title: Polymyositis with respiratory muscle weakness requiring mechanical ventilation in a patient with metastatic thymoma treated with octreotide.
abstract: Although most patients with thymoma present with a mediastinal mass amenable to surgical resection, some patients develop metastatic disease requiring systemic therapy. The majority of thymomas express somatostatin receptors as demonstrated by octreotide scanning, an observation which has prompted the clinical use of octreotide in patients with this disease. Many patients with thymoma exhibit autoimmune paraneoplastic syndromes, most frequently myesthenia gravis. We report here the case of a patient with metastatic thymoma who developed a profound autoimmune polymyositis and lupus-like syndrome that flared following treatment with octreotide and was associated with a clinical response to this agent. No evidence for myesthenia gravis was discovered. The severity of the myopathy necessitated mechanical ventilation for 12 weeks. The natural history of thymoma, treatment options including recent combination chemotherapy regimens, and potential mechanisms for flaring of autoimmune paraneoplastic syndromes triggered by therapy of thymoma are discussed.
authors.affiliation: Department of Medicine, University of Chicago, IL, USA.
authors.forename: B I
authors.initials: BI
authors.lastname: Rini
authors.forename: T F
authors.initials: TF
authors.lastname: Gajewski
mesh_terms: D000208:Acute Disease; D000293:Adolescent; D018931:Antineoplastic Agents, Hormonal / Q000009:adverse effects* / Q000627:therapeutic use; D000971:Antineoplastic Combined Chemotherapy Protocols / Q000627:therapeutic use; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D018763:Muscle Fatigue; D015282:Octreotide / Q000009:adverse effects* / Q000627:therapeutic use; D010257:Paraneoplastic Syndromes / Q000175:diagnosis / Q000188:drug therapy; D017285:Polymyositis / Q000139:chemically induced* / Q000628:therapy; D012121:Respiration, Artificial; D012132:Respiratory Muscles / Q000187:drug effects / Q000473:pathology*; D013945:Thymoma / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology / Q000556:secondary; D013953:Thymus Neoplasms / Q000175:diagnosis / Q000188:drug therapy* / Q000503:physiopathology; D014139:Tracheostomy
chemical_list: D018931:Antineoplastic Agents, Hormonal; D015282:Octreotide