Abstract
BackgroundCastleman'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.Case presentationWe 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.ConclusionCastleman'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.
Highlights
Castleman's disease is a rare lymphoproliferative syndrome
The hyaline vascular type frequently appears as a benign isolated mediastinal or rarely retroperitoneal mass, which does not recur after curative surgical excision
The plasma-cell type is associated with constitutional symptoms, multicentric lymphnode involvement, lymphoma development and autoimmune disease like clinical and laboratory abnormalities, including paraneoplastic pemphigus (PNP) [2]
Summary
Castleman's disease is a rare lymphoproliferative syndrome which was first described in 1956 [1]. The distinctive clinical findings in PNP include severe painful oral erosions and ulcerations with hemorrhagic crusting of the lips and polymorphous skin lesions resembling erythema multiforme, pemphigus vulgaris (PV) or lichen planus pemphigoides [9]. These clinical findings were observed in our patient. Following a removal of the left ureter catheter and change of the right one, the patient could be released in a good condition with almost complete remission of the mucocutaneous lesions four weeks later (2 months after initial surgery)
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