To determine if paracentral acute middle maculopathy (PAMM) and peripapillary intraretinal and subretinal fluid (IRF/SRF) could help distinguish between arteritic anterior ischemic optic neuropathy (A-AION) and nonarteritic AION (NA-AION) at an early stage. Nested prospective cross-sectional diagnostic accuracy study. This study used single-center optical coherence tomography (OCT) data from 8 patients with A-AION and 24 patients with NA-AION from two prospective cross-sectional studies with consecutive sampling (ClinicalTrials.gov: NCT05248906 and NCT05305079). The diagnosis of A-AION was based on expert interpretation of biochemical markers of inflammation, temporal artery biopsy and positron emission tomography/computed tomography. The diagnosis of NA-AION was made in cases without suspicion or clinical evidence of A-AION and with confirmed neuroophthalmological expert diagnosis. For this substudy patients were also required to have an OCT scan in relation to the diagnosis of AION. Macular OCT scans were graded by two independent, masked graders for the presence of PAMM and for IRF/SRF. The extension of IRF/SRF was assessed using an Early Treatment Diabetic Retinopathy Study (ETDRS) grid. PAMM was found in 50% of patients with A-AION and in 0% of patients with NA-AION (P = .0019). In the setting of AION, the sensitivity of PAMM for the diagnosis of A-AION was 50% (95% CI: 16%-84%) while the specificity was 100% (95% CI: 86%-100%). Conversely, peripapillary IRF/SRF with extension into the ETDRS grid was observed in 83% of patients with NA-AION but in 0% of patients with A-AION (P = .000047). The sensitivity of central macula-involving IRF/SRF for the diagnosis of NA-AION was 83% (95% CI: 63%-95%), while the specificity was 100% (95% CI: 63%-100%). Combining the two biomarkers, 75% of patients with AION could be classified based on OCT alone. PAMM appears to be a biomarker of A-AION while extensive peripapillary fluid appears to be a biomarker of NA-AION. Combining OCT biomarkers might allow for early classification of AION and warrants further prospective studies.
Primary cardiac tumors are extremely rare, with an incidence of 0.001–0.03%. Twenty-five percent of these tumors are malignant, with sarcomas accounting for approximately 95%. Cardiac intimal sarcoma is the least reported subtype of primary cardiac sarcoma. These endocardial mesenchymal tumors most often arise from great arterial vessels, and are rarely located in the heart. They often present with an aggressive clinical course and have a poor prognosis, with surgical resection with achievement of free margins being the mainstay of treatment. This emphasizes the importance of an early, correct diagnosis and timely intervention. We report a 60-year-old Caucasian male with several former cardiac surgical procedures due to congenital aortic stenosis, presenting with functional mitral stenosis/insufficiency and left ventricular outflow tract obstruction (LVOTO) due to massive masses in the left ventricle and atrium of the heart. Hybrid imaging with 2-deoxy-2-[18F]fluoro-D-glucose positron emission tomography/computed tomography (2-[18F]FDG PET/CT) was performed prior to surgery to characterize the intracardiac masses and estimate tumor burden, as well as to identify a potential extracardiac primary malignancy.
Angiosarcomas are highly malignant and rare tumors of vascular or lymphatic endothelial cell origin with a poor prognosis. Lymphangiosarcoma associated with chronic lymphedema is known as Stewart-Treves syndrome. Stewart-Treves syndrome is primarily described in patients with lymphedema of an upper extremity occurring after breast cancer surgery including radical axillary lymph node dissection and subsequent radiotherapy. It is rarely described in the presence of idiopathic chronic lymphedema of the lower extremities. We present a case of lymphangiosarcoma visualized on 18F-FDG PET/CT, where Stewart-Treves syndrome is secondary to probably a combination of idiopathic chronic lymphedema of the lower extremities and systemic immunosuppressive treatment.
Polymyalgia rheumatica (PMR) and large-vessel vasculitis (LVV) are related rheumatic diseases which are occasionally present concomitantly. PMR is characterized by synovitis and bursitis. In LVV, inflammation of the blood vessel wall is seen. Both disorders can be difficult to diagnose since patients often present non-specific symptoms and results of blood tests. The non-specific symptoms cannot always be distinguished from symptoms indicating an occult malignancy. We present a case of PMR and LVV in a Scandinavian man visualized on [18F]-2-deoxy-D-glucose positron emission tomography/computed tomography (18F-FDG PET/CT) with the presentation of typically affected sites of joints and arteries and with the same imaging modality ruling out occult malignancy.