Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature.
BACKGROUND
Castleman's disease is a rare lymphoproliferative syndrome. Its etiology and pathogenesis are unclear. The disease can be occasionally associated with a paraneoplastic pemphigus (PNP), an autoimmune mucocutaneous disorder commonly seen in neoplasms of lymphocytic origin.


METHODS
We present a case of a 63-year old male patient who was referred for surgical treatment of a lately diagnosed retroperitoneal pelvic mass. The patient had been already treated for two years due to progressive diffuse cutaneous lesions histologically consistent with lichen ruber verucosus and pemphigus vulgaris. Intraoperatively a highly vascularized solid mass occupying the small pelvis was resected after meticulous vascular ligation and hemostasis. After surgery and following immunosuppressive treatment a clear remission of the skin lesions was observed.


CONCLUSIONS
Castleman's tumor should be always suspected when a retroperitoneal mass is combined with PNP. In a review of the literature we found 37 additional cases. Complete surgical resection of the tumor can be curative in most of the cases.
Menenakos
Charalambos
C
Department of General, Visceral, Vascular and Thoracic Surgery, Universitaetsmedizin Berlin, Charité Campus Mitte, Berlin, Germany. menenakos@hotmail.com
Braumann
Chris
C
Hartmann
Jens
J
Jacobi
Christoph A
CA
D005871:Castleman Disease / Q000150:complications* / Q000473:pathology / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D010257:Paraneoplastic Syndromes / Q000150:complications* / Q000473:pathology; D010392:Pemphigus / Q000150:complications* / Q000473:pathology; D012187:Retroperitoneal Space