A 64-year-old man presented with an incidental right lung mass on chest X-ray. Mediastinoscopy and lymph node biopsy confirmed amyloid, remaining asymptomatic until four years later, developing exertional dyspnoea matched by significant increase of the mass by chest radiography. Basic laboratory evaluation and immunohistochemistry was unremarkable. Immunoelectrophoresis revealed a faint immunoglobulin G (IgG) kappa band. No mass spectrometry was performed. Colonic biopsy, bone marrow aspirate, immunophenotyping and trephine were normal. 123I Serum amyloid P-component (SAP) scintigraphy showed abnormal uptake within the mass only. Computerised tomography (CT) revealed mediastinal and hilar lymphadenopathy, with a 9.5 × 6.5 × 10 cm right mediastinal mass containing fat and calcification with a radiological differential diagnosis of a teratoma. F-18 fluorodeoxyglucose (FDG)-Positron Emission Tomography (PET)/CT demonstrated intermediate to high-grade uptake within the soft tissue and low-grade uptake in the fatty component (). Cardiac magnetic resonance imaging excluded cardiac involvement. Repeat core biopsy demonstrated fibrovascular tissue with focal amyloid deposition. The patient proceeded to surgical mass de-bulking for diagnosis and symptom relief and histology confirmed evidence of amyloid. The patient’s postoperative course was uneventful; no evidence of recurrence three months following surgery and marked symptomic improvement, with disappearance of the IGgk paraprotein following resection, currently planned for local consolidation radiotherapy.
A 71-year-old woman was investigated for a one-year history of deteriorating dyspnoea on a background of well-controlled asthma. Other co-morbidities included cutaneous systemic lupus erythematosus, rheumatoid arthritis and Sjogren’s syndrome. High-resolution computerised tomography (HRCT) showed multiple cysts, solid nodules and aggregate masses – the largest (2 × 1.5 cm) in the peripheral left lower lobe with fine-needle aspiration suggestive of amyloid. Serum biochemistry showed a polyclonal elevation of IgG, no detectable monoclonal band and normal serum-free light chains. Immunohistochemistry showed no specific staining for kappa or lambda antibodies. Mass spectrometry was not performed. Echocardiography excluded cardiac involvement and no visceral uptake by 123I SAP scintigraphy. Pulmonary function tests demonstrated a restrictive pattern, and a further HRCT raised suspicion of lymphocytic interstitial pneumonitis. FDG-PET/CT demonstrated similar low-grade uptake in all lung nodules (). A mini-thoracotomy was performed to obtain tissue from the largest nodule showing evidence of amyloid but too few infiltrating cells were present to confirm clonality.\nA low-grade lymphoplasmocytic lymphoma (LPL) was presumed to be driving the pulmonary amyloid, based on the assumption of low-grade B-cell clonal disorders that can occur with Sjogren’s syndrome. A trial of R-CVP (Rituximab – Cyclophoshamide, Vincristine, Prednisolone) chemotherapy was commenced for four months resulting in no progression of symptoms and stable lesions.