A Case of Good Syndrome Presumed Secondary to Metastatic Pancreatic Thymoma in a Patient Presenting With a Myasthenic Crisis Postthymectomy

Abstract

Introduction: 

Myasthenia gravis (MG) is an autoimmune disorder characterized by autoantibodies against the postsynaptic nicotinic acetylcholine receptors, muscle-specific tyrosine kinase, low-density lipoprotein receptor–related protein 4, and agrin. The incidence of thymoma in MG is reported as ∼10%–15%. The incidence of extrathoracic metastatic thymoma is exceedingly rare and may present years after resection. Associations between thymoma and immunodeficiency have also been described, including Good syndrome.

Methods and Results: 

We describe the clinical course, investigations, and treatments performed in a patient presenting with a myasthenic crisis in the setting of acetylcholine receptor antibody-positive generalized MG 10 years postthymectomy. Computed tomography imaging revealed 2 pancreatic lesions, but no residual thoracic thymoma. Biopsy confirmed metastatic pancreatic thymoma, which was successfully resected. His course was further complicated by cytomegalovirus retinitis with a depressed CD4 count and perniosis.

Discussion: 

This presentation was felt to be consistent with Good immunodeficiency syndrome.