Pulmonary Amyloidosis



Pulmonary Amyloidosis







Key Facts


Terminology



  • Nonneoplastic process characterized by deposition of amorphous proteinaceous material that may involve lung either primarily or secondarily


Etiology/Pathogenesis



  • Secondary to



    • Systemic amyloidosis


    • Light chain disease


    • Multiple myeloma


    • MALT lymphoma of lung


    • Sjögren syndrome


    • Familial neuropathy


    • Gaucher disease


Microscopic Pathology



  • Several distinct patterns of amyloid deposition





    • Nodular


    • Interstitial


    • Diffuse


    • Plaque-like


  • Cytologic features



    • Amorphous acellular material


    • Presence of giant cells toward periphery of lesion


    • Ossification may be present


    • Inflammatory reaction


Top Differential Diagnoses



  • Hyalinizing granuloma of lung


  • Alveolar proteinosis


  • Intrapulmonary solitary fibrous tumor (I-SFT)


  • Pneumocystis pneumonia


  • Pneumonia






Low-power view demonstrates lung parenchyma with nodules image of different sizes composed of an amorphous acellular material replacing lung parenchyma.






Closer view of the amorphous material replacing normal lung parenchyma is shown. The material is acellular image with a haphazard distribution.


TERMINOLOGY


Synonyms



  • Amyloidoma


Definitions



  • Nonneoplastic process characterized by deposition of amorphous proteinaceous material that may involve lung either primarily or secondarily


ETIOLOGY/PATHOGENESIS


May Develop Secondary to



  • Systemic amyloidosis


  • Light chain disease


  • Multiple myeloma


  • Waldenström macroglobulinemia


  • Familial neuropathy


  • Niemann-Pick disease


  • Gaucher disease


  • Sjögren syndrome


  • MALT lymphoma of lung


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare condition in lung


  • Age



    • Generally affects adults


  • Gender



    • No apparent gender predilection


  • Ethnicity



    • No apparent ethnic predilection


Site



  • Lung


Presentation



  • Dyspnea


  • Cough


  • Chest pain


  • Asymptomatic


Laboratory Tests



  • Potassium permanganate oxidation technique



    • Separates amyloid type AA from other forms of amyloid


Treatment



  • Surgical approaches



    • Surgical resection of tumor nodules is usually performed


Prognosis



  • Depends on clinical background, such as whether patient has systemic amyloidosis or any other disease associated with amyloid deposition


MACROSCOPIC FEATURES


General Features



  • Lung parenchyma can have 1 or more pulmonary nodules


Size



  • Varies; between 1 and > 10 cm


MICROSCOPIC PATHOLOGY


Histologic Features



  • Several distinct patterns of amyloid deposition in lung



    • Nodular


    • Interstitial


    • Diffuse


    • Plaque-like


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Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Pulmonary Amyloidosis

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