Radical surgery may be not an optimal treatment approach for XXXX .

@entity1 @entity66 @entity1074 @entity786 @entity3118 @entity1349
UNASSIGNED: Primary @entity1074 is a rare disease, and no standard treatments have been defined yet. In this study, 38 consecutive @entity1 from single center were reviewed. Among 25 @entity1 with @entity66 , radical surgery were performed in 12 @entity1 , and the other 13 @entity1 had chemotherapy combined with (7 @entity1 ) or without (6 @entity1 ) radiotherapy. No significant difference in @entity1349 ( @entity1349 ) was found between @entity1 who received surgery or not; however, @entity1 treated with chemotherapy had superior progression-free survival (PFS) than those treated with upfront surgery (P   =   0.032). Among the 12 @entity1 who received radical surgery, 7 were given adjuvant chemotherapy and 1 @entity1 had consolidation radiotherapy. No significant differences in PFS and @entity1349 exist between those who received adjuvant treatment or not (P   >   0.05). For @entity1 who received chemotherapy, PFS and @entity1349 were significantly better for those treated with @entity786 -based therapy than @entity3118 -based therapy. At a median follow-up time of 61.1  months, 5- and 10-year PFS rate was 70.0 and 43.0  %, respectively, and 5- and 10-year @entity1349 rate was both 81.0  %. In conclusion, we confirmed the indolent behavior and favorable outcome of this disease. In order to preserve lung function and reduce the risks associated with surgery, radiotherapy or rituximab in combination with alkylating drug-based chemotherapy should be considered as first-line option for @entity1074 .
