Adverse events beyond the RUBY trial: reporting immunotherapy-associated myocarditis, myositis, and myasthenia gravis in a real-world endometrial cancer case

Abstract

Objectives

To report a rare case of immune checkpoint inhibitor (ICI)-associated myocarditis, myositis, and myasthenia gravis (MMM syndrome) in a patient with advanced endometrial cancer treated with dostarlimab, highlighting the diagnostic and therapeutic challenges, in addition to the importance of biomarker-informed treatment selection.

Methods

We report the case of a 75-year-old woman with unresectable stage IIIC2 endometrial adenocarcinoma and mismatch repair (MMR) deficiency treated with carboplatin and paclitaxel, followed by dostarlimab. After initiation of dostarlimab, the patient presented with neuromuscular and cardiac symptoms, leading to the diagnosis of myocarditis, myositis, myasthenia gravis (MMM) syndrome requiring hospitalization. A multidisciplinary care including neurologic, cardiac, and critical care management was required.

Results

The patient developed MMM syndrome 81 days after starting dostarlimab, presenting with bilateral ptosis, dysphagia, diplopia and respiratory compromise. Evaluation revealed elevated troponins, elevated creatine kinase, and a positive acetylcholine receptor antibody. Cardiac MRI confirmed myocarditis. Management included high-dose steroids, mycophenolate, IVIG, abatacept, and tofacitinib, with initial stabilization.

Conclusions

MMM syndrome is a rare but life-threatening complication of ICI therapy. Early recognition and multidisciplinary management are crucial. This case underscores the importance of weighing the risks and benefits of initiating immunotherapy and utilizing biomarker-driven clinical decisions.