[Hematuria of papillary or medullar origin: a difficult diagnosis entity]

Renal haemangiomas of renal papillary or medullar origin are a difficult diagnosis entity, being one of the most frequent processes of chronic episodes of hematuria, secondary to benign disease, mainly in young patients. The objective of this paper is to show the difficulty of this diagnosis and the need to suspect it in cases with clinical history similar to the ones described in these cases.

We report 4 cases of spontaneous hematuria of renal origin, with clinical presentation as renal colic, from the historical case series of the Fundacion Jimenez Díaz-Capio, the last two from the years 2005-6. We present the diagnostic and therapeutic methodology employed, including angio-CT and flexible ureterorenoscopy (URS) as well as various treatment options.

Hematuria was identified as "essential" when any relation with tumor or lithiasic pathologies was ruled out, and of renal origin when the source was clearly pointed. We interpreted it was related to angiomas or microangiomas of papillary or medullar origin. In one case, the vascular malformation was interpreted as an arterial venous fistula (AVF) at that level. Hematuria stopped spontaneously in two cases after exploratory URS. The eldest historical case required surgical expiration of the caliceal structures.

Years ago, following the professional development of Urology as speciality, conventional surgery was carried out in all these cases, of very difficult diagnosis, with a very small number of cases undergoing a conservative approach based on the examination of renal cavities trying to observe and find the bleeding point. Most cases underwent complete or partial nephrectomy. Currently, the possibility of exploration of all renal cavities with the flexible ureterorenoscope enables a better diagnosis of the lesions and a more conservative treatment. The ultimate diagnosis of renal papillary angioma is the pathologic diagnosis, without pathognomonic data in the imaging tests. This pathology is thought of at the end of the diagnostic workup, and when the papillary area is identified as the source of bleeding. The historical case series, with the pathologic findings from nephrectomy specimens, permits us to point out this entity as papillary angioma, in patients with similar clinical presentation.