Meningiomas are generally slowly growing benign tumors attached to the dura mater and composed of neoplastic meningothelial (arachnoidal) cells. They typically manifest in adults and are predominant in women. Meningiomas account for approximately 20% of all intracranial tumors in males and 38% in females. The prevalence of Meningiomas is estimated to be 97.,5 in 100,.000 with over 138,.000 individuals diagnosed with this tumor. Chordoid meningioma (CM) is a meningioma containing regions that are histologically similar to chordoma, with trabeculae of eosinophilic, vacuolated cells in a myxoid background and it corresponds to WHO grade II. CM is a rare variant first described in young patients associated with Castleman syndrome by Kepes et al. In 1988.
To the best of our knowledge, about 70 cases have been previously published, but unpublished ones are often recorded. Meningioma is most common in middle-age and elderly patients (peak 6th decade). CM confirms this trend but a peak is also present in 2nd decade. It arises within supratentorial cranial space (cerebral convexities) in most cases, while a minority arises in posterior fossa and tentorium, ; it is rarely located in the spine. Castelman’s syndrome (CS), more or less complete, is associated with this kind of meningioma. Progesterone receptors are scarcely represented in CM and are almost absent in chordoid areas. Such datum could be associated with a higher growing rate of meningiomas.
The histological findings and epidemiological data from the literature suggest that in children this kind of lesion has more features similar to meningioma than in adult form; in relapse there is less presence of meningothelial areas and progesterone receptors than in the first tumor. CM is probably only a real subtype of meningioma in children.
To the best of our knowledge, about 70 cases have been previously published, but unpublished ones are often recorded. Meningioma is most common in middle-age and elderly patients (peak 6th decade). CM confirms this trend but a peak is also present in 2nd decade. It arises within supratentorial cranial space (cerebral convexities) in most cases, while a minority arises in posterior fossa and tentorium, ; it is rarely located in the spine. Castelman’s syndrome (CS), more or less complete, is associated with this kind of meningioma. Progesterone receptors are scarcely represented in CM and are almost absent in chordoid areas. Such datum could be associated with a higher growing rate of meningiomas.
The histological findings and epidemiological data from the literature suggest that in children this kind of lesion has more features similar to meningioma than in adult form; in relapse there is less presence of meningothelial areas and progesterone receptors than in the first tumor. CM is probably only a real subtype of meningioma in children.
References
- 1.Claus EB, Bondy ML, Schildkraut JM et al..Epidemiology of intracranial meningioma. Neurosurgery 57:1088-1095, 2005.
- 2.Bondy M, Ligon BL. Epidemiology and etiology of intracranial meningiomas: A Review. J Neuro-oncol 23:197-205, 1996.
- 3.Surawicz TS, McCarthy BJ, Kupelian V, Jukich PJ, Bruner JM, Davis FG. Descriptive epidemiology of primary brain and CNS tumors: Results from the Central Brain Tumor Registry of the United States, 1990-1994. Neuro-oncol 1:14-25, 1999.
- 4.Donato G, Ferraro G, Signorelli F, Iofrida G, Lavano A et al. Chordoid Meningioma: Case Report and Literature Review. Ultrastruct Pathol 30(4):309-14, 2006
