Retroperitoneal involvement with light chain amyloidosis- case series and literature review

Abstract

Retroperitoneal involvement is rare in patients with light chain (AL) amyloidosis and has only been published as case reports. Only 5 cases of retroperitoneal deposition have previously been reported in the setting of systemic AL amyloidosis. Data regarding the characteristics and clinical course of these patients are scarce. Herein we report on eleven patients with systemic AL amyloidosis diagnosed with retroperitoneal deposition at all three Mayo Clinic sites and Davidoff cancer center in Israel. All patients had retroperitoneal amyloid deposition at presentation. All patients received systemic treatment. Eight patients presented with hydronephrosis and 7 had nephrostomy tubes or stents inserted. Regression of the deposition was documented in one patient and one patient was able to have his nephrostomy tube removed. The median OS from diagnosis was 150 months, suggesting that retroperitoneal deposition might confer improved prognosis compared to the general amyloid population.

Keywords:

• Amyloidosis
• periureteral
• retroperitoneal
• soft tissue
• nephrostomy

Disclosure statement

Dr. Gertz reports personal fees from Ionis/Akcea, personal fees from Alnylam, personal fees from Prothena, personal fees from Janssen, grants and personal fees from Spectrum, personal fees from Annexon, personal fees from Appellis, personal fees from Amgen, personal fees from Medscape, personal fees from Physicians Education Resource, personal fees for Data Safety Monitoring board from Abbvie and Celgene, personal fees from Research to Practice, workforce training Sanofi, speaker fees from Teva, Speaker fees from Johnson and Johnson; Speaker fees from Medscape, Speaker fees DAVA oncology; Advisory Board for Pharmacyclics Advisory Board for Proclara; Development of educational materials for i3Healt, Educational Program development i3Health, Royalties from Springer Publishing, Stock Options Aurora Bio

Additional information

Funding

Grant Funding Amyloidosis Foundation; International Waldenstom Foundation NCI SPORE MM SPORE 5P50 CA186781-04.