SESSION TITLE: Rare Disease Case Report Posters
SESSION TYPE: Case Report Poster
PRESENTED ON: Sunday, March 23, 2014 at 01:15 PM - 02:15 PM
INTRODUCTION: The differential diagnosis of lung nodules is vast. A history of smoking often leads the clinician to common entities such as malignancy, however less likely scenarios should be considered.
CASE PRESENTATION: A 65 year old man with COPD and an extensive smoking history was referred to our clinic for multiple pulmonary nodules and blebs seen on imaging. He later underwent VATS pleurodesis for a spontaneous pneumothorax, at which time a lung biopsy was performed of the nodules. Congo Red stain was positive and subsequent laboratory and imaging evaluation in the Hematology/Oncology Clinic was negative for systemic involvement, suggestive of primary pulmonary nodular amyloidosis.
DISCUSSION: The term "amyloidosis" refers to the deposition, aggregation, and accumulation of abnormally folded polypeptides. Although reports were published as early as that of Virchow in 1854, primary, localized pulmonary amyloidosis is rarely described in recent literature. In the pulmonary parenchyma, it may present in the nodular or diffuse alveolar septal forms. The nodular form, which has been termed “amyloidoma,” is often incidentally discovered on imaging, as in our patient. Congo Red staining is often used, and polarized microscopy, under which amyloid protein appears red-greed, and exhibits birefringence - a classic and unique feature confirms the diagnosis. Bronchoscopy with biopsy, thoracoscopy, closed pleural biopsy, or open lung biopsy are often reported means of obtaining tissue. Management often consists of local resection, and newer modalities such as the Nd:YAG-laser, however re-appearance is not uncommon. This patient’s presentation to the hospital for a pneumothorax and subsequent clinical course was serendipitous in that it provided a surgical setting for a biopsy.
CONCLUSIONS: The clinician is often faced with a broad differential diagnosis for lung nodules. Less common entities should be given consideration, such as primary pulmonary nodular amyloidosis.
Reference #1: Pinney J., and Hawkins B. Amyloidosis. Ann Clin Biochem 2012;4 : 229-241
Reference #2: Utz JP, Swensen SJ, Gertz MA. Pulmonary amyloidosis. The Mayo Clinic experience from 1980 to 1993. Ann Intern Med. 1996;124:407-1
Reference #3: Thompson P, Citron K., Amyloid and the Lower Respiratory Tract. Thorax; 1983; 38: 84-87
DISCLOSURE: The following authors have nothing to disclose: Stephen Kuperberg, Samir Fahmy
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