Transchateter arterial chemoinfusion (TACI) in patient with giant neurofibromatosis

• DOI: 10.1016/j.jns.2019.10.981
• Publication Date: 2019-12-26T16:06:35Z
• Authors: C. Thursina Srie ...
• Keywords: 03 medical and health sciences; 0302 clinical medicine; 3. Good health

Background Giant neurofibromatosis (NF1) is an autosomal dominant genetic disorder with an incidence of 1 in 3000 live births. It has distinct cafe-au-lait lesion, optical glioma, plexiform neurofibroma and peripheral nerve sheet tumor. Transchateter arterial chemoinfusion (TACI) was an invasive procedure for administration of the chemotherapy drug after targeted embolization. Case report 32 years oldmale presenting with multiple skin nodules followed with progressive swelling giant size of the left leg for 12 years. Patient also had uncontrolled absence epilepsy with and abnormal irritative diffuse pattern of electroencephalogram (EEG) and normal head computed tomography (CT) scan. Inoperable NF1 was concluded by clinical manifestation and left leg CT scan. This patient is planning for 7 times of TACI with interval procedure 1 until 2 months. The size of the diameter of left leg decrease for 11.8cm, decreasing pain from numeric pain scale (NPS) 7-8 to NPS 1-2 after the 3rdTACI. Discussion Chemotherapy drug administration with TACI was an alternative therapy for inoperable NF1 patient. This is the first case in Yogyakarta Dr. Sardjito General Hospital and need further follow-up for 1 years after the first TACI. Conclusion Inoperable NF1 was treated with TACI for 3 times from 7 times planning, and have good clinical outcomes.