Case report
Clonal Light Chain Restricted Primary Intrapulmonary Nodular Amyloidosis

https://doi.org/10.1016/j.athoracsur.2004.03.075Get rights and content

Primary intrapulmonary nodular amyloidosis is a rare form of immunoglobulin associated amyloidosis, also falling under the alternative appellation of amyloidoma. Although amyloidomas in other organ sites may be reflective of a localized or more generalized plasma cell dyscrasia, in the context of its presentation in the lung the presumptive basis has long been held to be one of chronic inflammation. We encountered 2 patients with nodular amyloidosis in whom the pathologic examination disclosed the basis to be one of a light chain restricted clonal lymphocytic plasma cell infiltrate, although without morphologic features of coexisting pulmonary lymphoma. These 2 patients serve to underscore the potential categorization of some cases of pulmonary nodular amyloidosis as a form of low grade B cell lymphoproliferative disease.

Section snippets

Patient 1

This 60-year-old woman presented in the summer of 2003 with a pneumothorax. Radiographic studies at that point in time revealed a right lung mass. Specifically, computed tomography of the chest revealed a 2.3 × 2.7 cm mass involving the lateral segment of the middle lobe as well as two smaller subpleural nodules, more anteriorly in the middle lobe measuring roughly 7 mm in size and 1.1 cm, respectively (Fig 1, Fig 2, Fig 3). A fine needle aspiration was interpreted as representing nonsmall cell

Comment

We have presented two cases of localized intrapulmonary nodular amyloidosis, whereby the pathologic evidence of their categorization was low grade B cell lymphoproliferative disease and more specifically, localized forms of plasma cell dyscrasia. First, light chain restricted plasmacellular infiltrates were observed in both cases, one with cardinal features of B cell neoplasia. Second, both cases had an accompanying small lymphocytic infiltrate composed almost exclusively of B cells in contrast

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    At the periphery of the nodules usually scant infiltrate of lymphocytes or plasma cells may be seen. In cases associated with a B-cell lymphoproliferative disorder, the B-cell component may be only identified using sensitive clonality studies. 16-18 The differential diagnosis includes pulmonary hyalinizing granuloma and light chain deposition disease, which may show amyloid-like deposition. 19

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    Furthermore, the rarity of amyloid stroma in marginal zone lymphomas was stressed [5,6]. Ross et al. published 2 amyloid tumors of the lung without overt lymphoma, but with a kappa light-chain restricted plasma cell infiltrate, confirmed by polymerase chain reaction testing of paraffin-embedded tissue [21]. Ross et al's report and others have shown light chain restriction in plasma cell infiltrates of nodular pulmonary amyloidomas [17,22–26], and have somewhat blurred the previous clear-cut distinction between isolated amyloidoma of the lung and plasmacytoid lymphoma.

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