Giant Extrapleural Solitary Fibrous Tumor of the Thigh

489 healing, smooth muscle loss, and fibrosis [1,2]. An article reported that the most common vasculopathy in NF-I patients is isolated renal artery disease, followed by mesenteric artery stenosis/aneurysm and aortic dissection. AVM does not occur as frequently, but most cases arise in the cervical vertebral artery and lumbar vertebral artery, and an occurrence in the trunk, extremities, and face is very rare [3]. Systemic manifestations from this vascular pathology, such as hypovolemic shock, are rare, but bleeding is difficult to control once it has started. NF-1 vasculopathy conditions such as aneurysm or psuedoaneurysm are generally treated with transcatheter embolization of the feeding artery, which is usually limited to one or two major vessels. However, AVM has multiple tortuous vessels that feed the mass, which makes vascular intervention difficult. Moreover, in the case that the mass is superficially located and the feeding vessel is too small to perform selective embolization, a surgical approach is preferred. Although the pathologic diagnosis was not AVM in our case, the massive bleeding nature and clinical diagnosis from the preoperative evaluation included a differential diagnosis of AVM. Based on the finding that the inguinal portion and the pelvic portion of the mass were septated, a bimodal approach combining selective embolization of the pelvic mass and surgical excision of the inguinal portion of the mass was successfully performed. Here, we have reported successful treatment of a severe case of hypervascular neurofibroma with intractable bleeding, which was successfully controlled with selective embolization and surgical removal followed by reconstruction with a free flap. Moreover, because a hypervascular neurofibroma has clinical features similar to those of an AVM, our bimodal approach could be a good option for the treatment of a huge AVM that is caused as a complication of neurofibromatosis.