Retroperitoneal Castleman's tumor and paraneoplastic pemphigus: report of a case and review of the literature
<p>Abstract</p> <p>Background</p> <p>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 common...
| Main Authors: | , , , |
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| Format: | Article |
| Language: | English |
| Published: |
BMC
2007-04-01
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| Series: | World Journal of Surgical Oncology |
| Online Access: | http://www.wjso.com/content/5/1/45 |
| Summary: | <p>Abstract</p> <p>Background</p> <p>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.</p> <p>Case presentation</p> <p>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.</p> <p>Conclusion</p> <p>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.</p> |
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| ISSN: | 1477-7819 |