Giant cell angioblastoma in the femur: three additional pediatric cases provide more data of its clinicopathological features

Objective: To study the clinical and pathological features of giant cell angioblastoma in children. Method: The clinical and pathological data of 3 pediatric cases of giant cell angioblastoma were retrieved and reviewed from the medical records in Children’s Hospital of Fudan University. Histological and immunohistochemical analysis was made. Results: There were 2 boys and 1 girls. The age was 6 years, 1 years, and 7 years respectively. The tumors were all located in the femur. The size of lesion was between 1 cm to 2 cm in maximum diameter. The main complaint included joint dysfunction, unsteady gait, and leg length discrepancy. Histologically, all the three cases showed the same morphological features: onion-skin nodules, multinucleated giant cells, and fibrocollagenous stroma. The tumor stroma could vary from the abundant fibroblasts and myxoid area to the totally collagen according to the different age. Immunohistochemically, they had the same immunoprofile. The spindle to oval mononuclear tumor cells expressed variably markers of vascular endothelium such as CD34, CD31, and Factor VIII. The multinucleated giant cells were positive for CD68. All the patients received tumor resection and two of the tumors recurred in the follow-up interval. Conclusion: Giant cell angioblastoma is a rare vascular tumor. It has a typical morphological feature and unique immunophenotype. All the data show it may be an intermediate tumor for its invasive growth pattern and repeatedly recurrence.