Abstract
This study investigates the clinical, radiological, and pathological features of two cases of intraosseous schwannoma that arose in patients with multiple soft tissue schwannomas. In both cases, the patients were adult females and the tibial bone was affected. Vestibular schwannomas were not identified, indicating that these were not cases of neurofibromatosis 2 (NF2). Radiographs showed a well-defined lytic lesion in the proximal tibia; in one case, this was associated with a pathological fracture. Histologically, both cases showed typical features of benign schwannoma. Molecular analysis of one of the excised tumors showed different alterations in the NF2 gene in keeping with a diagnosis of schwannomatosis. Our findings show for the first time that intraosseous schwannomas can occur in schwannomatosis.
Similar content being viewed by others
References
Ida CM, Scheithauer BW, Yapicier O, Carney JA, Wenger DE, Inwards CY, et al. Primary schwannoma of the bone: a clinicopathologic and radiologic study of 17 cases. Am J Surg Pathol. 2011;35:989–97.
Dorfman HD, Czerniak B. Intraosseous schwannoma. In: Bone tumours. St. Louis: Mosby; 1998. p. 825–8.
Unni KK. Neuriliemmoma and related tumours In: Dahlin’s Bone Tumours. General aspects and date on 11087 cases. Philadelphia: Lippincott Raves; 1996. p. 343–7.
Schajowicz F. Neuriliemmoma (schwannoma, neuroma). In: Tumours and tumourlike lesions of bone and joints. Berlin Heidelberg New York: Springer Verlag; 1981. p. 390–2.
Sherman MS. The nerves of bone. J Bone Joint Surg [Am]. 1963;45:522–8.
Herskovits MS, Singh IJ, Sandhu HS. Innervation of bone. In: Hal BK, editor. Bone. Vol 3. Boca Raton, FL: CRC Press; 1991. p. 165–86.
Jones KB, Mollano AV, Morcuende JA, Cooper RR, Saltzman CL. Bone and brain: a review of neural, hormonal, and musculoskeletal connections. Iowa Orthop J. 2004;24:123–32.
de Satnos DA, Burgess E. Primary and secondary neurilemmoma of bone. Surg Gynecol Obstet. 1940;71:454–61.
Baser M, Friedman JM, Wallace AJ, Ramsden RT, Joe H, Evans DG. Evaluation of clinical diagnostic criteria for neurofibromatosis 2. Neurology. 2002;59:1759–65.
Baser ME, Friedman JM, Evans DG. Increasing the specificity of diagnostic criteria for schwannomatosis. Neurology. 2006;15:730–2.
MacCollin M, Woodfin W, Kronn D, Short MP. Schwannomatosis: a clinical and pathologic study. Neurology. 1996;46:1072–9.
Plotkin SR, Blakeley JO, Evans DG, Hanemann CO, Hulsebos TJ, Hunter-Schaedle K, et al. Update from the 2011 International schwannomatosis workshop: from genetics to diagnostic criteria. Am J Med Genet A. 2013;1:405–16.
Koontz NA, Wiens AL, Agarwal A, Hingtgen CM, Emerson RE, Mosier KM. Schwannomatosis: the overlooked neurofibromatosis? AJR Am J Roentgenol. 2013;200:W646–53.
Jacobs RL, Fox TA. Neurilemmoma of bone. A case report with a review of the literature. Clin Orthop. 1972;87:248–53.
Fawcett KJ, Dahlin DC. Neurilemmoma of bone. Am J Clin Pathol. 1967;47:759–66.
Gordon EJ. Solitary intraosseous neurilemmoma of the tibia: review of intraosseous neurilemmoma and neurofibroma. Clin Orthop Relate Res. 1976;117:271–82.
de la Monte SM, Dorman HD, Chandra R, Malawer M. Intraosseous schwannoma: histologic features, ultrastructure, and review of the literature. Hum Pathol. 1984;15:551–8.
Ang WM, Yates P, Robbins P, Wood D. Recurrent benign solitary intraosseous schwannoma of the tibia. Orthopedics. 2008;31:176.
Ahlawat S, Blakeley J, Montgomery E, Subramaniam RM, Belzberg A, Fayad LM. Schwannoma in neurofibromatosis type 1: a pitfall for detecting malignancy by metabolic imaging. Skeletal Radiol. 2013;42:1317–22.
Scheithauer BW, Woodruff JM, Erlandon RA. Tumours of the Peripheral Nervous System. Atlas of Tumour Pathology Third Series, Fascicle 24. Washington, DC: AFIP; 1997. p. 105–76.
Acknowledgments
We would like to thank Chris Lowe for typing the manuscript. The authors acknowledge the support of the Oxford NIHR BRU.
Conflict of interest statement
The authors declare there is no conflict of interest.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Kashima, T.G., Gibbons, M.R.J.P., Whitwell, D. et al. Intraosseous schwannoma in schwannomatosis. Skeletal Radiol 42, 1665–1671 (2013). https://doi.org/10.1007/s00256-013-1712-6
Received:
Revised:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00256-013-1712-6