic figures were also seen. These cells showed positive staining with several immunocytochemical cytokeratin cocktails.
In making the diagnosis of BOOP, one should always be alert to the possibility of BOOP as a secondary or reactive component of some other specific disorder. In this case, the BOOP is associated with metastatic carcinoma. Other disorders in which BOOP can be a prominent component include extrinsic allergic alveolitis and pulmonary eosinophilic granuloma (Langerhans’ cell histiocytosis) among many others. It is, therefore, risky to make a specific diagnosis of BOOP on the basis of a transbronchial biopsy. The combination of BOOP and interstitial inflammation and fibrosis may be seen in a non-specific interstitial pneumonitis as described by Katzenstein, however, the lack of an inflammatory interstitial component and the presence of relatively “young” collagen in this case argues against that diagnosis. On the other hand, the presence of a relatively immature collagen in the interstitium should suggest the diagnosis of organizing diffuse alveolar damage, however, the absence of hyaline membranes or intraalveolar fibrin or of a more prominent intraalveolar fibroblastic component argues against that diagnosis. Basically the unusual histologic picture here is a very fibroblastic form of lymphangytic pulmonary carcinomatosis.
