CLOVES Syndrome
Published in Dongyou Liu, Handbook of Tumor Syndromes, 2020
Dongyou Liu
Treatment options for CLOVES syndrome and other PIK3CA-related overgrowth disorders include surgical debulking (for truncal lipomatous mass), orthopedic care (for scoliosis/kyphosis and leg-length discrepancy), neurosurgical intervention (for obstructive hydrocephalus, increased intracranial pressure, progressive/symptomatic cerebellar tonsillar ectopia, Chiari malformation, and epilepsy), and other procedures (for cardiac and renal abnormalities; intellectual disabilities, behavior problems, motor difficulties, speech, swallowing, and feeding difficulties). Use of PI3K inhibitor (pictilisib, copanlisib, duvelisib), Akt inhibitor (ipatasertib, MK-2206, ARQ-092), mTOR inhibitor (rapamycin), and dual PI3K/AKT/mTOR inhibitor (gedatolisib, apitolisib) offers another approach for management of PIK3CA-related overgrowth spectrum [25].