Plexiform Schwannoma: A Report of Two Unusual Cases, and a Review of the Literature


Plexiform schwannoma is a benign tumour arising from the sheath of myelinated nerve fibres that may occur in any part of the body. It is very rare in the head and neck region and poses diagnostic challenges. Here we present two unusual cases. The first is about a fifteen-year-old boy with a large plexiform schwannoma in the right retro maxillary region, extending up to but not invading the orbit. Initial cytology reported it as a pleomorphic adenoma, but histological findings after excision confirmed it to be a benign plexiform schwannoma. A zygomatic osteotomy was required to excise the tumour in its entirety. The second reported case is about solitary lesion on the tongue of a 46-year-old Chinese man, clinically mimicking a mucocoele. This case report highlights the diagnostic challenges posed by plexiform schwannoma, and the role of imaging and cytopathology, as well as the importance of definitive histopathological analysis. It also describes the surgical management of these lesions, and provides a review of the literature, in particular of cases occurring in the head and neck region.

Share and Cite:

Ali, S. , Vassiliou, L. and Stenhouse, P. (2014) Plexiform Schwannoma: A Report of Two Unusual Cases, and a Review of the Literature. Open Journal of Stomatology, 4, 174-178. doi: 10.4236/ojst.2014.44026.

Conflicts of Interest

The authors declare no conflicts of interest.


[1] Skouras, A., Skouras, G., Diab, A., Asimakopoulou, F.A., Dimitriadi, K. and Divritsioti, M. (2011) Schwannoma of the Nasal Tip: Diagnosis and Treatment. Aesthetic Plastic Surgery, 35, 657-661.
[2] Kohyama, S., Hara, Y., Nishiura, Y., Hara, T., Nakagawa, T. and Ochiai, N. (2011) A Giant Plexiform Schwannoma of the Brachial Plexus: Case Report. Journal of Brachial Plexus and Peripheral Nerve Injury, 6, 9.
[3] Reed, R.J. and Harkin, J.C. (1978)Pacinian Neurofibroma. Histopathology, 2, 153.
[4] Suchak, R., Luzar, B., Bacchi, C.E., Maguire, B. and Calonje, E. (2010) Cutaneous Neuroblastoma-Like Schwannoma: A Report of Two Cases, One with a Plexiform Pattern, and a Review of the Literature. Journal of Cutaneous Pathology, 37, 997-1001.
[5] Megahed, M. (1994) Plexiform schwannoma. The American Journal of Dermatopathology, 16, 288-293.
[6] Wright, B.A. and Jackson, D. (1980) Neural Tumours of the Oral Cavity: A Review of the Spectrum of Benign and Malignant Oral Tumours of the Oral Cavity and Jaws. Oral Surgery, Oral Medicine, Oral Pathology, 49, 509-522.
[7] Woodruff, J.M., Marshall, M.L., Godwin, T.A., Funkhouser, J.W., Thompson, N.J. and Erlandson, R.A. (1983) Plexiform (Multinodular) Schwannoma. A Tumor Simulating the Plexiform Neurofibroma. American Journal of Surgical Pathology, 7, 691-697.
[8] Enzinger, F.M. and Weiss, S.M. (1995) Soft Tissue Tumors. 3rd Edition, Missouri Mosby, St. Louis, 829-838.
[9] Pfeifle, R., Baur, D.A., Paulino, A. and Helman, J. (2001) Schwannoma of the Tongue: Report of 2 Cases. Journal of Oral and Maxillofacial Surgery, 59, 802-804.
[10] Gallo, W.J., Moss, M., Shapiro, D.N. and Gaul, J.V. (1977) Neurilemoma: Review of the Literature and Report of Five Cases. Journal of Oral Surgery, 35, 235-236.
[11] Ferreti Bonan, P.R., Martelli Jr., H., Nogueira Dos Santos, L.A., Comini Mol, V. and Paes De Almeida, O. (2008) Multinodular Neurilemmoma of the Tongue: A Case Report with Differential Immunohistochemical Profile. Minerva Stomatologica, 57, 71-75.
[12] Husain, S., Yunus, M.R., Ramli, R. and Athar, P.P. (2011) Schwannoma of the Tongue in a Ten-Year Old Child. Journal of Pakistan Medical Association, 61, 500-501.
[13] Gallesio, C. and Berrone, S. (1992) Schwannoma Located in the Tongue—A Clinical Case Report. Minerva Stomatologica, 41, 583-590.
[14] Hsu, Y.C., Hwang, C.F., Hsu, R.F., Kuo, F.Y. and Chien, C.Y. (2006) Schwannoma (Neurilemmoma) of the Tongue. Acta Oto-Laryngologica, 126, 861-865.
[15] Ioannou, M., Papanastassiou, I., Iakowidou, I., Kottakis, S. and Demertzis, N. (2009) Plexiform Schwannoma of the Posterior Tibial Nerve: A Case Report. Cases Journal, 2, 8392.
[16] Enoz, M., Suoglu, Y. and Ilhan, R. (2006) Lingual Schwannoma. Journal of Cancer Research and Therapeutics, 2, 76-78.
[17] Kar, M., Deo, S.V., Shukla, N.K., et al. (2006) Malignant Peripheral Nerve Sheath Tumours (MPNST) Clinicopathological Study and Treatment Outcome of Twenty-Four Cases. World Journal of Surgical Oncology, 4, 55.
[18] Agaram, N.P., Prakash, S. and Antonescu, C.R. (2005) Deep-Seated Plexiform Schwannoma: A Pathologic Study of 16 Cases and Comparative Analysis with the Superficial Variety. The American Journal of Surgical Pathology, 29, 1042-1048.
[19] Krolls, S.O., McGinnis Jr., J.P. and Quon, D. (1994) Multinodular versus Plexiform Neurilemmoma of the Hard Palate—Report of a Case. Oral Surgery, Oral Medicine, Oral Pathology, 77, 154-157.
[20] Fletcher, C.D. and Davies, S.E. (1986) Benign Plexiform (Multinodular) Schwannoma: A Rare Tumour Unassociated with Neurofibromatosis. Histopathology, 10, 971-780.
[21] Beggs, I. (1997) Pictorial Review: Imaging of Peripheral Nerve Tumours. Clinical Radiology, 52, 8-17.
[22] Cohen, M. and Wang, M.B. (2009) Schwannoma of the Tongue: Two Case Reports and Review of the Literature. European Archives of Oto-Rhino-Laryngology, 266, 1823-1829.

Copyright © 2022 by authors and Scientific Research Publishing Inc.

Creative Commons License

This work and the related PDF file are licensed under a Creative Commons Attribution 4.0 International License.