Hemangioma in Kidney with End-Stage Renal Disease: A Novel Association
Susan L Haley, Oleksandr N Kryvenko, Manju Aron, Steven S Shen, Jonathan I Epstein, Nilesh S Gupta, Mahul Amin, Luan D Truong. The Methodist Hospital, Houston, TX; Cornell University, New York, NY; Cedars Sinai Medical Center, Los Angeles, CA; Henry Ford Medical Center, Detroit, MI; The John Hopkins University, Baltimore, MD
Background: Tumors often develop in kidneys with end-stage renal disease (ESRD), almost all of which are epithelial neoplasms. Renal hemagioma associated with ESRD has been recently described, but this condition remains largely unknown.
Design: Hemagiomas in 10 nephrectomy specimens from 8 patients from three institutions were studied.
Results: All 8 patients had ESRD due to lupus nephritis (3), hypertension (2), diabetes (1), focal segmental glomerulosclerosis (1), or unknown cause (1). Seven patients were on dialysis, from 1.1 to 31 years. Hemangiomas were detected on surveillance renal imaging (8 kidneys) or incidentally (in 1 kidney with a mass on CT suspicious for renal cell carcinoma (RCC); and in 1 kidney with imaging features of acquired cystic kidney disease [ACK]). Symptoms directly related to hemangioma were not seen in any patient. Bilateral hemangiomas were noted in two patients. Tumors measured 0.1 to 2.8 cm, significantly smaller than their imaging sizes. Hemangiomas appeared as a single mass in 7 kidneys; two masses (one intrarenal and one in sinus fat) in 1; at least four intrarenal masses in 1; and multiple masses throughout the kidney, sinus fat, and hilar lymph node in 1. Almost all tumors were in the medulla and often abutted on sinus fat. All tumor masses demonstrated isolated or interconnected capillary-sized vascular channels lined by a single layer of benign cuboidal CD34+, CD31+, D2-40 - endothelial cells, separated by a cellular stroma that molded to the contour of the vascular channels and contained smooth muscle actin positive cells. Most had no capsule, and none demonstrated necrosis or increased mitoses. Extramedullary hematopoiesis was noted in each tumor. The background tissue showed changes of ESRD in all 10 kidneys, ACK in 5, ACK-related inflammatory/hemorrhagic masses in 2, RCCs in 2, and incidental papillary adenomas in 1. There was no recurrence at 1-36 months of follow-up.
Conclusions: Renal hemangioma may represent a novel type of neoplasm that develops against the background of ESRD, with or without ACK. It has a uniform and disctinctive morphology amenable to diagnostic accuracy. Its pathogenesis and the basis for its recent emergence remain unknown, but its characteristic clinicopathologic profile points to ESRD-related angiogenic factors affecting a localized vascular bed in the kidney with ESRD.
Category: Genitourinary (including renal tumors)
Wednesday, March 21, 2012 9:30 AM
Poster Session V # 83, Wednesday Morning