Clinical Features of Postthymectomy Myasthenia Gravis
배미경1, 유우식2, 변고은2, 김대준2, 정경영2
서울대학교 의과대학 분당서울대학교병원 흉부외과학교실1
, 연세대학교 의과대학 세브란스병원 흉부외과학교실2
Background : Postthymectomy myasthenis graivs (PTMG) occasionally develops in patients who underwent complete thymoma resection, although there was no sign of myasthenia gravis before the operation. The mechanisms and the clinical features of PTMG are obscure. The aim of this study is to assess the clinical features of the patients with PTMG.
Methods : Between 1986 and 2013, a total of 591patients underwent resection of epithelial tumor of the thymus. We excluded patients with undetermined WHO histological type, type C thymoma and patients who underwent incomplete resection. Out of the remaining 430 patients, 223 patients were diagnosed as myasthenia gravis before operation and 207 patients had no sign of myasthenia gravis. We retrospectively reviewed these 207 thymoma patients without preoperative myasthenia gravis.
Results : The median follow-up time after thymoma resection was 49.0 months (range, 6.0 to 246.0 months). PTMG was found in 13 (6.3%) of 207 thymoma patients without preoperative MG. The patients included 9 male and 4 female with a median age of 42 (range, 27~60) years at the time of thymoma resection. Masaoka-Koga stages of initial thymomas were stage I in 7 patients, stage IIa in 1 patient, stage IIb in 2 patients, stage III in 1 patient and stage IVa in 2 patients. WHO histologic types were type AB in 2 patient, type B1 in 5 patients, type B2 in 4 patients, type B3 in 1 patients and type B2+B3 in 1 patient. The surgical types of thymoma resection were complete thymectomy in 2 patients and limited thymectomy in 11 patients.
The median interval between thymectomy and the onset of postoperative MG was 12 (range, 2~ 148) months. The symptoms of first manifestation were ocular symptoms in 6 patients and weakness affecting other than ocular muscles in 7 patients. Almost all patients had high serum titers of antiacetylcholine receptor-binding antibody (ARAb; mean, 9.466 ± 4.553 nmol/L). There were 2 patients (2.4 %) of PTMG in the complete thymectomy group (n=82) and 11 patients (8.8 %) in the limited thymectomy group (n=125). Seven patients (53.8%) of 13 patients with PTMG were associated with thymoma recurrence. Three patients were detected thymoma recurrence and PTMG simultaneously. Four patients were diagnosed thymoma recurrence and PTMG at different times during follow-up period.
Conclusion : The prevalence of PTMG is higher in the patients underwent limited thymectomy than the patients underwent complete thymectomy. In addition, More than half patients with PTMG are associated with thymoma recurrence. These findings suggested that the thymus might have significant role in the pathogenesis of PTMG.
책임저자: 정경영
연세대학교 의과대학 세브란스병원 흉부외과학교실
연락처 : 배미경, Tel: 031-787-7140 , E-mail : mkbae@snubh.org