Israel Medical Association Journal

Klippel-Trenaunay syndrome (KTS) is a rare congenital complex vascular multisystem disorder characterized by bony and soft-tissue hypertrophy. It is famous for its hallmarks like port-wine stains and varicose veins. The syndrome is sporadic, although rare familial cases have been reported [1]. The most common ophthalmological alterations encountered in KTS are conjunctival telangiectasia, anterior chamber malformation, raised episcleral venous pressure with associated glaucoma, and choroidal hemangiomas [2].

The purpose of this report is to raise awareness of KTS and its diverse scale of expressions as well as complications. This study was conducted in accordance with the ethical standards set by the Declaration of Helsinki. The patient gave signed informed consent.

Abstract

Background: Renal hemangiomas are rare benign tumors seldom distinguished from malignant tumors preoperatively.

Objectives: To describe the Memorial Sloan-Kettering Cancer Center (MSKCC) experience with diagnosing and treating renal hemangiomas, and to explore possible clinical and radiologic features that can aid in diagnosing renal hemangiomas preoperatively.

Methods: Patients with renal hemangiomas treated at MSKCC were identified in our prospectively collected renal tumor database. Descriptive statistics were used to describe the patient characteristics and the tumor characteristics. All available preoperative imaging studies were reviewed to assess common findings and explore possible characteristics distinguishing benign hemangiomas from malignant renal tumors preoperatively.

Results: Of 6341 patients in our database 15 were identified. Eleven (73%) were males, median age at diagnosis was 53.3 years, and the affected side was evenly distributed. All but two patients were treated surgically. The mean decrease in estimated glomerular filtration rate (eGFR) after surgery was 36.3%; one patient had an abnormal presurgical eGFR and only two patients had a normal eGFR after surgery. We could not identify radiographic features that would make preoperative diagnosis certain, but we did identify features characteristic of hepatic hemangiomas that were also present in some of the renal hemangiomas.

Conclusions: Most renal hemangiomas cannot be distinguished from other common renal cortical tumors preoperatively. In select cases a renal biopsy can identify this benign lesion and the deleterious effects of extirpative surgery can be avoided.