Specific Discussion

98-100. The answers are 98-a, 99-c, 100-d. The question concerns primarily the chest radiograph abnormality, which is an incidental finding in this elderly patient with urosepsis. The CXR is consistent with subster-nal goiter. These are usually benign, and only infrequently large enough to significantly compress the upper airway. However, the extent of tracheal deviation in this patient requires further evaluation via CT scan. A flow volume loop would be helpful to rule out extrathoracic obstruction and potential for stridor and respiratory distress. The clinical feature and the bilateral smooth contour of the opacity make the other options less likely.

101-102. The answers are 101-b, 102-c. The patient has an anterior mediastinal mass, which is a thymoma. Thymoma usually occurs at the level of or just superior to the hilum. There are four well-established para-neoplastic or clinical syndromes associated with thymoma. (1) Bronchiec-tasis results from repeated infections from acquired hypoglobulinemia occurring in 10% of patients with thymoma. (2) Primary red cell aplasia occurs in 5% of the patients with thymoma with normocytic, normo-chromic anemia. Of all patients with primary red cell aplasia, 50% will have accompanying thymoma. Thymectomy will induce remission. (3) Myasthenia gravis resulting in muscle weakness is due to antibodies directed at the postsynaptic acetylcholine receptors. Up to 40% of patients with thymoma have myasthenia gravis, but thymectomy rarely alters the clinical course. (4) Extrathymic malignancies, lymphoma, thyroid cancer, and lung cancer occur in 20% of thymoma patients. Eaton-Lambert syndrome is a rare paraneoplastic neuromuscular defect in which the auto-antibodies are directed against P/Q-type voltage-gated calcium channels called VGCCs. The presence of autoantibodies blocks calcium influx into the nerves. It is associated with certain malignancies, especially small cell lung cancer. The Tensilon test is not always definitive in patients with mysasthenia gravis; assessment for antibodies to acetylcholine receptors, detected in 90% of patients with myasthenia gravis, is the test of choice. Hepatitis and aspiration pneumonia are not associated with thymoma.

103. The answer is c. The patient is from an endemic area where histoplasmosis is prevalent. The clinical symptoms with erythema nodosum are consistent with this diagnosis. Serum ACE level is typically increased in sarcoidosis but can also be increased in histoplasmosis. The chest x-ray showing "eggshell" calcifications is seen in granulomatous diseases, sar-coidosis, and silicosis. As there is no history of exposure to silica or silica-related occupational hazard, option c is the best answer.

104-105. The answers are 104-a, 105-b. Bilateral hilar lymph-adenopathy (BHL) in a young African American female with non-specific symptoms is suggestive of sarcoidosis. Generally the other causes of BHL include granulomatous diseases, carcinomatosis, and lymphomatosis. Bronchiolitis obliterans (BO) is defined pathologically as injury to small airways with granulation tissue reaction and repair resulting in obliterative bron-chiolar scarring. When this process extends into the alveolar ducts, it is termed organizing pneumonia and the entity is called bronchiolitis obliterans with organizing pneumonia (BOOP). It is usually secondary to a prolonged viral or infectious illness, inhalational or toxic exposure, chronic antigenic insult, or connective tissue disorders such as rheumatoid arthritis and presents with persistent infiltrates on a chest x-ray. It can lead to pulmonary fibrosis. The clinical scenario and CXR are not consistent with this diagnosis in this patient. Hypersensitivity pneumonitis is generally secondary to an inhalational exposure causing pulmonary infiltrates. Sarcoidosis is not associated with clubbing or osteoporosis. It causes bone cyst formations and deformities and leads to reduced diffusing capacity on pulmonary function tests.

106-107. The answers are 106-b, 107-c. The symptoms described in this patient and the characteristic CXR are consistent with pulmonary hypertension. This could be further worked up and confirmed by an echocardiogram to estimate PA pressures. Chronic bronchitis is unlikely in this nonsmoker, and deconditioning would not give the physical signs observed. There is no clinical or radiographic evidence of mitral stenosis. PFT and exercise test would be abnormal but nonspecific, and aggressive exercise programs would be contraindicated.

108-109. The answers are 108-d, 109-a. Chest symptoms in an uncontrolled hypertensive with end organ damage are suggestive of either an acute coronary event or aortic dissection. The ECG does not show any acute ischemia or injury pattern. CXR reveals a double shadow in the region of the aortic arch, suggesting a dissection of undetermined age and/or an aneurysm. An ultrasound of the chest would be unhelpful; repeat chest x-ray would be redundant and the lordotic view is only helpful for evaluating apical pulmonary disease. A renal scan would probably only confirm the renal insufficiency as depicted by the increased creatinine. A CT scan would be most helpful to confirm this diagnosis, but has some risk in a patient with azotemia.

110. The answer is c. This large posterior mediastinal mass is an esophageal lesion, and the presence of steady, constant, boring pain is indicative of mediastinal involvement and therefore inoperability. Cough may be due to aspiration or laryngeal reflux; clubbing can at times be seen in both benign and malignant lesions of the esophagus and does not indicate mediastinal involvement or inoperability.

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