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.