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

Abstract

Abstract. Ollier's disease is characterized by the hamartomatous proliferation of cartilage cells, producing masses termed chondromas. A patient presented with Ollier's disease which was found to be associated with diffuse gliomas. Investigating this disease is crucial as there is a high risk of sarcomatous transformation of the skeletal lesions as well as an increased risk of developing extra-osseous malignancies.

Ollier disease with anaplastic astrocytoma: A review of the literature and a unique case

“…Her age at presentation was almost twice the median age at diagnosis for gliomas in Ollier disease, as cited in the literature. [ 9 ] In addition, the disease seems to be well-controlled after her subtotal resection even without postoperative adjuvant therapy, as shown in the second MRI image from 2014 [ Figure 3 ]. The patient continues to survive without postoperative treatment 3 years from diagnosis, and after subtotal resection, compared to an observed 27% 5-year survival in untreated non-Ollier astrocytomas, where despite implementation of intensive therapeutic strategies and supportive care, the median survival of glioblastomas has remained at 12 months over the past decade.…”

“…[ 3 ] The mean survival of the 19 patients with Ollier disease and CNS malignancy reported in the literature is not clear, but undoubtedly the prognosis for such patients is poor. [ 9 ] In patients with non-Ollier disease astrocytomas, the median progression free survival (PFS) is 4.6 months and the overall survival (OS) is 20.5 months. [ 6 ]…”

“…[ 3 ] This is different from non-Ollier astrocytomas, which most commonly involve the temporal lobe. [ 9 ] Our patient is a 55-year-old woman with a large left-sided frontotemporal lesion. Her age at presentation was almost twice the median age at diagnosis for gliomas in Ollier disease, as cited in the literature.…”

“…Because the signs and symptoms of a brain tumor may initially be vague, a neurological examination and MRI, if necessary, should be conducted during the follow-up period. [ 9 ] In addition, immunohistochemistry for IDH1 should be performed whenever a specimen is available.…”

“…Intracranial lesions have been described previously and the most prevalent type of CNS tumors associated with Ollier disease is astrocytoma (low-grade to glioblastoma multiforme) and oligodendroglioma. [ 9 ] In this paper, the authors describe a case of an anaplastic astrocytoma in Ollier disease with a survival time of over 3 years, which, to our knowledge, is the first reported case of anaplastic astrocytoma with such a long survival time.…”