Title | A critical role of surgery in the treatment for paraneoplastic pemphigus caused by localized Castleman's disease |
Authors | Fang, Yujiang Zhao, Lei Yan, Feng Cui, Xijun Xia, Yue Duren, Alicia |
Affiliation | Univ Missouri, Dept Internal Med, Columbia, MO 65212 USA. Peking Univ, Affiliated Hosp 3, Dept Urol, Beijing 100083, Peoples R China. Harry S Truman Mem Vet Hosp, Dept Vet Affairs, Res Serv, Columbia, MO 65212 USA. Second Affiliated Hosp, Wannan Med Coll, Dept Internal Med, Wuhu 241004, Peoples R China. Peoples Hosp Ningjin, Dept Surg, Ningjin 253400, Shandong, Peoples R China. Wuhan Univ, Renmin Hosp, Dept Urol, Wuhan 430060, Peoples R China. |
Keywords | Castleman's disease Paraneoplastic pemphigus Surgical treatment INTERLEUKIN-6 NEOPLASIA VULGARIS |
Issue Date | 2010 |
Publisher | medical oncology |
Citation | MEDICAL ONCOLOGY.2010,27,(3),907-911. |
Abstract | Paraneoplastic pemphigus (PNP) is an autoimmune mucocutaneous disease associated with lympho-proliferative tumors, and sometimes with a very rare tumor, Castleman's disease (CD). PNP can present as a variety of dermatological diseases, and so far, only limited studies of PNP caused by CD have been reported, resulting in its higher possibility of misdiagnosis. Because of the variability of clinical presentation of PNP caused by CD, selection of the appropriate therapeutic approach remains unclear. To investigate the efficacy of surgery to patients with PNP caused by localized CD, the clinical, laboratory and pathological data of 5 patients with PNP caused by localized CD, 3 females and 2 males, aged 34 years (ranging from 29 to 42), with the tumor size from 6 x 4 x 4 cm(3) to 8 x 8 x 7 cm(3), located in the mediastinum (n = 3) and retroperitoneum (n = 2), were compared before and after surgery. All patients underwent surgery, and the diagnosis was confirmed by pathological examination. Surgery significantly improved mucosal lesions, cured skin lesions and decreased serum pemphigus autoantibody 6 months after surgery. Most patients were followed up for 6-48 months, and they all had no clinical or radiological recurrence and remain disease free. Therefore, surgery is an effective approach to cure patients with PNP caused by localized CD. |
URI | http://hdl.handle.net/20.500.11897/242400 |
ISSN | 1357-0560 |
DOI | 10.1007/s12032-009-9304-y |
Indexed | SCI(E) |
Appears in Collections: | 第三医院 |