Ann Thorac Surg 2000;69:944-946
© 2000 The Society of Thoracic Surgeons
Case Reports
Mediastinal amyloidosis
John D. Urschel, MDa,
Dorothy M. Urschel, MSa
a Department of Thoracic Surgical Oncology, Roswell Park Cancer Institute, Buffalo, New York, USA
Address reprint requests to Dr Urschel, Department of Surgery, St. Joseph’s Hospital, 50 Charlton Ave E, Hamilton, ON, Canada L8N 4A6
e-mail: urschelj{at}fhs.mcmaster.ca
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Abstract
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Systemic amyloidosis is an unusual cause of mediastinal lymphadenopathy. Thoracic surgeons are often called upon to establish a diagnosis in patients with mediastinal lymphadenopathy, so familiarity with mediastinal amyloidosis is valuable. We report our experience with 2 patients, and discuss the diagnostic role of mediastinoscopy and other less invasive biopsy techniques. General anesthesia may pose significant risks in this disease; nonoperative biopsy techniques should be considered if the diagnosis of amyloidosis is suspected.
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Introduction
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Mediastinal lymphadenopathy is a common diagnostic problem. Lymphoma, sarcoidosis, metastatic carcinoma, and granulomatous diseases are often responsible for mediastinal lymphadenopathy, but the differential diagnosis also includes many other conditions. Thoracic surgeons are frequently consulted for this problem. Amyloidosis is an unusual cause of mediastinal lymphadenopathy. We report our recent experience with 2 patients suffering from mediastinal amyloidosis, and draw attention to diagnostic approaches and pitfalls relevant to thoracic surgical consultation in this disease.
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Case reports
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Patient 1
A 64-year-old man with a history of smoking complained of a cough and dyspnea. Examination did not show lymphadenopathy, organomegaly, finger clubbing, or peripheral edema. Electrocardiogram was normal. Chest computed tomography (CT) scan showed mediastinal lymphadenopathy and pleural effusions, but no lung masses (Fig 1). Lymphoma was suspected. Mediastinoscopy and biopsy of abnormal paratracheal lymph nodes showed diffuse amorphous eosinophilic material that stained positive with Congo red; amyloidosis was diagnosed (Fig 2).
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Fig 1. CT of the chest shows mediastinal lymphadenopathy in a man with dyspnea. Pleural effusions are also present.
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Fig 2. Lymph node biopsies show a foreign body giant cell reaction to an amorphous eosinophilic material (amyloid) (hematoxylin and eosin, x750).
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The patient was treated with prednisone and melphalan, but his condition worsened. Thoracoscopy, pleural biopsy, and talc pleurodesis were done for a bloody exudative right pleural effusion that recurred after thoracentesis. Postoperative congestive heart failure was treated with diuretics. The pleural biopsies showed amyloidosis. Bone marrow transplantation was eventually done.
Patient 2
A 76-year-old nonsmoking woman complained of dyspnea. Examination showed mild peripheral edema, elevation of the jugular venous pressure, and bibasilar lung crackles. There was no palpable lymphadenopathy. Electrocardiogram showed first-degree arterio-ventricular block. Chest roentgenogram and CT scan showed prominent mediastinal lymphadenopathy and a pleural effusion (Fig 3). Lymphoma was suspected, but amyloidosis was also considered. An echocardiogram suggested a restrictive cardiomyopathy. Instead of proceeding with mediastinoscopy, cardiac catheterization and endomyocardial biopsies were done. The cardiac biopsies showed amyloidosis. The mediastinal lymphadenopathy was consistent with a diagnosis of amyloidosis, so mediastinal lymph node biopsy was not needed. The patient’s condition deteriorated, and she died within 5 months of diagnosis.
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Fig 3. CT of the chest shows mediastinal lymphadenopathy in a woman with dyspnea and clinical features of heart failure. A pleural effusion is also present.
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Comment
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Amyloidosis is a heterogeneous group of disorders characterized by extracellular deposition of abnormal proteins in a ß-pleated configuration [1]. Although the precise etiology of amyloidosis is not known, the abnormal protein deposition may result from a disordered immune response to a prolonged antigenic challenge. Amyloidosis may be localized to one organ or body tissue, but more often it is systemic. The systemic form can be primary (idiopathic), associated with multiple myeloma, secondary to a chronic disease (such as rheumatoid arthritis, Crohn’s disease, cystic fibrosis), or familial. Localized amyloidosis has a good prognosis, but systemic amyloidosis often causes death within several years of diagnosis. Heart disease and renal failure are common causes of death.
Thoracic manifestations can occur in both the localized and systemic forms of amyloidosis [2]. Thoracic manifestations include adenopathy, pulmonary nodules, diffuse lung disease, pleural effusions, and laryngotracheobronchial disease [3]. Patients with localized amyloidosis involving the chest usually have pulmonary nodules (single or multiple) or laryngotracheobronchial lesions. Adenopathy is very unusual in patients with localized amyloidosis [3]. In contrast, patients with systemic amyloidosis can have any of the thoracic manifestations mentioned above, and they often occur in combination. The mediastinal lymph nodes are the most common site of thoracic involvement in systemic amyloidosis; 75% of patients with thoracic disease have adenopathy [3]. The lymph nodes may show punctate calcification, and the lymphadenopathy is often associated with parenchymal lung disease or pleural effusions; these features are diagnostic clues for amyloidosis [3, 4]. Adenopathy may dominate the clinical picture and lymphoma is often suspected [3, 4].
Our 2 patients suffered from primary systemic amyloidosis. Mediastinal lymphadenopathy, suggestive of lymphoma, was a prominent presenting feature in both cases. Mediastinoscopy is appropriate in this clinical situation, but thoracic surgeons should be aware of amyloidosis as a diagnostic possibility. Patients with systemic amyloidosis often have cardiac involvement, and this ultimately causes death in many patients with this disease. Surgical biopsies, and general anesthesia, are not without risk in patients with systemic amyloidosis.
If the possibility of mediastinal amyloidosis is considered, nonsurgical biopsy approaches can be attempted initially [1–4]. Biopsy of skin or subcutaneous fat may give a diagnosis. If pulmonary lesions are present, bronchoscopy will often be diagnostic [3]. Fine-needle aspiration biopsy of a pulmonary or mediastinal lesion is also an option [4]. Finally, endomyocardial biopsies often establish the diagnosis, as was the case in 1 of our patients. All of these biopsy techniques have a role in the diagnosis of amyloidosis. When a thoracic surgeon is confronted with mediastinal lymphadenopathy, amyloidosis and its diagnostic implications should be considered.
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References
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Cohen A.S. Amyloidosis. In: Isselbacher K.J., Braunwald E., Wilson J.D., Martin J.B., Fauci A.S., Kasper D.L., eds. Harrison’s principles of internal medicine, 13th ed. New York: McGraw-Hill, 1994:1625-1630.
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Utz J.P., Swensen S.J., Gertz M.A. Pulmonary amyloidosis. The Mayo clinic experience from 1980 to 1993. Ann Intern Med 1996;124:407-413.[Abstract/Free Full Text]
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Pickford H.A., Swensen S.J., Utz J.P. Thoracic cross-sectional imaging of amyloidosis. Am J Roentgenol 1997;168:351-355.[Abstract/Free Full Text]
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Hiller N., Fisher D., Shmesh O., Gottschalk-Sabag S., Dollberg M. Primary amyloidosis presenting as an isolated mediastinal mass. Thorax 1995;50:908-909.[Abstract/Free Full Text]
Accepted for publication July 16, 1999.
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Br. J. Radiol.,
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Abstract
Introduction
