Key result
Two familial cases of renal retroperitoneal lymphangiomatosis highlight long-term urological and systemic complications.
Why the study?
Lymphangiomatosis of the kidneys and perirenal-retroperitoneal tissues is a rare disease of unknown etiology with unclear familial nature and long-term complications.
Case Report (n=2)
This report provides evidence supporting the familial nature of rare renal retroperitoneal lymphangiomatosis.
Raises possibility of familial screening in this rare disease; leaves open genetic studies and management guidelines.
Lymphangiomatosis of the kidneys and perirenal-retroperitoneal tissues is a rare disease of unknown etiology. We present two cases affecting members of the same family, supporting the familial nature of the disease. The natural history and related urological and systematic complications of the disease during a long-term follow-up are highlighted, while a comprehensive literature review is presented.
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Antonopoulos et al. (2010) conducted a case report in Familial renal retroperitoneal lymphangiomatosis (n=2). Two cases of renal retroperitoneal lymphangiomatosis in the same family support the familial nature of the disease and highlight its long-term urological and systemic complications.
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