J Clin Neurol.  2012 Sep;8(3):161-169. 10.3988/jcn.2012.8.3.161.

Incidence of Thymoma in Myasthenia Gravis: A Systematic Review

Affiliations
  • 1Institute of Neurology, First Affiliated Hospital, Guangxi Medical University, Nanning, Guangxi, People's Republic of China. mxa550@hotmail.com
  • 2Department of Hematology, First Affiliated Hospital, Guangxi Medical University, Nanning, Guangxi, People's Republic of China.
  • 3Neurological and Mental Health Division, The George Institute for International Health, The University of Sydney, Sydney, Australia.

Abstract

BACKGROUND AND PURPOSE
Myasthenia gravis (MG) is usually comorbid with thymoma. More accurate estimates of the incidence thymoma in MG will help inform patients and their physicians, facilitate health policy discussions, provide etiologic clues, and optimize the management of MG.
METHODS
We conducted a systematic review search of relevant English-language studies published between 1960 and 2012 using MEDLINE and Embase. We identified additional studies by reviewing the bibliographies of the retrieved articles and hand searched the main neurology journals. Only incidence studies and case series of unselected MG patients in which information about thymoma were included.
RESULTS
Out of 2206 potentially relevant studies, 49 met the inclusion criteria. Although there was a considerable degree of heterogeneity, the pooled estimate of the incidence of thymoma in MG was 21% (95% confidence interval, 20-22%). The pooled incidence was significantly higher for surgery-based studies than for population- and hospital-based studies. A large proportion of the reported thymomas were noninvasive. Furthermore, thymoma appears to occur significantly more frequently among male MG patients and those older than 40 years at the onset of MG.
CONCLUSIONS
Thymoma is common in MG patients, but appears to be found more often in male MG patients and those older than 40 years at the onset of MG. Further research is needed to expand our understanding of these association conditions.

Keyword

incidence; thymoma; myasthenia; systematic review

MeSH Terms

Cohort Studies
Hand
Health Policy
Humans
Incidence
Male
Myasthenia Gravis
Neurology
Population Characteristics
Thymoma

Figure

  • Fig. 1 Flow chart of systematic review search strategy. MG: myasthenia gravis.

  • Fig. 2 Frequency of thymoma in myasthenia gravis: a meta-analysis plot.


Cited by  1 articles

Incidence and Prevalence of Myasthenia Gravis in Korea: A Population-Based Study Using the National Health Insurance Claims Database
Su-Yeon Park, Jin Yong Lee, Nam Gu Lim, Yoon-Ho Hong
J Clin Neurol. 2016;12(3):340-344.    doi: 10.3988/jcn.2016.12.3.340.


Reference

1. Thomas CR, Wright CD, Loehrer PJ. Thymoma: state of the art. J Clin Oncol. 1999. 17:2280–2289.
Article
2. Potagas C, Dellatolas G, Tavernarakis A, Molari H, Mourtzouhou P, Koutra H, et al. Myasthenia gravis: changes observed in a 30-years retrospective clinical study of a hospital-based population. J Neurol. 2004. 251:116–117.
Article
3. Oosterhuis HJ. The natural course of myasthenia gravis: a long term follow up study. J Neurol Neurosurg Psychiatry. 1989. 52:1121–1127.
Article
4. Stroup DF, Berlin JA, Morton SC, Olkin I, Williamson GD, Rennie D, et al. Meta-analysis of observational studies in epidemiology: a proposal for reporting. Meta-analysis Of Observational Studies in Epidemiology (MOOSE) group. JAMA. 2000. 283:2008–2012.
Article
5. Pendlebury ST, Rothwell PM. Prevalence, incidence, and factors associated with pre-stroke and post-stroke dementia: a systematic review and meta-analysis. Lancet Neurol. 2009. 8:1006–1018.
Article
6. Hackett ML, Yapa C, Parag V, Anderson CS. Frequency of depression after stroke: a systematic review of observational studies. Stroke. 2005. 36:1330–1340.
7. Lew W, Pai M, Oxlade O, Martin D, Menzies D. Initial drug resistance and tuberculosis treatment outcomes: systematic review and meta-analysis. Ann Intern Med. 2008. 149:123–134.
Article
8. Romi F. Thymoma in myasthenia gravis: from diagnosis to treatment. Autoimmune Dis. 2011. 2011:474512.
Article
9. Castleman B. The pathology of the thymus gland in myasthenia gravis. Ann N Y Acad Sci. 1966. 135:496–505.
Article
10. Oosterhuis HJ, de Haas WH. Rheumatic diseases in patients with myasthenia gravis. An epidemiological and clinical investigation. Acta Neurol Scand. 1968. 44:219–227.
11. Mulder DG, Herrmann C Jr, Keesey J, Edwards H. Thymectomy for myasthenia gravis. Am J Surg. 1983. 146:61–66.
Article
12. Monden Y, Uyama T, Nakahara K, Fujii Y, Hashimoto J, Ohno K, et al. Clinical characteristics and prognosis of myasthenia gravis with other autoimmune diseases. Ann Thorac Surg. 1986. 41:189–192.
Article
13. Papatestas AE, Genkins G, Kornfeld P, Eisenkraft JB, Fagerstrom RP, Pozner J, et al. Effects of thymectomy in myasthenia gravis. Ann Surg. 1987. 206:79–88.
Article
14. Giagheddu M, Puggioni G, Sanna G, Tamburini G, Marrosu F, Rachele MG, et al. Epidemiological study of myasthenia gravis in Sardinia, Italy (1958-1986). Acta Neurol Scand. 1989. 79:326–333.
Article
15. Maggi G, Casadio C, Cavallo A, Cianci R, Molinatti M, Ruffini E. Thymectomy in myasthenia gravis. Results of 662 cases operated upon in 15 years. Eur J Cardiothorac Surg. 1989. 3:504–509. discussion 510-511.
16. Thorlacius S, Aarli JA, Riise T, Matre R, Johnsen HJ. Associated disorders in myasthenia gravis: autoimmune diseases and their relation to thymectomy. Acta Neurol Scand. 1989. 80:290–295.
Article
17. Tola MR, Granieri E, Paolino E, Caniatti L, Quatrale R, Mazzanti B, et al. Epidemiological study of myasthenia gravis in the province of Ferrara, Italy. J Neurol. 1989. 236:388–390.
Article
18. Mantegazza R, Beghi E, Pareyson D, Antozzi C, Peluchetti D, Sghirlanzoni A, et al. A multicentre follow-up study of 1152 patients with myasthenia gravis in Italy. J Neurol. 1990. 237:339–344.
Article
19. Ferrari G, Lovaste MG. Epidemiology of myasthenia gravis in the province of Trento (northern Italy). Neuroepidemiology. 1992. 11:135–142.
Article
20. Yu YL, Hawkins BR, Ip MS, Wong V, Woo E. Myasthenia gravis in Hong Kong Chinese. 1. Epidemiology and adult disease. Acta Neurol Scand. 1992. 86:113–119.
21. Antonini G, Morino S, Gragnani F, Fiorelli M. Myasthenia gravis in the elderly: a hospital based study. Acta Neurol Scand. 1996. 93:260–262.
Article
22. Masaoka A, Yamakawa Y, Niwa H, Fukai I, Kondo S, Kobayashi M, et al. Extended thymectomy for myasthenia gravis patients: a 20-year review. Ann Thorac Surg. 1996. 62:853–859.
Article
23. Nicolaou S, Müller NL, Li DK, Oger JJ. Thymus in myasthenia gravis: comparison of CT and pathologic findings and clinical outcome after thymectomy. Radiology. 1996. 201:471–474.
Article
24. Aiello I, Pastorino M, Sotgiu S, Pirastru MI, Sau GF, Sanna G, et al. Epidemiology of myasthenia gravis in northwestern Sardinia. Neuroepidemiology. 1997. 16:199–206.
Article
25. Beekman R, Kuks JB, Oosterhuis HJ. Myasthenia gravis: diagnosis and follow-up of 100 consecutive patients. J Neurol. 1997. 244:112–118.
Article
26. Bulkley GB, Bass KN, Stephenson GR, Diener-West M, George S, Reilly PA, et al. Extended cervicomediastinal thymectomy in the integrated management of myasthenia gravis. Ann Surg. 1997. 226:324–334. discussion 334-335.
Article
27. Cosi V, Romani A, Lombardi M, Raiola E, Bergamaschi R, Piccolo G, et al. Prognosis of myasthenia gravis: a retrospective study of 380 patients. J Neurol. 1997. 244:548–555.
Article
28. Christensen PB, Jensen TS, Tsiropoulos I, Sørensen T, Kjaer M, Højer-Pedersen E, et al. Mortality and survival in myasthenia gravis: a Danish population based study. J Neurol Neurosurg Psychiatry. 1998. 64:78–83.
Article
29. Guidetti D, Sabadini R, Bondavalli M, Cavalletti S, Lodesani M, Mantegazza R, et al. Epidemiological study of myasthenia gravis in the province of Reggio Emilia, Italy. Eur J Epidemiol. 1998. 14:381–387.
30. Robertson NP, Deans J, Compston DA. Myasthenia gravis: a population based epidemiological study in Cambridgeshire, England. J Neurol Neurosurg Psychiatry. 1998. 65:492–496.
Article
31. Lavrnić D, Jarebinski M, Rakocević-Stojanović V, Stević Z, Lavrnić S, Pavlović S, et al. Epidemiological and clinical characteristics of myasthenia gravis in Belgrade, Yugoslavia (1983-1992). Acta Neurol Scand. 1999. 100:168–174.
Article
32. Holtsema H, Mourik J, Rico RE, Falconi JR, Kuks JB, Oosterhuis HJ. Myasthenia gravis on the Dutch antilles: an epidemiological study. Clin Neurol Neurosurg. 2000. 102:195–198.
Article
33. Abt PL, Patel HJ, Marsh A, Schwartz SI. Analysis of thymectomy for myasthenia gravis in older patients: a 20-year single institution experi-ence. J Am Coll Surg. 2001. 192:459–464.
Article
34. Budde JM, Morris CD, Gal AA, Mansour KA, Miller JI Jr. Predictors of outcome in thymectomy for myasthenia gravis. Ann Thorac Surg. 2001. 72:197–202.
Article
35. Fink JN, Wallis WE, Haydock DA. Myasthenia gravis with thymoma is more common in the Maori and Pacific Island populations in New Zealand. Intern Med J. 2001. 31:206–210.
Article
36. Mussi A, Lucchi M, Murri L, Ricciardi R, Luchini L, Angeletti CA. Extended thymectomy in myasthenia gravis: a team-work of neurologist, thoracic surgeon and anaesthesist may improve the outcome. Eur J Cardiothorac Surg. 2001. 19:570–575.
Article
37. de Perrot M, Liu J, Bril V, McRae K, Bezjak A, Keshavjee SH. Prognostic significance of thymomas in patients with myasthenia gravis. Ann Thorac Surg. 2002. 74:1658–1662.
Article
38. Rastenyte D, Vaitkus A, Neverauskas R, Pauza V. Demographic and clinical characteristics of patients with myasthenia gravis. Medicina (Kaunas). 2002. 38:611–616.
39. Remes-Troche JM, Téllez-Zenteno JF, Estañol B, Garduño-Espinoza J, García-Ramos G. Thymectomy in myasthenia gravis: response, complications, and associated conditions. Arch Med Res. 2002. 33:545–551.
40. Roth T, Ackermann R, Stein R, Inderbitzi R, Rösler K, Schmid RA. Thirteen years follow-up after radical transsternal thymectomy for myasthenia gravis. Do short-term results predict long-term outcome? Eur J Cardiothorac Surg. 2002. 21:664–670.
Article
41. Au WL, Das A, Tjia HT. Myasthenia gravis in Singapore. Neurol J Southeast Asia. 2003. 8:35–40.
42. Wirtz PW, Nijnuis MG, Sotodeh M, Willems LN, Brahim JJ, Putter H, et al. The epidemiology of myasthenia gravis, Lambert-Eaton myasthenic syndrome and their associated tumours in the northern part of the province of South Holland. J Neurol. 2003. 250:698–701.
Article
43. Casetta I, Fallica E, Govoni V, Azzini C, Tola M, Granieri E. Incid-ence of myasthenia gravis in the province of Ferrara: a community-based study. Neuroepidemiology. 2004. 23:281–284.
Article
44. Yasukawa Y, Yoshikawa H, Iwasa K, Yamada M, Takamori M. Comparative study of pre-operative thymic imaging and pathology in patients with myasthenia gravis. J Clin Neurosci. 2004. 11:610–613.
Article
45. Huang CS, Hsu HS, Huang BS, Lee HC, Kao KP, Hsu WH, et al. Factors influencing the outcome of transsternal thymectomy for myasthenia gravis. Acta Neurol Scand. 2005. 112:108–114.
Article
46. Matsuda M, Dohi-Iijima N, Nakamura A, Sekijima Y, Morita H, Matsuzawa S, et al. Increase in incidence of elderly-onset patients with myasthenia gravis in Nagano Prefecture, Japan. Intern Med. 2005. 44:572–577.
Article
47. Levin N, Abramsky O, Lossos A, Karussis D, Siegal T, Argov Z, et al. Extrathymic malignancies in patients with myasthenia gravis. J Neurol Sci. 2005. 237:39–43.
Article
48. Suzuki S, Shimoda M, Kawamura M, Sato H, Nogawa S, Tanaka K, et al. Myasthenia gravis accompanied by alopecia areata: clinical and immunogenetic aspects. Eur J Neurol. 2005. 12:566–570.
Article
49. Aghajanzadeh M, Khoshrang H, Mohammadzadeh A, Roudbari SA, Ghayeghran AR. Thymectomy for myasthenia gravis: prognostic factors in 70 patients. Asian Cardiovasc Thorac Ann. 2007. 15:371–375.
Article
50. Kim HK, Park MS, Choi YS, Kim K, Shim YM, Han J, et al. Neurologic outcomes of thymectomy in myasthenia gravis: comparative analysis of the effect of thymoma. J Thorac Cardiovasc Surg. 2007. 134:601–607.
Article
51. Tsinzerling N, Lefvert AK, Matell G, Pirskanen-Matell R. Myasthenia gravis: a long term follow-up study of Swedish patients with specific reference to thymic histology. J Neurol Neurosurg Psychiatry. 2007. 78:1109–1112.
Article
52. Al-Moallem MA, Alkali NH, Hakami MA, Zaidan RM. Myasthenia gravis: presentation and outcome in 104 patients managed in a single institution. Ann Saudi Med. 2008. 28:341–345.
Article
53. Singhal BS, Bhatia NS, Umesh T, Menon S. Myasthenia gravis: a study from India. Neurol India. 2008. 56:352–355.
Article
54. Citterio A, Beghi E, Millul A, Evoli A, Mantegazza R, Antozzi C, et al. Risk factors for tumor occurrence in patients with myasthenia gravis. J Neurol. 2009. 256:1221–1227.
Article
55. Matsui N, Nakane S, Nakagawa Y, Kondo K, Mitsui T, Matsumoto T, et al. Increasing incidence of elderly onset patients with myasthenia gravis in a local area of Japan. J Neurol Neurosurg Psychiatry. 2009. 80:1168–1171.
Article
56. Chmura Kraemer H, Periyakoil VS, Noda A. Kappa coefficients in medical research. Stat Med. 2002. 21:2109–2129.
Article
57. Masaoka A, Monden Y, Nakahara K, Tanioka T. Follow-up study of thymomas with special reference to their clinical stages. Cancer. 1981. 48:2485–2492.
Article
58. Detterbeck FC. Clinical value of the WHO classification system of thymoma. Ann Thorac Surg. 2006. 81:2328–2334.
Article
59. Voltz RD, Albrich WC, Nägele A, Schumm F, Wick M, Freiburg A, et al. Paraneoplastic myasthenia gravis: detection of anti-MGT30 (titin) antibodies predicts thymic epithelial tumor. Neurology. 1997. 49:1454–1457.
Article
60. Chen XJ, Qiao J, Xiao BG, Lu CZ. The significance of titin antibodies in myasthenia gravis--correlation with thymoma and severity of myasthenia gravis. J Neurol. 2004. 251:1006–1011.
61. Giraud M, Vandiedonck C, Garchon HJ. Genetic factors in autoimmune myasthenia gravis. Ann N Y Acad Sci. 2008. 1132:180–192.
Article
62. Tomaszek S, Wigle DA, Keshavjee S, Fischer S. Thymomas: review of current clinical practice. Ann Thorac Surg. 2009. 87:1973–1980.
Article
63. Meriggioli MN, Sanders DB. Autoimmune myasthenia gravis: emerging clinical and biological heterogeneity. Lancet Neurol. 2009. 8:475–490.
Article
Full Text Links
  • JCN
Actions
Cited
CITED
export Copy
Close
Share
  • Twitter
  • Facebook
Similar articles
Copyright © 2024 by Korean Association of Medical Journal Editors. All rights reserved.     E-mail: koreamed@kamje.or.kr