Extrathymic malignancies in patients with myasthenia gravis.
BACKGROUND
Myasthenia gravis (MG) is considered a paraneoplastic phenomenon of thymomas in 15% of patients. Co-existence of MG with extrathymic malignancies, and an increased risk of second malignancy in patients with thymoma have been reported. Data on clinical characteristics of MG patients with extrathymic malignancies and the role of concomitant diseases and their treatment are lacking.


METHODS
The clinical records of 188 consecutive MG patients were studied retrospectively. We examined whether gender, age, generalized disease, seropositivity for acetyl-choline receptor antibodies, occurrence of thymoma, immunosuppressive therapy and occurrence of other autoimmune diseases determined an increased risk for development of extrathymic malignancy.


RESULTS
This group followed the typical epidemiological characteristics of MG. Thirty-three patients (17.6%) had a thymoma. Twenty-nine patients (15.4%) had 30 extrathymic malignant tumors of various origins. Only four patients with extrathymic tumors had an associated thymoma. Tumors were diagnosed between 20 years prior to and 35 years after the appearance of MG. Older age of MG onset was the only risk factor identified for development of malignancy in MG.


CONCLUSIONS
Extrathymic malignancies are common in MG patients, especially in the older age group. There are no specific clinical features of the subgroup of MG patients with cancer. Although MG is not a paraneoplastic phenomenon of extrathymic malignancy, the association between MG and malignancy may be due to a common background of immune dysregulation.
Department of Neurology, The Agnes Ginges Center for Human Neurogenetics, Hadassah-Hebrew University Hospital, Jerusalem 91120, Israel.
Netta
N
Levin
Oded
O
Abramsky
Alexander
A
Lossos
Dimitrios
D
Karussis
Tali
T
Siegal
Zohar
Z
Argov
Tamir
T
Ben Hur
D000328:Adult; D000367:Age Factors; D017668:Age of Onset; D000368:Aged; D001327:Autoimmune Diseases / Q000150:complications / Q000453:epidemiology / Q000473:pathology; D001379:Azathioprine / Q000627:therapeutic use; D018450:Disease Progression; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D009157:Myasthenia Gravis / Q000150:complications* / Q000473:pathology; D009369:Neoplasms / Q000453:epidemiology* / Q000209:etiology* / Q000473:pathology; D011950:Receptors, Cholinergic / Q000276:immunology; D012189:Retrospective Studies; D012737:Sex Factors; D013945:Thymoma / Q000473:pathology
D007166:Immunosuppressive Agents; D011950:Receptors, Cholinergic; D001379:Azathioprine