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. CASE PRESENTATION: 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. CONCLUSION: 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

Neoplasms of the genitourinary system

Nephroblastoma or Wilms’ tumor (WT) is the most common renal neoplasm in children accounting for 90 % of pediatric renal tumors (Pastore et al. 2006). It is a tumor with a good prognosis and with well-established treatment strategies. Other rare malignant renal tumors, such as clear cell sarcoma and rhabdoid tumor of the kidney, have a poor prognosis despite aggressive treatment. Renal cell carcinoma occurs in older children, while mesoblastic nephroma is the most frequent renal tumor in the neonate. Hematological malignancies, the most frequent neoplasms in children, may also involve the kidney, most often as part of a multi-organ involvement. Renal infections and malformations are much more common in children than renal tumors and may show a pseudotumoral pattern mimicking a renal tumor. In all cases, close collaboration among radiologists, pediatricians, and pathologists is essential so as to avoid diagnostic pitfalls due to atypical presentations