Large intramuscular plexiform neurofibroma of thigh: a multidisciplinary team approach

Authors

  • Aminda Mandal Department of Plastic Surgery, Bokaro General Hospital, Bokaro Steel City, Jharkhand, India
  • Satyam Kumar Department of Orthopaedics, Bokaro General Hospital, Bokaro Steel City, Jharkhand, India
  • Avinash K. Sinha Department of Plastic Surgery, Bokaro General Hospital, Bokaro Steel City, Jharkhand, India
  • Deepak Kumar Department of Orthopaedics, Bokaro General Hospital, Bokaro Steel City, Jharkhand, India
  • Prabhat K. Dwivedi Department of Anaesthesia, Bokaro General Hospital, Bokaro Steel City, Jharkhand, India
  • Nikesh D. Panchbhai Department of Orthopaedics, Bokaro General Hospital, Bokaro Steel City, Jharkhand, India
  • Lavleen Pandey Department of Surgery, Bokaro General Hospital, Bokaro Steel City, Jharkhand, India

DOI:

https://doi.org/10.18203/issn.2455-4510.IntJResOrthop20204603

Keywords:

Neurofibromatosis, Plexiform neurofibroma, Schwannoma, Intramuscular, Von Recklinghausen

Abstract

Large plexiform neurofibroma of lower extremity involving the muscle is a rare entity. In this article we present the case of plexiform neurofibroma of right thigh involving the muscular plane and entrapment of the sciatic nerve. A 28 year lady presented with a plexiform neurofibroma of right thigh of size 60 cm×30 cm×25 cm with a positive family history of neurofibromatosis type 1. MRI was done for the assessment of the tumour and the adjacent structure involvement. The neurofibroma was removed with a multidisciplinary team approach with an intensive peri operative management. The pathological diagnosis was plexiform neurofibroma with diffuse neurofibroma. We have reported a rare case of large plexiform neurofibroma of lower extremity with muscle involvement and nerve entrapment.

References

Freeman BJC, Lavy C. Spine, Bailey & Love’s Short Practice of Surgery. 27th ed. Great Britain: CRC Press; 2018;481.

Ferner RE, Gutmann DH. International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis. Cancer Research. 2002;62(5):1573-7.

Reddy PRK, Rajashekar S, Samagandi S. Giant plexiform neurofibroma of left gluteal and thigh region: a case report. JEMDS. 2015;4(16):2813-7.

Gutmann DH, Aylsworth A, Carey JC, Korf B, Marks J, Pyeritz RE, et al. The diagnostic evaluation and multidisciplinary management of neurofibromatosis and neurofibromatosis 2. Journal of the American Medical Association. 1997;278(1): 51-7.

Le LQ, Parada LF. Tumor microenvironment and neurofibromatosis type I: connecting the GAPs. Oncogene. 2007;26(32):4609-16.

Greenbaum AR, Chan CLH. Skin and subcutaneous tissue, Bailey & Love’s Short Practice of Surgery. 27th ed. Great Britain: CRC Press; 2018;594-5.

Woodruff JM. Pathology of tumors of the peripheral nerve sheath in type neurofibromatosis. Am J Med Genet. 1999;89:23-30.

Mukherji MM. Giant neurofibroma of the head and neck. Plast Reconstr Surg. 1974;53(2):184-9.

Korf BR. Malignancy in neurofibromatosis type 1. Oncologist. 2000;5(6):477-85.

Vélez R, Barrera-Ochoa S, Barastegui D, Pérez-Lafuente M, Romagosa C, Pérez M. Multidisciplinary management of a giant plexiform neurofibroma by double sequential preoperative embolization and surgical resection. Case Rep Neurol Med. 2013;2013:1-8.

Zhou J, Li M, Luo C, He Q, Yin Z, Peng H, et al. Giant neurofibroma in the right lower limb of a 26-year-old woman: report of a case. Int. Surg. 2012;97: 71-7.

Ross AL, Panthaki Z, Levi AD. Surgical management of a giant plexiform neurofibroma of the lower extremity. World Neurosurg. 2011;75(5): 754-7.

Vélez R, Barrera-Ochoa S, Barastegui D, Pérez-Lafuente M, Romagosa C, Pérez M. Multidisciplinary management of a giant plexiform neurofibroma by double sequential preoperative embolization and surgical resection. Case Reports in Neurological Med. 2013;2013:1-8.

Ros PR, Eshaghi N. Plexiform neurofibroma of the pelvis: CT and MRI findings. Magn Reson Imaging. 1991;9(3):463-5.

Morello F, Shah P, Dowling K, Siskin G. A hemorrhagic complication of neurofibromatosis. J Vascular Interventional Radiol. 2001;12(6):773-4.

White N, Gwanmesia I, Akhtar N, Withey SJ. Severe haemorrhage in neurofibromatoma: a lesson. British Journal of Plastic Surgery. 2004;57(5):456-7.

Weiss SW, Goldblum JR. Enzinger and weiss, soft tissue tumours. 4th ed. St. Louis: Mosby-Harcourt health sciences company; 2002.

Villani R, Michelina VV, Fabbri E, Tassone D, Morselli PG. Lower limb giant plexiform neurofibroma: preoperative evaluation and surgical planning. Int Surg J. 2016;3:1646-9.

Dave SP, Farooq U, Civantos FJ. Management of advanced laryngeal and hypopharyngeal plexiform neurofibroma in adults. Am J Otolaryngol. 2008;29:279-83.

Needle MN, Cnaan A, Dattilo J, Chatten J, Phillips PC, Shochat S, et al. Prognostic signs in the surgical management of plexiform neurofibroma: the Children's Hospital of Philadelphia experience, 1974-1994. J Pediatr. 1997;131(5):678-82.

Downloads

Published

2020-10-22