Temporal Evolution and Management of Fast Flow Vascular Anomalies in PTEN Hamartoma Tumor Syndrome

Int J Angiol DOI: 10.1055/s-0038-1661377Phosphatase and tensin homolog (PTEN) hamartoma tumor syndrome (PHTS) is characterized by formation of recurrent benign tumors described as PTEN hamartoma of soft tissue that may contain fast flow vascular anomalies (FFVA). The purpose of this study is to review the temporal evolution and management of FFVA in PHTS. A retrospective review of 22 patients (9 males), age 1 to 18 (median 9) years diagnosed with PHTS at a tertiary care pediatric hospital between October 2002 and August 2017 revealed 4 patients with FFVA. Imaging, management, and treatment complications were reviewed. During median follow-up of 8 (range: 4–13) years, ultrasound and magnetic resonance imaging performed for recurrent pain, showed progressive increase in the size of hamartomas and development of new FFVA in three-fourth patients. Medical management included pain medications, oral sirolimus, and physical and psychiatric therapy. Surgical excision of hamartoma (n = 1) resulted in recurrence within 3 months. Between 4 and 24 (average 1.5/year) embolizations were performed per patient. Pain related to FFVA responded well to embolization. Pain secondary to PTEN hamartoma responded poorly to percutaneous sclerosant injection, but demonstrated improvement with sirolimus. There was no correlation between serum sirolimus levels and frequency/timing of recurrence of FFVA/hamartoma. Complications included sclerosant migration into digital arteries (n = 1), subcla...
Source: International Journal of Angiology - Category: Cardiology Authors: Tags: Original Article Source Type: research