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: Amyloidosis is a consequent of extracellular deposition of insoluble fibrillar proteins. It could present either localized or systemic. Half of the cases have involvement of the trachea, bronchi or the lung parenchyma. We report a case of multinodular pulmonary amyloidosis and its course over six years.
CASE PRESENTATION: An 87-year-old male presented with new onset leg edema. He denied dyspnea, loss of appetite or weight loss. Physical exam revealed a well developed man with moderate dependent edema on the legs but no crackles. A computed tomography of the chest confirmed multiple nodules on the lung parenchyma as well as the pleura, pericardium and retroperitoneal membrane. Per history, patient had innumerable pulmonary noncalcified pulmonary nodules six years prior to this presentation. He had received a core needle biopsy of one of the nodules and had a diagnosis of nodular amyloidosis was established. The biopsy had revealed amorphous eosinophilic material. Congo red stain was also performed and the material was congophilic and displayed apple-green birefringence when examined under polarized light. On this admission, the nodularities had enlarged in size and increased in number within six years. His protein electrophoresis slightly elevated IgG-lambda measured at 0.4 g/dl. He refused any further investigation and was discharged home in a stable condition.
DISCUSSION: Respiratory involvement of amyloidosis is usually associated with its systemic form of and is either AA or AL type. Lung is rarely affected as the single organ. Radiologically, the primary nodular pulmonary amyloidosis appears as single or multiple nodules in any lobe, and should be considered in the differential diagnosis of pulmonary primary or metastatic neoplasms. Since the prognosis of pulmonary amyloidosis is better than its systemic involvement, we concluded that our patient has multinodular pulmonary amyloidosis without systemic involvement.
CONCLUSIONS: Involvement of lung as a single organ in amyloidosis is very rare and should always raise a concern for pulmonary metastasis of a distal neoplasm. Moreover, pulmonary amyloidosis should always be in differential when managing multinodular lung lesions.
Reference #1: Zhang LA. et al. Mimicking pulmonary multiple metastatic tumors: A case of primary nodular parenchymal pulmonary amyloidosis with review of the literature. Oncology Letters 4: 1366-1370, 2012
DISCLOSURE: The following authors have nothing to disclose: Mehdi Shahidi, Mingchen Song, Viral Doshi, Robel Haile
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