Childhood meningiomas associated with meningioangiomatosis: report of five cases and literature review

Meningioangiomatosis is a unique, rare hamartomatous lesion. Meningiomas arising in the background of meningioangiomatosis are rare conditions which pathologically and radiologically mimic invasive meningiomas, but have a benign clinical course in children and young adults. In this study, five such cases are reported. To our knowledge, this is the largest reported collection of meningiomas associated with meningioangiomatosis. Less immunoreactivity for progesterone receptor and high Ki-67 labelling index are generally known to be associated with invasive meningiomas. However, high expression of progesterone receptor and low Ki-67 labelling index in the present cases supports the idea that brain invasion is not an indicator of malignancy but an independent finding associated with meningiomas which have arisen from meningioangiomatosis. We emphasize the good prognosis of such tumours and discuss pathogenesis of meningiomas with meningioangiomatosis.     

Childhood and juvenile onset multiple sclerosis: clinical and paraclinical features

It is well known that multiple sclerosis (MS) is a demyelinating disease of the central nervous system that mostly starts in the second to third decade. In the present study, we reviewed our own observations of the clinical and paraclinical features in the 36 of 890 (4.04%) MS patients whose symptoms started before 16 years of age. The average age at onset of the disease in these 36 patients was 12.9 years. In 18 patients, the disease onset was monosymptomatic. Diplopia and sensory disturbances were the most common initial manifestations and occurred in 27.7% of cases. Twenty-one patients (59%) had a relapsing and 11 patients (30.5%) had a secondary progressive course. On the last evaluation, the EDSS score was above 5 in 11 patients and it was below 5 in 21 patients. As a result of this study, we concluded that childhood onset MS does not significantly differ from that it has been typically seen in adults in terms of major clinical manifestations and course of disease.     

Clinical and histological features of multiple meningiomas compared with solitary meningiomas

Between 1991 and 2002, 456 patients with an intracranial meningioma were treated. Thirty-nine of these had more than one meningioma (8.6%). The mean age was 58 years (27-85 years). Sex distribution was 8.8:1 (35 female, four male). There was no associated spinal meningioma. No patient had neurofibromatosis. In 19 patients all meningiomas were removed. Twelve showed the same histology, seven had different histological features. In the remaining 20 patients only the symptomatic meningioma was removed. Recurrences occurred in 11 patients (28.2%). Six patients died during follow-up. Multiple meningiomas have their own clinical features. Besides a high female preponderance, PR expression was stronger in multiple meningiomas than in solitary meningiomas while p53 status and MIB-1 LI were similar between the two groups. Progesterone receptor, p53 status and MIB-1 LI were valuable markers for predicting a patient's outcome in multiple meningiomas. The number of meningiomas is growing in patients with recurrent meningiomas.     

Microcystic meningiomas

Four patients (two male and two female) underwent surgery for the removal of tumors with clinical and radiographic features typical of a meningioma. Despite an incorrect intraoperative frozen section diagnosis in two cases, the tumors were totally resected because the surgeon was convinced of the meningiomatous nature of the lesion. Subsequent histology revealed features of a microcystic meningioma and careful scrutiny of paraffin sections showed that each of the tumors contained occasional typical meningothelial cellular whorls which were a helpful diagnostic clue. Ultrastructural examination and the absence of GFAP confirmed the diagnosis. Close liaison with the neurosurgeon, on the part of the pathologist and an awareness of this unusual variant of meningioma should prevent misdiagnosis and inappropriate treatment of a potentially curable tumor.     

Optic nerve gliomas and meningiomas

Optic nerve gliomas are benign astrocytic neoplasms that primarily affect children. The ultimate prognosis for vision is poor. For lesions confined to the optic nerves, long-term survival is excellent, but with involvement of the chiasm and especially of the hypothalamus or third ventricle, prognosis falls significantly. For this group, no form of therapy has proven to be of any benefit. Optic sheath meningiomas are neoplasms arising from arachnoid cap cells. They primarily affect middle-aged adults. Like gliomas, the prognosis for vision is poor, but unlike gliomas, prognosis for life is excellent.     

Polycystic and ring-shaped intracranial meningiomas

Intracranial meningiomas may be associated with peritumoral, unilocular cysts. In two middle-aged women we have recently treated, CT scan revealed ring-shaped and polycistic tumors which proved to be meningiomas. This unusual aspect has previously received scant attention. In the first patient the cysts were intratumoral and the walls consisted entirely of meningiomatous tissue; in the second case the cysts were peritumoral and their connective walls contained scattered nests of tumor cells.

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Sommario I meningiomi intracranici possono essere associati con cisti peritumorali, uniloculari. In due donne di media età che noi abbiamo recentemente operato una TAC ha mostrato tumori policistici e ad anello, che si sono rivelati dei meningiomi. Questo aspetto inusuale è stato raramente descritto. Nella prima paziente le cisti erano endotumorali e le pareti erano costituite interamente di tessuto meningiomatoso; nel secondo caso, le cisti erano peritumorali e le loro pareti connettivali contenevano nidi di cellule tumorali.