JPRAS Open 5 (2015) 24e28. Contents lists available at ScienceDirect. JPRAS Open. journal homepage:

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1 JPRAS Open 5 (2015) 24e28 Contents lists available at ScienceDirect JPRAS Open journal homepage: jpras-open Case report Life-threatening intratumoral hemorrhage in plexiform neurofibroma: A case report T. Ueno a, *, Y. Takagi a, H. Yamada a, Y. Kanazawa a, H. Ebara a, K. Shimozaki a, H. Tsuchiya b a Department of Orthopedic Surgery, Tonami General Hospital, Shichontomi-cho 1-61, Tonami, Toyama, Japan b Department of Orthopedic Surgery, Graduate School of Medical Science, Kanazawa University, Takaramachi 13-1, Kanazawa, Ishikawa, Japan article info abstract Article history: Received 30 May 2015 Accepted 20 June 2015 Available online 27 June 2015 Keywords: Plexiform neurofibroma Intratumoral hemorrhage von Recklinghausen's disease Neurofibromatosis type 1 Neurofibromatosis type 1 (von Recklinghausen's disease) is an inherited nervous system disease affecting around 1 in 3000 and is characterized by cafe-au-lait spots, pigmented hamartomas of the iris (Lisch nodules), and multiple neurofibromas. Plexiform neurofibromas can show intratumoral bleeding, and the fragile surrounding blood vessels and soft tissues carry a risk of escalation to lethal bleeding. We encountered a 59-year-old man with neurofibromatosis type 1 who presented with a rapidly growing massive hematoma in the left buttock after hitting it against a chair 12 hours earlier. He was tachycardic and hypotensive (hemoglobin, 5.7 g/dl), with severe swelling and tenderness in the left gluteal region. Plain computed tomography revealed a large, hyperdense, subcutaneous soft-tissue mass on the left buttock measuring cm. Selective angiography demonstrated significant arterial supply to the lesion from a highly hypertrophied left L4 lumbar artery as well as the left superior gluteal artery. Embolization of these vessels was performed to reduce tumor blood supply. After returning to the intensive care unit, a central area of skin necrosis was noted. The wound was debrided; healing with scarring after 6 weeks with the tumor returning to its original size within 6 months. Hemorrhage in neurofibromatosis type 1 is attributed to a friable vasculature secondary to arterial dysplasia or vascular invasion by the neurofibroma. As neurofibroma is highly vascular, vessel rupture can occur spontaneously or with trivial trauma. Arterial embolization represents the method of choice for * Corresponding author. Department of Orthopedic Surgery, Tonami General Hospital, Shichontomi-cho 1-61, Tonami, Toyama, , Japan. Tel.: þ address: takuro1006@gmail.com (T. Ueno) / 2015 The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. This is an open access article under the CC BY-NC-ND license (

2 T. Ueno et al. / JPRAS Open 5 (2015) 24e28 25 treating intratumoral bleeding in neurofibromatosis type 1, as a minimally invasive means of controlling arterial bleeding The Authors. Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons. This is an open access article under the CC BY-NC-ND license ( creativecommons.org/licenses/by-nc-nd/4.0/). Introduction Neurofibromatosis type 1 (NF1), also known as von Recklinghausen's disease, is an inherited disease of the nervous system with a frequency of around 1 in This pathology shows three major features: cafe-au-lait spots; pigmented hamartomas of the iris (Lisch nodules); and multiple neurofibromas. The tendency toward bleeding within the plexiform neurofibromas is well recognized, but lifethreatening hemorrhages are rare. Most reported cases have been associated with intrathoracic and gastrointestinal tumors, 2,3 but tumors of the face, chest wall, and sacral region have also been reported. 4e6 Although hemostasis is necessary with intratumoral hemorrhage, surgical procedures are difficult due to the fragile nature of surrounding blood vessels and soft tissues and carry a risk of leading to lethal bleeding. 4,5,7,8 We report the case of a patient with NF1 who experienced life-threatening intratumoral hemorrhage in the left buttock and was successfully treated with intravascular embolization. Case report A 59-year-old man with NF1 presented with a rapidly growing massive hematoma in the left buttock. Twelve hours prior to arrival, the patient felt pain in the region after he had hit his buttock against a chair. He was tachycardic and hypotensive, with a hemoglobin level of 5.7 g/dl. Severe swelling and tenderness were observed on the left buttock region (Figure 1). He received a transfusion of 2 liters of crystalloid fluid and 4 units of packed red blood cells and became hemodynamically stable with a blood pressure of 100/60 mmhg. Plain computed tomography (CT) revealed a large, hyperdense, subcutaneous soft-tissue mass on the left buttock measuring cm (Figure 2). As intratumoral bleeding was suspected, angiography was performed under local anesthesia via the right common femoral artery. Selective angiography demonstrated significant arterial supply to the lesion from a highly hypertrophied left L4 lumbar artery, as well as the left superior gluteal artery as a major branch of the left internal iliac artery (Figure 3). No extravasation of contrast material was identified, but all feeding vessels were tortuous Figure 1. Appearance of the mass lesion on arrival.

3 26 T. Ueno et al. / JPRAS Open 5 (2015) 24e28 Figure 2. Plain CT showing a large, hyperdense subcutaneous soft-tissue mass on the buttock measuring cm. Figure 3. Selective angiography demonstrating significant arterial supply to the tumor from a large left hypertrophied L4 lumbar artery and left superior gluteal artery. and hypertrophied, indicating a rich blood supply. Embolization of the left L4 artery and left superior gluteal artery was performed with the purpose of reducing blood supply to the tumor. Trisacryl gelatin microspheres (Embosphere ; BioSphere Medical, Roissy-en-France, France) were used as an embolic material. After the procedure, the patient returned to the intensive care unit with a hemoglobin level of 9.1 g/ dl, where no significant worsening of anemia was seen. Subsequently, a central area of skin necrosis occurred. The wound was left open, debrided and treated with dressing changes, and healed with scarring after 6 weeks. The hematoma gradually reduced in size and at the 6-month follow-up, the tumor had returned to its original size seen before the trauma (Figure 4). Discussion Life-threatening intratumoral hemorrhage is uncommon in NF1, but has been reported. 4e9 Hemorrhage in NF1 has been thought to result from a friable vasculature secondary to arterial dysplasia or vascular invasion by the neurofibroma. 5 The tumor tissue itself also has an abnormal vascular structure with thin-walled ecstatic blood vessels lying in loose neural stroma that replaces normal adipose tissue. 8 In addition to the vascular fragility, neurofibroma shows highly vascular structures, so vessel rupture can occur spontaneously or with trivial trauma. In either event, commencement of

4 T. Ueno et al. / JPRAS Open 5 (2015) 24e28 27 Figure 4. Appearance of the hematoma, which gradually reduced in size. a) Two weeks after admission. A central area of skin necrosis required debridement and dressing changes. b) Six weeks after admission. The wound has healed and the patient was discharged. c) At the 6-month follow-up. The tumor has reduced to its original size. intratumoral bleeding leads to a cascade of rupturing vessels, followed by more bleeding within the very friable tumor mass, causing yet more vessels to rupture. 4 Urgent hemostasis is needed if the bleeding is leading toward a lethal condition. Pressure hemostasis by external compression is difficult to achieve in cases involving bleeding from soft tissues close to the body surface due to the fragile nature of the skin and connective tissues in NF1. 7 Surgical procedures have been reported. 4e6,8 Tumor excision and ligation represent viable options for treatment, but open surgery is invasive and hemostasis is often difficult to achieve using sutures because of the fragile nature of the vascular tissue in NF1. 7,9 Furthermore, even in successful cases, massive amounts of blood transfusion have often been required during and after the operation. 4,5,8 Recently, arterial embolization has become the method of choice for treating intratumoral bleeding in NF1. 6,7,9,10 as a minimally invasive means of controlling arterial bleeding. In this case report, angiography showed no extravasation of contrast material that would have indicated arterial bleeding. However, all feeding vessels were tortuous and hypertrophied, indicating a rich blood supply to the tumor. Embolization of the left L4 artery and left superior gluteal artery was therefore performed. We used trisacryl gelatin microspheres as a permanent option in case temporary embolic materials had a chance of allowing recurrent bleeding. The patient showed central skin necrosis of the tumor after embolization, but the wound healed with scarring after 6 weeks. Conclusion We have reported the case of an NF1 patient who experienced life-threatening intratumoral hemorrhage. Embolization should be considered for treatment as a less-invasive option compared to surgical procedures. Conflict of interest statement None. Acknowledgments The authors wish to thank Dr. Ryu Yasuji, Department of Radiology, Tonami General Hospital, who performed the angiographic studies and embolization; and Dr.Teruo Ueno, Department of Plastic Surgery, Tonami General Hospital, for helping with wound treatment.

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