TY - JOUR
T1 - Thymoma and the increased risk of developing extrathymic
malignancies: a multicentre study
AU - Margaritora, Stefano
PY - 2013
Y1 - 2013
N2 - OBJECTIVES: Although thymoma is considered a relatively indolent neoplasia, patients affected by this disease are at high risk of developing
second tumours (STs). The aim of this study is to assess the risk of developing STs after surgical thymoma resection.
METHODS: A multicentre retrospective study of patients operated on for thymoma within five Italian Thoracic Surgery Institutions,
between 2000 and 2011, was conducted. The overall STs number and incidence were calculated. The number of metachronous STs was
compared with the expected cancer number (ECN) in an Italian population, and the standardized incidence ratio (SIR) and 95% confidence
intervals were calculated. Potential variables of STs predictors were also evaluated.
RESULTS: There were 302 patients; myasthenia gravis (MG) was observed in 166 (55%) and other autoimmune syndromes in 49 of
them. In 118 patients (39.1%), the Masaoka thymoma stage was greater than II and in 194, the WHO histological type ranged from B1
to C. Fifty STs were observed (28 metachronous, 4 synchronous and 18 detected before thymoma). The observed metachronous STs
number was significantly higher than ECN. An increased risk of STs development was observed in advanced stage thymomas and in
those with histological high grade. On the contrary, MG seems to be a protective factor in STs development.
CONCLUSIONS: Our study confirms the high risk of developing STs in patients with thymoma. Aggressive forms of thymoma are those
in which this risk appears to be more evident. The central role of an intrinsic immune system alteration might be the key to interpret
this phenomenon.
AB - OBJECTIVES: Although thymoma is considered a relatively indolent neoplasia, patients affected by this disease are at high risk of developing
second tumours (STs). The aim of this study is to assess the risk of developing STs after surgical thymoma resection.
METHODS: A multicentre retrospective study of patients operated on for thymoma within five Italian Thoracic Surgery Institutions,
between 2000 and 2011, was conducted. The overall STs number and incidence were calculated. The number of metachronous STs was
compared with the expected cancer number (ECN) in an Italian population, and the standardized incidence ratio (SIR) and 95% confidence
intervals were calculated. Potential variables of STs predictors were also evaluated.
RESULTS: There were 302 patients; myasthenia gravis (MG) was observed in 166 (55%) and other autoimmune syndromes in 49 of
them. In 118 patients (39.1%), the Masaoka thymoma stage was greater than II and in 194, the WHO histological type ranged from B1
to C. Fifty STs were observed (28 metachronous, 4 synchronous and 18 detected before thymoma). The observed metachronous STs
number was significantly higher than ECN. An increased risk of STs development was observed in advanced stage thymomas and in
those with histological high grade. On the contrary, MG seems to be a protective factor in STs development.
CONCLUSIONS: Our study confirms the high risk of developing STs in patients with thymoma. Aggressive forms of thymoma are those
in which this risk appears to be more evident. The central role of an intrinsic immune system alteration might be the key to interpret
this phenomenon.
KW - Thymoma
KW - Thymoma
UR - http://hdl.handle.net/10807/72248
U2 - 10.1093/ejcts/ezs663
DO - 10.1093/ejcts/ezs663
M3 - Article
SN - 1010-7940
VL - 44
SP - 219
EP - 224
JO - European Journal of Cardio-thoracic Surgery
JF - European Journal of Cardio-thoracic Surgery
ER -