Emergency management and perioperative strategies for intra-tumoral hemorrhage in neurofibromatosis type 1-related giant plexiform neurofibromatosis_Chinese Journal of Reparative and Reconstructive Surgery

Authors：

ZHU Baochen , XIAO Yang , LIAO Ruiheng , ZHAO Hanxing , XU Xuewen ,  ZHANG Yange

Department of Burns and Plastic Surgery, West China Hospital, Sichuan University, Chengdu Sichuan, 610041, P. R. China;

Corresponding author：

ZHANG Yange, Email: Zhangyange7801@163.com

Keywords：

Giant plexiform neurofibroma; neurofibromatosis type 1; rupture hemorrhage; arterial embolism; surgical excision

DOI：

10.7507/1002-1892.202406074

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Objective To review the emergency management and perioperative strategies for ruptured neurofibromatosis type 1 (NF1)-related giant plexiform neurofibromatosis (pNF), providing a systematic treatment protocol to enhance effectiveness and patient quality of life. Methods The literature on the management of giant pNF rupture hemorrhages was reviewed and the diagnosis, treatment and perioperative management of giant pNF rupture and hemorrhage were summarized based on clinical experience. Results By implementing an integrated diagnostic and treatment strategy that includes early diagnosis, imaging evaluation, emergency ultra-selective arterial embolization combined with surgical excision, not only can effectively controls acute hemorrhage but also reduces the risk of major intraoperative bleeding and minimizes postoperative complications. As a result, it significantly improves treatment success rates and patient quality of life. Conclusion For ruptured NF1-related giant pNF, employing emergency ultra-selective arterial embolization combined with surgical excision, under the collaboration of a multidisciplinary team, can effectively enhance the success rate of treatment, reduce recurrence rates, and lower mortality. Future research should focus on assessing the long-term quality of life post-treatment for patients with ruptured giant pNF and on further optimizing treatment protocols.

1.	Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol, 2007, 6(4): 340-351.
2.	朱以诚. Ⅰ型神经纤维瘤病多学科诊治指南 (2023版). 罕见病研究, 2023, 2(2): 210-230.
3.	Chang JB, Graver LM. Regarding “Vascular abnormalities in patients with neurofibromatosis syndrome type Ⅰ: Clinical spectrum, management, and results”. J Vasc Surg, 2008, 47(6): 1378-1379.
4.	徐潇, 周桂文, 陈康, 等. 超选择动脉栓塞联合外科序贯治疗Ⅰ型神经纤维瘤伴巨大神经纤维瘤临床疗效观察. 中国美容医学, 2022, 31(1): 9-12.
5.	Fisher MJ, Blakeley JO, Weiss BD, et al. Management of neurofibromatosis type 1-associated plexiform neurofibromas. Neuro Oncol, 2022, 24(11): 1827-1844.
6.	张璠, 陈尚雄, 张彬, 等. 遵循“AISS”策略精准治疗躯干部巨大神经纤维瘤. 中华整形外科杂志, 2024, 40(2): 179-186.
7.	Leier CV, DeWan CJ, Anatasia LF. Fatal hemorrhage as a complication of neurofibromatosis. Vasc Surg, 1972, 6(2): 98-101.
8.	Carrington GL. Hemorrhage in a case of von Recklinghausen’s disease. JAMA, 1926, 87(3): 166-167.
9.	Heuer GJ, Bell HG. Hamorrhage into and infection of large tumors (pachydermatoceles) of von recklinghausen’s disease. Annals of Surgery, 1931, 94(1): 15-24.
10.	Seitz IA, Song DH, Schechter LS, et al. Giant neurofibroma of the trunk: Surgical approach and pathology. Plastic and Reconstructive Surgery, 2006, 118(Supple): 122-123.
11.	Wei W, Yang L, Zhu Y, et al. Massive hemorrhage following acupuncture treatment in a neurofibromatosis type 1 patient. Cureus, 2023, 15(10): e47825. doi: 10.7759/cureus.47825.
12.	Stephen E, Kariyattil R, Mittal A, et al. Spontaneous near fatal hemorrhage into neurofibromatosis type 1 lesion in the scalp. Oman Med J, 2022, 37(3): e387. doi: 10.5001/omj.2022.02.
13.	Feng Y, Yang ZG, Chen T, et al. Giant plexiform neurofibroma with hemorrhage in cranio-maxillofacial region as depicted on CT and MRI. Eur J Med Res, 2010, 15(2): 84-87.
14.	Michimoto K, Ashida H, Higuchi T, et al. Hemorrhagic complication in surgical resection for massive plexiform neurofibroma in body trunk: The flow-void sign as a predictor and preoperative embolization as prevention. World J Surg, 2021, 45(12): 3603-3608.
15.	Rao V, Affifi RA, Ghazarian D. Massive subcutaneous hemorrhage in a chest-wall neurofibroma. Can J Surg, 2000, 43(6): 459-460.
16.	Naunton RF, Hemenway WG. A case of neurofibromatosis presenting as an acute surgical emergency. Arch Otolaryngol, 1960, 72: 369-375.
17.	Tak MS, Cho SE, Kang SG, et al. Management of giant facial neurofibroma with intratumoral hematoma in neurofibromatosis type 1 patient. J Craniofac Surg, 2016, 27(6): 1521-1523.
18.	Miller J, Perotti V, Moore P. Massive spontaneous haemorrhage in a plexiform neurofibroma: A case report and discussion of the literature. J Clin Neurosci, 2020, 80: 229-231.
19.	Tomei KL, Gupta V, Prestigiacomo CJ, et al. Spontaneous hemorrhage of a facial neurofibroma: endovascular embolization before surgical intervention. J Craniofac Surg, 2013, 24(5): e514-e517.
20.	Azhar AF, Bittle JSH, Kwarcinski TJ, et al. Embolization of a hemorrhaging abdominal plexiform neurofibroma. Proc (Bayl Univ Med Cent), 2020, 33(3): 448-450.
21.	Gao Q, Qu S, Ma N, et al. Risk factors for intraoperative hemorrhage of type Ⅰ neurofibromatosis. BMC Surg, 2023, 23(1): 157. doi: 10.1186/s12893-023-02067-7.
22.	Lin YC, Chen HC. Rare complication of massive hemorrhage in neurofibromatosis with arteriovenous malformation. Ann Plast Surg, 2000, 44(2): 221-224.
23.	Sawada Y. Hemorrhage into a plexiform neurofibroma induced by trauma: a rare complication of von Recklinghausen’s disease. J Dermatol, 1987, 14(4): 378-381.
24.	Littlewood AH, Stilwell JH. The vascular features of plexiform neurofibroma with some observations on the importance of pre-operative angiography and the value of pre-operative intra-arterial embolisation. Br J Plast Surg, 1983, 36(4): 501-506.
25.	Lee SM, Leem DH. Facial hematoma induced spontaneously or by minimal trauma in a facial plexiform neurofibroma: a case report and literature review. J Korean Assoc Oral Maxillofac Surg, 2023, 49(3): 152-156.
26.	Cavallaro G, Pedullà G, Crocetti D, et al. Vacuum-assisted closure treatment of leg skin necrosis after angiographic embolization of a giant plexiform neurofibroma. G Chir, 2012, 33(6-7): 239-242.
27.	Ross AL, Panthaki Z, Levi AD. Surgical management of a giant plexiform neurofibroma of the lower extremity. World Neurosurg, 2011, 75(5-6): 754-757.
28.	Washington EN, Placket TP, Gagliano RA, et al. Diffuse plexiform neurofibroma of the back: report of a case. Hawaii Med J, 2010, 69(8): 191-193.
29.	Sekiyama H, Nobeyama Y, Nakagawa H. Successful treatment by negative-pressure wound therapy for ulcer located on diffuse plexiform neurofibroma. J Dermatol, 2017, 44(12): e313-e314.
30.	Zhang Y, Tong Y, Chen X, et al. Rapidly expanding giant neurofibroma of the back with intratumoral hematoma: a case report and literature review. Br J Neurosurg, 2023, 37(6): 1709-1713.
31.	Hasibuan LY, Putri AC, Pramono GNTW. Case report: Remarkable efficacy of negative-pressure wound therapy in giant lower extremity elephantiasis neuromatosa for vascularization, skin grafting, and fluid control. Int J Surg Case Rep, 2024, 116: 109428. doi: 10.1016/j.ijscr.2024.109428.
32.	Xiao Y, Zhao H, Zhang Y, et al. Role of embolization therapy followed by debulking surgery in the treatment of giant neurofibroma with spontaneous hemorrhage. Asian J Surg, 2024. doi: 10.1016/j.asjsur.2024.06.032.

1. Ferner RE. Neurofibromatosis 1 and neurofibromatosis 2: a twenty first century perspective. Lancet Neurol, 2007, 6(4): 340-351.
2. 朱以诚. Ⅰ型神经纤维瘤病多学科诊治指南 (2023版). 罕见病研究, 2023, 2(2): 210-230.
3. Chang JB, Graver LM. Regarding “Vascular abnormalities in patients with neurofibromatosis syndrome type Ⅰ: Clinical spectrum, management, and results”. J Vasc Surg, 2008, 47(6): 1378-1379.
4. 徐潇, 周桂文, 陈康, 等. 超选择动脉栓塞联合外科序贯治疗Ⅰ型神经纤维瘤伴巨大神经纤维瘤临床疗效观察. 中国美容医学, 2022, 31(1): 9-12.
5. Fisher MJ, Blakeley JO, Weiss BD, et al. Management of neurofibromatosis type 1-associated plexiform neurofibromas. Neuro Oncol, 2022, 24(11): 1827-1844.
6. 张璠, 陈尚雄, 张彬, 等. 遵循“AISS”策略精准治疗躯干部巨大神经纤维瘤. 中华整形外科杂志, 2024, 40(2): 179-186.
7. Leier CV, DeWan CJ, Anatasia LF. Fatal hemorrhage as a complication of neurofibromatosis. Vasc Surg, 1972, 6(2): 98-101.
8. Carrington GL. Hemorrhage in a case of von Recklinghausen’s disease. JAMA, 1926, 87(3): 166-167.
9. Heuer GJ, Bell HG. Hamorrhage into and infection of large tumors (pachydermatoceles) of von recklinghausen’s disease. Annals of Surgery, 1931, 94(1): 15-24.
10. Seitz IA, Song DH, Schechter LS, et al. Giant neurofibroma of the trunk: Surgical approach and pathology. Plastic and Reconstructive Surgery, 2006, 118(Supple): 122-123.
11. Wei W, Yang L, Zhu Y, et al. Massive hemorrhage following acupuncture treatment in a neurofibromatosis type 1 patient. Cureus, 2023, 15(10): e47825. doi: 10.7759/cureus.47825.
12. Stephen E, Kariyattil R, Mittal A, et al. Spontaneous near fatal hemorrhage into neurofibromatosis type 1 lesion in the scalp. Oman Med J, 2022, 37(3): e387. doi: 10.5001/omj.2022.02.
13. Feng Y, Yang ZG, Chen T, et al. Giant plexiform neurofibroma with hemorrhage in cranio-maxillofacial region as depicted on CT and MRI. Eur J Med Res, 2010, 15(2): 84-87.
14. Michimoto K, Ashida H, Higuchi T, et al. Hemorrhagic complication in surgical resection for massive plexiform neurofibroma in body trunk: The flow-void sign as a predictor and preoperative embolization as prevention. World J Surg, 2021, 45(12): 3603-3608.
15. Rao V, Affifi RA, Ghazarian D. Massive subcutaneous hemorrhage in a chest-wall neurofibroma. Can J Surg, 2000, 43(6): 459-460.
16. Naunton RF, Hemenway WG. A case of neurofibromatosis presenting as an acute surgical emergency. Arch Otolaryngol, 1960, 72: 369-375.
17. Tak MS, Cho SE, Kang SG, et al. Management of giant facial neurofibroma with intratumoral hematoma in neurofibromatosis type 1 patient. J Craniofac Surg, 2016, 27(6): 1521-1523.
18. Miller J, Perotti V, Moore P. Massive spontaneous haemorrhage in a plexiform neurofibroma: A case report and discussion of the literature. J Clin Neurosci, 2020, 80: 229-231.
19. Tomei KL, Gupta V, Prestigiacomo CJ, et al. Spontaneous hemorrhage of a facial neurofibroma: endovascular embolization before surgical intervention. J Craniofac Surg, 2013, 24(5): e514-e517.
20. Azhar AF, Bittle JSH, Kwarcinski TJ, et al. Embolization of a hemorrhaging abdominal plexiform neurofibroma. Proc (Bayl Univ Med Cent), 2020, 33(3): 448-450.
21. Gao Q, Qu S, Ma N, et al. Risk factors for intraoperative hemorrhage of type Ⅰ neurofibromatosis. BMC Surg, 2023, 23(1): 157. doi: 10.1186/s12893-023-02067-7.
22. Lin YC, Chen HC. Rare complication of massive hemorrhage in neurofibromatosis with arteriovenous malformation. Ann Plast Surg, 2000, 44(2): 221-224.
23. Sawada Y. Hemorrhage into a plexiform neurofibroma induced by trauma: a rare complication of von Recklinghausen’s disease. J Dermatol, 1987, 14(4): 378-381.
24. Littlewood AH, Stilwell JH. The vascular features of plexiform neurofibroma with some observations on the importance of pre-operative angiography and the value of pre-operative intra-arterial embolisation. Br J Plast Surg, 1983, 36(4): 501-506.
25. Lee SM, Leem DH. Facial hematoma induced spontaneously or by minimal trauma in a facial plexiform neurofibroma: a case report and literature review. J Korean Assoc Oral Maxillofac Surg, 2023, 49(3): 152-156.
26. Cavallaro G, Pedullà G, Crocetti D, et al. Vacuum-assisted closure treatment of leg skin necrosis after angiographic embolization of a giant plexiform neurofibroma. G Chir, 2012, 33(6-7): 239-242.
27. Ross AL, Panthaki Z, Levi AD. Surgical management of a giant plexiform neurofibroma of the lower extremity. World Neurosurg, 2011, 75(5-6): 754-757.
28. Washington EN, Placket TP, Gagliano RA, et al. Diffuse plexiform neurofibroma of the back: report of a case. Hawaii Med J, 2010, 69(8): 191-193.
29. Sekiyama H, Nobeyama Y, Nakagawa H. Successful treatment by negative-pressure wound therapy for ulcer located on diffuse plexiform neurofibroma. J Dermatol, 2017, 44(12): e313-e314.
30. Zhang Y, Tong Y, Chen X, et al. Rapidly expanding giant neurofibroma of the back with intratumoral hematoma: a case report and literature review. Br J Neurosurg, 2023, 37(6): 1709-1713.
31. Hasibuan LY, Putri AC, Pramono GNTW. Case report: Remarkable efficacy of negative-pressure wound therapy in giant lower extremity elephantiasis neuromatosa for vascularization, skin grafting, and fluid control. Int J Surg Case Rep, 2024, 116: 109428. doi: 10.1016/j.ijscr.2024.109428.
32. Xiao Y, Zhao H, Zhang Y, et al. Role of embolization therapy followed by debulking surgery in the treatment of giant neurofibroma with spontaneous hemorrhage. Asian J Surg, 2024. doi: 10.1016/j.asjsur.2024.06.032.

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