Skip to main content
Top
Published in:

03-03-2025 | Neurofibromatosis | Clinical Report

Massive Plexiform Neurofibroma Involving Multiple Salivary Glands: A Rare Case Report

Authors: Rita Rathore, Sana Ahuja, Akansha Gautam, Shikhar Chohan, Sufian Zaheer

Published in: Indian Journal of Otolaryngology and Head & Neck Surgery | Issue 4/2025

Login to get access

Abstract

Plexiform neurofibroma in the salivary glands is a rare condition, comprising only 0.4% of all salivary gland tumors. This benign but locally invasive tumor, commonly associated with neurofibromatosis type 1 (NF-1), arises from Schwann cells of the peripheral nerve sheath. In this report, we present a case of a 20-year-old male with a massive plexiform neurofibroma involving both the left parotid and submandibular glands. The patient had a painless swelling in the left preauricular region since 2 years, which had gradually enlarged over the last one year. Imaging revealed extensive involvement of the tumor, extending into the parapharyngeal space and infratemporal fossa. A surgical excision was performed, and a histopathological examination confirmed the diagnosis. The extensive size and involvement of multiple salivary glands posed significant challenges in management, emphasizing the need for careful surgical planning and thorough clinical evaluation. This case highlights the rare occurrence of large plexiform neurofibromas in the salivary glands and underscores the complexities involved in treating such extensive tumors.
Literature
1.
go back to reference Bourgeois JM, Radhi J, DenEi L (2001) Plexiform neurofibromas of the submandibular gland in a child. Can J Gastroenterol 15:835–837CrossRefPubMed Bourgeois JM, Radhi J, DenEi L (2001) Plexiform neurofibromas of the submandibular gland in a child. Can J Gastroenterol 15:835–837CrossRefPubMed
2.
go back to reference Burger PC, Scheithauer BW, Vogel FS (2002) Surgical pathology of the nervous system and its covering. 4, vol 1. Churchill Livingstone, Philadelphia, pp 594–611 Burger PC, Scheithauer BW, Vogel FS (2002) Surgical pathology of the nervous system and its covering. 4, vol 1. Churchill Livingstone, Philadelphia, pp 594–611
3.
go back to reference Tamer E, Yalein B, Alli N (2003) Multiple nodular plexiform neurofibromas in a neurofibromatosis 1 Familial tendency? A case report and review of literature. Int J Dermatol 42:357–358CrossRefPubMed Tamer E, Yalein B, Alli N (2003) Multiple nodular plexiform neurofibromas in a neurofibromatosis 1 Familial tendency? A case report and review of literature. Int J Dermatol 42:357–358CrossRefPubMed
4.
go back to reference Shekar TY, Gautam G, Prrabhala S, Gole S (2010) Plexiform neurofibroma: A rare tumor of submandibular salivary gland. J Surg Tech Cse Rep 2:81–83CrossRef Shekar TY, Gautam G, Prrabhala S, Gole S (2010) Plexiform neurofibroma: A rare tumor of submandibular salivary gland. J Surg Tech Cse Rep 2:81–83CrossRef
5.
go back to reference Lin J, Martel W (2001) Cross sectional image of peripheral nerve sheath tumors: characteristic sign on CT, MR imaging and sonography. AJR Am J Roentgenol 176:75–82CrossRefPubMed Lin J, Martel W (2001) Cross sectional image of peripheral nerve sheath tumors: characteristic sign on CT, MR imaging and sonography. AJR Am J Roentgenol 176:75–82CrossRefPubMed
6.
go back to reference Rapado F, Simo R, Small M (2001) Neurofibromatosis type 1 of the head and neck: dilemmas in management. J Laryngol Otol 115:151–154CrossRefPubMed Rapado F, Simo R, Small M (2001) Neurofibromatosis type 1 of the head and neck: dilemmas in management. J Laryngol Otol 115:151–154CrossRefPubMed
7.
go back to reference Cebesoy O, Tutar E, Isik M, Arpacioglu O (2007) A case of isolated giant plexiform neurofibroma involving all branches of the common peroneal nerve. Arch Orthop Trauma Surg 127:709–712CrossRefPubMed Cebesoy O, Tutar E, Isik M, Arpacioglu O (2007) A case of isolated giant plexiform neurofibroma involving all branches of the common peroneal nerve. Arch Orthop Trauma Surg 127:709–712CrossRefPubMed
8.
go back to reference Qadri S, Rabindranath D, Quadri S et al (2020) Facial plexiform neurofibroma as an intraparotid nodule: A rare case presentation. Int J Otorhinolaryngol Clin 12:8–10 Qadri S, Rabindranath D, Quadri S et al (2020) Facial plexiform neurofibroma as an intraparotid nodule: A rare case presentation. Int J Otorhinolaryngol Clin 12:8–10
9.
go back to reference Blioskas S, Sotiriou S, Rizou K et al (2017) An exceptional case of intraparotid plexiform neurofibroma originating from autonomic fibers of the auriculotemporal nerve. Case Rep Med 2017:8327215CrossRefPubMedPubMedCentral Blioskas S, Sotiriou S, Rizou K et al (2017) An exceptional case of intraparotid plexiform neurofibroma originating from autonomic fibers of the auriculotemporal nerve. Case Rep Med 2017:8327215CrossRefPubMedPubMedCentral
10.
go back to reference Weitzner S (1980) Plexiform neurofibroma of major salivary glands in children. Oral Surg Oral Med Oral Pathol 50:53–57CrossRefPubMed Weitzner S (1980) Plexiform neurofibroma of major salivary glands in children. Oral Surg Oral Med Oral Pathol 50:53–57CrossRefPubMed
11.
go back to reference Souaid JP, Nguyen VH, Zeitouni AG, Manoukian J (2003) Intraparotid facial nerve solitary plexiform neurofibroma: a first paediatric case report. Int J Pediatr Otorhinolaryngol 67:1113–1115CrossRefPubMed Souaid JP, Nguyen VH, Zeitouni AG, Manoukian J (2003) Intraparotid facial nerve solitary plexiform neurofibroma: a first paediatric case report. Int J Pediatr Otorhinolaryngol 67:1113–1115CrossRefPubMed
12.
go back to reference Lekhbal A, Lyoubi H, Wydadi O et al (2020) Intraparotid facial nerve plexiform neurofibroma in a child (case report). Ann Med Surg 55:9–12CrossRef Lekhbal A, Lyoubi H, Wydadi O et al (2020) Intraparotid facial nerve plexiform neurofibroma in a child (case report). Ann Med Surg 55:9–12CrossRef
13.
go back to reference Khan AA, Ahuja S, Zaheer S (2024) Intraparotid plexiform neurofibroma: A rare diagnostic challenge. Diagn Cytopathol.;1–4 Khan AA, Ahuja S, Zaheer S (2024) Intraparotid plexiform neurofibroma: A rare diagnostic challenge. Diagn Cytopathol.;1–4
Metadata
Title
Massive Plexiform Neurofibroma Involving Multiple Salivary Glands: A Rare Case Report
Authors
Rita Rathore
Sana Ahuja
Akansha Gautam
Shikhar Chohan
Sufian Zaheer
Publication date
03-03-2025
Publisher
Springer India
Published in
Indian Journal of Otolaryngology and Head & Neck Surgery / Issue 4/2025
Print ISSN: 2231-3796
Electronic ISSN: 0973-7707
DOI
https://doi.org/10.1007/s12070-025-05405-8