Efficacy of [177Lu]Lu-DOTATATE in metastatic neuroendocrine neoplasms of different locations: data from the SEPTRALU study.

BACKGROUND: Peptide receptor radionuclide therapy (PRRT) is one of the most promising therapeutic strategies in neuroendocrine neoplasms (NENs). Nevertheless, its role in certain tumor sites remains unclear. This study sought to elucidate the efficacy and safety of [177Lu]Lu-DOTATATE in NENs with different locations and evaluate the effect of the tumor origin, bearing in mind other prognostic variables. Advanced NENs overexpressing somatostatin receptors (SSTRs) on functional imaging, of any grade or location, treated at 24 centers were enrolled. The protocol consisted of four cycles of 177Lu-DOTATATE 7.4 GBq iv every 8 weeks (NCT04949282). RESULTS: The sample comprised 522 subjects with pancreatic (35%), midgut (28%), bronchopulmonary (11%), pheochromocytoma/ paraganglioma (PPGL) (6%), other gastroenteropancreatic (GEP) (11%), and other non-gastroenteropancreatic (NGEP) (9%) NENs. The best RECIST 1.1 responses were complete response, 0.7%; partial response, 33.2%; stable disease, 52.1%; and tumor progression, 14%, with activity conditioned by the tumor subtype, but with benefit in all strata. Median progression-free survival (PFS) was 31.3 months (95% CI, 25.7-not reached [NR]) in midgut, 30.6 months (14.4-NR) in PPGL, 24.3 months (18.0-NR) in other GEP, 20.5 months (11.8-NR) in other NGEP, 19.8 months (16.8-28.1) in pancreatic, and 17.6 months (14.4-33.1) in bronchopulmonary NENs. [177Lu]Lu-DOTATATE exhibited scant severe toxicity. CONCLUSION: This study confirms the efficacy and safety of [177Lu]Lu-DOTATATE in a wide range of SSTR-expressing NENs, regardless of location, with clinical benefit and superimposable survival outcomes between pNENs and other GEP and NGEP tumor subtypes different from midgut NENs.

Isolated Immunoglobulin G4-Related Disease of Nasal Septum and Maxilla: Diagnosis and Follow-up With 18F-FDG PET/CT.

Immunoglobulin G4-related disease (IgG4-RD) is a systemic inflammatory condition that can manifest in atypical locations. We present the case of a middle-aged woman presenting with isolated IgG4-RD of left maxilla and nasal septum, confirmed with F-FDG PET/CT. She achieved complete metabolic response with multiple pharmacologic treatment, but 1 year later, she experienced local relapse, and FDG PET/CT found new additional lesions in humeral vasculature and lungs. This case illustrates the usefulness of FDG PET/CT for initial evaluation and monitoring of IgG4-RD with unusual nasomaxillary involvement.

Neumonía por SARS-CoV-2 (COVID-19): hallazgo casual en estudio PET/TC con 18F-FDG para el diagnóstico de vasculitis / SARS-CoV-2 (COVID-19) pneumonia: incidental finding on 18F-FDG PET/CT study for vasculitis diagnosis

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Late-onset Cogan's syndrome associated with large-vessel vasculitis / Síndrome de Cogan de inicio tardío asociado con una vasculitis de gran vaso

Cogan's syndrome is a rare autoimmune disease that usually affects young Caucasian adults and is classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years. Nevertheless, cases with atypical ophthalmologic and audiovestibular features, with systemic manifestations or affecting children and older patients have also been reported, expanding the clinical spectrum of Cogan's syndrome. Herein, we present the case of a late-onset Cogan's syndrome associated with a large-vessel vasculitis

El síndrome de Cogan es una enfermedad autoinmune rara, que afecta frecuentemente a pacientes jóvenes de raza caucásica y que se define clásicamente por la combinación de queratitis intersticial no sifilítica y síntomas audiovestibulares similares a una enfermedad de Ménière, que se desarrollan en un intervalo de menos de 2 años. Sin embargo, se han descrito casos con manifestaciones oftalmológicas o audiovestibulares atípicas, con síntomas sistémicos o que afectan a niños o pacientes ancianos, expandiendo de este modo el espectro clínico del síndrome de Cogan. Presentamos aquí el caso de un síndrome de Cogan de inicio tardío asociado con una vasculitis de gran vaso

Late-onset Cogan's syndrome associated with large-vessel vasculitis.

Cogan's syndrome is a rare autoimmune disease that usually affects young Caucasian adults and is classically defined as the combination of nonsyphilitic interstitial keratitis and audiovestibular symptoms resembling Meniere's disease, both of them developed in an interval of less than two years. Nevertheless, cases with atypical ophthalmologic and audiovestibular features, with systemic manifestations or affecting children and older patients have also been reported, expanding the clinical spectrum of Cogan's syndrome. Herein, we present the case of a late-onset Cogan's syndrome associated with a large-vessel vasculitis.