Improved outcomes after complete resection of underlying tumors  for patients with paraneoplastic pemphigus: a single-center  experience of 22 cases.
BACKGROUND
Paraneoplastic pemphigus (PNP) is an autoimmune-related acquired bullous disease. Delayed diagnosis and treatment of this clinically rare disease often result in poor prognosis.


METHODS
Between January 1999 and December 2009, 22 patients with confirmed PNP who underwent surgical resection of underlying tumors were enrolled in this study. Clinicopathologic characteristics, treatment options, and perioperative and long-term results were analyzed.


RESULTS
Among 22 patients, 2 patients died of severe infection several weeks after surgery. Postoperative major complications included pulmonary infections (n = 10) and septicemia (n = 4). Respiratory symptoms persisted in 13 patients. Tumors were completely resected in 20 patients. Two patients whose tumors were not completely resected died of relapse 2 and 32 months after surgery. Two patients with completely resected tumors died of respiratory failure 10 and 24 months after surgery, respectively. One patient whose pathological result was follicular dentritic cell sarcoma had a relapse recently. The remaining 15 patients have survived till now.


CONCLUSIONS
Early detection, prompt treatment, and complete resection of PNP can effectively decrease the mortality and speed up the recovery.
Zhang
Jun
J
Department of General Surgery, Peking University First Hospital, No 8 Xishiku Street, Xicheng District, Beijing 100034, China.
Qiao
Qi-lu
QL
Chen
Xi-xue
XX
Liu
Ping
P
Qiu
Jian-xing
JX
Zhao
Hu
H
Zhao
Jian-xun
JX
Liu
Yu-cun
YC
Wan
Yuan-lian
YL
D000293:Adolescent; D000328:Adult; D005871:Castleman Disease / Q000601:surgery; D002681:China; D003713:Dendritic Cells; D005260:Female; D006801:Humans; D008228:Lymphoma, Non-Hodgkin / Q000601:surgery; D008297:Male; D008875:Middle Aged; D009369:Neoplasms / Q000601:surgery*; D010257:Paraneoplastic Syndromes / Q000473:pathology*; D010392:Pemphigus / Q000209:etiology / Q000473:pathology*; D016019:Survival Analysis; D013945:Thymoma / Q000601:surgery; D013953:Thymus Neoplasms / Q000601:surgery; D016896:Treatment Outcome
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