Lela S Mansoori1, Colin P Catel, Micol S Rothman. 1. Department of Medicine, University of Colorado at Denver and Health Sciences Center, Aurora, Colorado 80045 , USA. lela.mansoori@ucdenver.edu
Abstract
OBJECTIVE: To report the case of a patient with polyostotic fibrous dysplasia of the cranium who showed dramatic improvement after treatment with intravenous zoledronic acid. METHODS: We present the clinical findings, laboratory test results, surgical pathology report, and imaging studies of a man with extensive fibrous dysplasia of the cranium and review the literature regarding the use of bisphosphonates in patients with this debilitating skeletal disorder. RESULTS: A 32-year-old man presented with chronic occipital headache, and computed tomography of the head revealed extensive bony lesions of the middle and posterior cranial fossa. Bone biopsy confirmed the diagnosis of fibrous dysplasia. Laboratory blood test results revealed elevated serum alkaline phosphatase (140 U/L [reference range, 39-117 U/L]) and elevated serum bone-specific alkaline phosphatase (30.6 μg/L [reference range, 6-20 μg/L]). The patient was treated with intravenous pamidronate without improvement, and therapy was switched to zoledronic acid, which resulted in rapid resolution of headache symptoms, decrease in bone-specific alkaline phosphatase levels to 24.9 μg/L, and dramatic radiologic improvement on repeated computed tomography of the head. CONCLUSIONS: The treatment options for fibrous dysplasia of the cranium have been limited to conservative follow-up or surgery, and the use of zoledronic acid in this condition has not been previously reported. Intravenous bisphosphonate therapy is suggested to be a useful option in the treatment of cranial fibrous dysplasia.
OBJECTIVE: To report the case of a patient with polyostotic fibrous dysplasia of the cranium who showed dramatic improvement after treatment with intravenous zoledronic acid. METHODS: We present the clinical findings, laboratory test results, surgical pathology report, and imaging studies of a man with extensive fibrous dysplasia of the cranium and review the literature regarding the use of bisphosphonates in patients with this debilitating skeletal disorder. RESULTS: A 32-year-old man presented with chronic occipital headache, and computed tomography of the head revealed extensive bony lesions of the middle and posterior cranial fossa. Bone biopsy confirmed the diagnosis of fibrous dysplasia. Laboratory blood test results revealed elevated serum alkaline phosphatase (140 U/L [reference range, 39-117 U/L]) and elevated serum bone-specific alkaline phosphatase (30.6 μg/L [reference range, 6-20 μg/L]). The patient was treated with intravenous pamidronate without improvement, and therapy was switched to zoledronic acid, which resulted in rapid resolution of headache symptoms, decrease in bone-specific alkaline phosphatase levels to 24.9 μg/L, and dramatic radiologic improvement on repeated computed tomography of the head. CONCLUSIONS: The treatment options for fibrous dysplasia of the cranium have been limited to conservative follow-up or surgery, and the use of zoledronic acid in this condition has not been previously reported. Intravenous bisphosphonate therapy is suggested to be a useful option in the treatment of cranial fibrous dysplasia.
Authors: Xuefeng Zhao; Peng Deng; Ramiro Iglesias-Bartolome; Panomwat Amornphimoltham; Dana J Steffen; Yunyun Jin; Alfredo A Molinolo; Luis Fernandez de Castro; Diana Ovejero; Quan Yuan; Qianming Chen; Xianglong Han; Ding Bai; Susan S Taylor; Yingzi Yang; Michael T Collins; J Silvio Gutkind Journal: Proc Natl Acad Sci U S A Date: 2017-12-27 Impact factor: 11.205