A 70-year-old Caucasian male was diagnosed with iris deposits and elevated IOP LE. On examination, VA was 20/25 RE and 20/30 LE. IOP was 10 mm Hg RE and 16 mm Hg LE, on topical anti-glaucoma medication LE. Gonioscopy revealed ~200 translucent spherules of 200–400 microns diameter in the angle LE [Fig. and ]. Glaucomatous optic disc cupping with superior and inferior arcuate field defects were noted LE. Fine needle aspiration biopsy revealed amorphous material with mild Congo red staining, consistent with amyloid []. Systemic evaluation was normal. Medication compliance was poor with eventual glaucoma progression and refusal of surgical intervention. At 10-year follow-up, phthisis bulbi with no light perception was noted. The RE examination was normal all throughout.
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.