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Presented by Fred Askin, M.D. and prepared by Orin Buetens, M.D.
Case 4: The patient is a 53-year-old man with clinical complaints of shortness of breath.
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Week 18: Case 4
The patient is a 53-year-old man with clinical complaints of shortness of breath. A chest radiograph reveals multiple small nodules scattered throughout the upper two-thirds of the lung with relative sparing of the costophrenic angles. The patient has a long history of cigarette smoking./images/45708a.jpg
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Answer: Pulmonary eosinophilic granuloma (Langerhans’ cell histiocytosis)
Histology: The lesion is characterized by the presence of discrete interstitial stellate foci composed of large numbers of bland cells with eosinophilic cytoplasm and a large nucleus often with a prominent groove or fold. Eosinophils are scattered throughout the lesion but are not a prominent feature. The cells with folded, vesicular nuclei are positive with immunostains directed against S100 protein and CD1A.
Discussion: Pulmonary eosinophilic granuloma (Langerhans’ cell histiocytosis) is an exquisitely interstitial process. The interstitial pattern of growth along with fibrosis leads to the appearance of stellate or starfish shaped scars in the lung. The radiographic and especially CT scan images of pulmonary eosinophilic granuloma are relatively characteristic, emphasizing the importance of correlating histologic and radiographic findings. The proliferating cell in pulmonary eosinophilic granuloma (Langerhans’ cell histiocytosis) is a cell of the macrophage monocyte series related to the interdigitating reticulum cell, as demonstrated by S100 and CD1A immunoreactivity. The etiology of pulmonary eosinophilic granuloma is not clear, however, many if not all of the reported patients are cigarette smokers. Patients with pulmonary eosinophilic granuloma tend to be young adults and there is a female predominance of disease. Cells involved appear to be similar if not identical to those involved in systemic Histiocytosis X in children. Adults, however, who present with disease in the lung almost never develop disseminated disease, although a small number of these patients may have a solitary bone lesion or, rarely, diabetes insipidus related to pituitary involvement. Late stage of disease leads to a peculiar type of honeycomb change in the lung basically related to scar emphysema around the stellate interstitial areas of fibrosis. Pulmonary eosinophilic granuloma is, therefore, one of the few interstitial lung diseases that can present with large lungs rather than with small fibrotic lungs.
The diagnosis in this case was made by open lung biopsy but transthoracic needle biopsy or transbronchial biopsies may also be effective. The random nature and small size of the lesions and the difficulty of appreciating an interstitial growth pattern in small biopsies makes the diagnosis difficult on small biopsies and a high index of suspicion is necessary. The use of appropriate immunostaining along with radiologic correlation can be helpful.
Incorrect
Answer: Pulmonary eosinophilic granuloma (Langerhans’ cell histiocytosis)
Histology: The lesion is characterized by the presence of discrete interstitial stellate foci composed of large numbers of bland cells with eosinophilic cytoplasm and a large nucleus often with a prominent groove or fold. Eosinophils are scattered throughout the lesion but are not a prominent feature. The cells with folded, vesicular nuclei are positive with immunostains directed against S100 protein and CD1A.
Discussion: Pulmonary eosinophilic granuloma (Langerhans’ cell histiocytosis) is an exquisitely interstitial process. The interstitial pattern of growth along with fibrosis leads to the appearance of stellate or starfish shaped scars in the lung. The radiographic and especially CT scan images of pulmonary eosinophilic granuloma are relatively characteristic, emphasizing the importance of correlating histologic and radiographic findings. The proliferating cell in pulmonary eosinophilic granuloma (Langerhans’ cell histiocytosis) is a cell of the macrophage monocyte series related to the interdigitating reticulum cell, as demonstrated by S100 and CD1A immunoreactivity. The etiology of pulmonary eosinophilic granuloma is not clear, however, many if not all of the reported patients are cigarette smokers. Patients with pulmonary eosinophilic granuloma tend to be young adults and there is a female predominance of disease. Cells involved appear to be similar if not identical to those involved in systemic Histiocytosis X in children. Adults, however, who present with disease in the lung almost never develop disseminated disease, although a small number of these patients may have a solitary bone lesion or, rarely, diabetes insipidus related to pituitary involvement. Late stage of disease leads to a peculiar type of honeycomb change in the lung basically related to scar emphysema around the stellate interstitial areas of fibrosis. Pulmonary eosinophilic granuloma is, therefore, one of the few interstitial lung diseases that can present with large lungs rather than with small fibrotic lungs.
The diagnosis in this case was made by open lung biopsy but transthoracic needle biopsy or transbronchial biopsies may also be effective. The random nature and small size of the lesions and the difficulty of appreciating an interstitial growth pattern in small biopsies makes the diagnosis difficult on small biopsies and a high index of suspicion is necessary. The use of appropriate immunostaining along with radiologic correlation can be helpful.
