Abstract
Introduction Ollier Disease is a sporadic skeletal disorder with a predisposition to oncogenesis. It is estimated at around 1/100,000. We are presenting a young patient with Ollier Disease and high-grade astrocytoma. Case report A 14-year-old, Caucasian male with Ollier Disease presented with a history of headaches, vomiting, blurred vision, and unsteady gait. Brain MRI with contrast showed a 41 × 55 mm mass in the posterior fossa with spotty enhancement, which pathology proved to be anaplastic astrocytoma. Conclusion Despite the universal acceptance that Ollier Disease carries a high risk of developing malignancy there is very little in the literature about systematic screening. We recommended a cost-effective screening regime for these patients.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Anaplastic Astrocytoma (2006) Children’s Hospital Boston Website. http://www.childrenshospital.org/az/Site565/mainpageS565P0.html
Bruce JN, Waziri A, Senatus PB. Astrocytoma. EMedicine.http://www.emedicine.com/med/topic2693.html August 2006 [Monograph on the Internet] Accessed 1 Feb 2008
David JB, Carl DE, Eric JH, Walter EB (2001) Estimated Risks of Radiation-Induced Fatal Cancer from Pediatric CT. Am J Roentgenol 176:289–296
Frappaz D, Ricci AC, Kohler R, Bret P, Mottolese C (1999) Diffuse brain stem tumor in an adolescent with multiple enchondromatosis (Ollier’s disease). Childs Nerv Syst 15(5):222–225
Frassica FJ, Sim FH. Ollier disease and Maffucci syndrome: Tutorial. http://dev.slackinc.com/aaosdemo/Tutorials/tumors/ollier/tutorial.cfm#Link2 Orthopedics Hyperguide. SLACK Incorporated. Accessed 1 Feb 2008
Kleihues P, Cavenee WK (2000) World Health Organization Classification of Tumours: Pathology and Genetics: Tumours of the Nervous System, 2nd edn. Oxford University Press, USA
Mahafza WS (2004) Multiple enchondromatosis Ollier disease with two primary brain tumors. Saudi Med J 25(9):1261–1263
McDermott AL, Dutt SN, Chavda SV et al (2001) Maffucci’s syndrome: clinical and radiological features of a rare condition. J Laryngol Otol 115(10):845–847
Ollier Disease (2006) National Organization for Rare Disorders (NORD). http://www.rarediseases.org
Sandberg AA, Bridge JA (2003) Updates on the cytogenetics and molecular genetics of bone and soft tissue tumors chondrosarcoma and other cartilaginous neoplasms. Cancer Genet and Cytogenet 143(1):1–31
Schwartz HS, Zimmerman NB, Simon MA, Wroble RR, Millar EA, Bonfiglio M. (1987) The malignant potential of enchondromatosis. J Bone Joint Surg Am 69(2):269–274
Shankar PP, Wei H, Davee SM, Funk JL (2000) Parathyroid hormone-related protein is expressed by transformed and fetal human astrocytes and inhibits cell proliferation. Brain Res 868(2):230–240
Shannon P, Sabha N, Lau N, Kamnasaran D, Gutmann DH, Guha A (2005) Pathological and molecular progression of astrocytomas in a GFAP:12 V-Ha-Ras mouse astrocytoma model. Am J Pathol 167(3):859–867
Silve C, Jüppner H (2006) Ollier disease. Orphanet J Rare Dis 1:37
Thiffault I, Schwartz CE, Der Kaloustian V, Foulkes WD (2004) Mutation analysis of the tumor suppressor PTEN and the glypican 3 (GPC3) gene in patients diagnosed with Proteus syndrome. Am J Med Genet A 130(2):123–127
van Nielen KMB, de Jong BM (1999) A case of Ollier’s disease associated with two intracerebral low-grade gliomas. Clin Neurol Neurosurg 101(2):106–110
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
Walid, M.S., Troup, E.C. Cerebellar anaplastic astrocytoma in a teenager with Ollier Disease. J Neurooncol 89, 59–62 (2008). https://doi.org/10.1007/s11060-008-9583-8
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s11060-008-9583-8