Intravascular embolisation and surgical resection of a giant neurofibroma with intratumoural haemorrhage

Abstract

ABSTRACT. The use of pre-operative embolisation has been described for small neurofibromas, but not for giant lesions. Advances in embolisation techniques are extending the indications for this procedure, in particular to assist with operative intervention on a range of lesions. This case report describes a 45-year-old male with a giant neurofibroma who underwent embolisation to stabilise intratumoural haemorrhage and to assist with haemostasis during the subsequent surgical resection. Minimal transfusion was required and the patient has made a good recovery. This case demonstrates that pre-operative embolisation of these large and challenging lesions is technically feasible and appears to be beneficial in reducing perioperative blood loss and morbidity. Neurofibromas are common benign soft-tissue tumours arising within peripheral nerves. Solitary neurofibromas are more common, whereas multiple neurofibromas are often associated with neurofibromatosis type 1 (NF-1) [1]. Plexiform neurofibromas rarely grow to be larger than 5 cm; however, giant neurofibromas can occur and are commonly associated with NF-1. Neurofibromas are difficult to manage surgically as they are extensively infiltrative and highly vascular. Complications of neurofibromas are rare, but include malignant change and potentially life-threatening haemorrhage [2].We report a case of haemorrhage into a giant plexiform neurofibroma of the lower back and buttock causing hypovolaemic shock. This was subsequently managed with intravascular embolisation followed by surgical resection, demonstrating that this technique is technically feasible and should be considered for these lesions. Case reportAn otherwise well 45-year-old Caucasian man presented with bleeding from a solitary large mass over the posterior aspect of his right buttock and lumbar region. Although he initially denied it, he later revealed the mass had been present for over 10 years and had gradually enlarged during this time. Recently, the lesion had increased in size rather rapidly and suffered an overlying skin breakdown. He was bleeding from the surface of the lesion and became hypotensive with some renal impairment, which later recovered. He received a transfusion of 12 units of blood during stabilisation prior to transfer to our centre.Upon arriving at our centre, examination revealed a large, tender, soft mass measuring 70 6 50 6 20 cm extending from the thoracolumbar region to the gluteal fold and overhanging the posterior aspect of his thigh. A large café-au-lait spot covered the lesion (Figure 1). He was haemodynamically stable and the lesion was no longer bleeding from the surface.A CT scan revealed a large soft-tissue mass arising from the subcutaneous tissues of the lower back and extending outside of the available field of view. Loss of fat planes between the mass and the erector spinae muscle was suggestive of possible deep muscular invasion (Figure 2a).MRI was technically difficult and limited by motion artefact; much of the gluteal mass was not included on the field of view...

Life Threatening Complications following Excision of Giant Neurofibroma

“…Pre-operative coil embolization was used to reduce the blood flow to the tumor and to limit intraoperative blood loss [29,34,35]. The vessels were embolized proximally to reduce the flow of the major vascular pedicles at the surgical dissection plane and to reduce skin necrosis associated with a distal embolization technique.…”

“…Littlewood and Stilwell [35] embolized a plexiform neurofibroma; however, there was a delay of 1 month before surgical excision resulting in significant perioperative blood loss. Jones et al [34] embolized a giant plexiform neurofibroma prior to surgery, and only four units of packed red cells were required intraoperatively, but the patient had postoperative central areas of skin breakdown that were managed with vacuum-assisted closure dressings.…”

“…Some authors reserve this term for tumors weighing more than 20 kg [20], even though no clear consensus has been reached [34]. Reviewing the literature, we have found that all articles published on "giant" neurofibromas are case reports and in many of these articles, the information submitted is incomplete regarding tumor and patient specifications [41][42][43].…”

Spontaneous Hemorrhage of a Facial Neurofibroma

“…4,8 In the gastrointenstinal tract, bleeding has been reported to occur within a hypervascular mucosal plexiform neurofibroma. 9Y11 Other skin neurofibromas have been reported to bleed, including those arising from the back, 12 the buttock, 13 the scalp, 4 the chest wall, 14 and the sacrum. 15,16 When a malignant transformation and a subsequent vascular invasion are not present, the underlying mechanism is thought to be the NF1-associated vascular dysplasia and the associated fragility.…”

Preoperative Embolization of a Giant Neurofibroma of the Chest in a Patient with Neurofibromatosis Type II: A Case Report

“…Neurofibromas are common benign soft-tissue tumors arising within peripheral nerves, which are very rare in patients with neurofibromatosis type II (NFII) (1). Neurofibromas are classically divided into localized, diffuse, and plexiform types (2).…”