Simultaneous occurrence of a supra- and an infratentorial glioma in a patient with Ollier's disease: more evidence for non-mesodermal tumor predisposition in multiple enchondromatosis

Abstract

A case is presented in which two neuro-ectodermal tumors, an infra- and a supratentorial glioma, developed in a young man with multiple enchondromatosis of Ollier's disease. This is the third such case of multifocal low-grade glioma in Ollier's disease, suggesting a predisposition for non-mesodermal tumors in Ollier's disease. The related condition of multiple enchondromatosis and hemangiomas (Maffucci's syndrome) is well known for its malignant potential, developing both mesodermal and non-mesodermal tumors. Along with other authors, we support the concept of two variants of the same disease with a predisposition to development of tumors from various germ layers.

Diffuse gliomas in an adolescent with multiple enchondromatosis (Ollier’s disease)

“…To our knowledge, 14 cases of Ollier's disease associated with gliomas have been reported, including the present case (5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15). The patients were predominantly male and the median age at diagnosis of the glioma was 26.4 years .…”

Multifocal intracranial astrocytoma in a pediatric patient with Ollier disease

“…[12] These cartilaginous masses cause thinning of the overlying cortices, with associated shortening and deformity of the bone. Limb length discrepancies and pathological fractures can also occur.…”

“…[1–356] In 1899, Ollier described enchondromatosis in a patient with no evidence of vascular anomalies, known as Ollier disease (OD). [12]…”

Multifocal supratentorial diffuse glioma in a young patient with Ollier disease

“…Previous reports of Ollier disease patients with concomitant glioma have been documented in patients as young as 14 years old 4 , and with up to two synchronous gliomas. 9,[11][12][13][14] This report indicates the importance for those clinicians who treat patients with Ollier disease to be aware of the increased risk of primary brain tumours and to have a low threshold for investigating symptoms that may herald the development of a primary brain tumour.…”