Journal of Clinical Oncology, Vol 19, Issue 1
(January), 2001: 274-276
© 2001 American Society for Clinical Oncology
Pediatric Tumors
Case 2. Anaplastic Meningioma in a 3 1/2-Year-Old Girl
Martin Benesch,
Reinhold Kleinert,
Elke Spork,
Hans Georg Eder,
Christian Urban
University of Graz, Graz, Austria
A 31/2-year-old girl developed a discrete unilateral drooping of the corner of the mouth and also arm weakness after tonsillectomy and adenotomy. Immediate radiographic investigations of the brain were not considered necessary because the remainder of her neurologic examination was unremarkable. Two weeks later the girl was hospitalized because of increasing general weakness, ataxia, and tremor of the right arm. Computed tomography brain scan ( Fig 1) revealed a huge heterogeneous tumor (7.8 x 7 x 7.6 cm) in the left frontotemporal region with calcifications, compression of the ipsilateral ventricle, and subsequent obstructive hydrocephalus. Contrast-enhanced, coronal, T1-weighted magnetic resonance imaging scans ( Fig 2) also showed a left frontotemporal tumor with irregular borders and inhomogeneous enhancement due to areas of hemorrhage and necrotic transformation. The tumor extended into the left basal ganglia and insula. A remarkable mass effect with midline shift and signs of raised intracranial pressure were also present.
During surgery, a pronounced thinness of the skull was visible, and adherence of the tumor to the dura was noted. The tumor itself was stiff and highly vascularized. Due to the extent and vascularization of the tumor, which resulted in massive bleeding during surgery, only a biopsy could be performed. The girl’s condition worsened dramatically during the following days with development of a right-sided hemiparesis and coma. Treatment with interferon and thalidomide as angiogenesis inhibitors was attempted, but the patient’s condition did not improve and she died 3 weeks after surgery.
Histopathologic examinations of biopsy and postmortem tissue specimens revealed anaplastic chordoid meningioma with transformation into fibrosarcoma. The autopsy section shows a well circumscribed brain tumor of the left hemisphere with multifocal hemorrhage and tumor necrosis; the site of the surgical approach in the left insular region is indicated by arrows ( Fig 3). Microscopically, a highly vascularized pleomorphic tumor with mitotic activity was present with spreading of storiform-arranged tumor cell clusters along the blood vessels and leptomeningeal and cortical infiltration ( Fig 4A). Chordoid-like structures, ie, cohesive strands of epithelioid tumor cells in a mucinous tumor matrix, were noted under Masson trichrome staining (Fig 4B).
Although tumors of the CNS are the second most common neoplasms in children, intracranial meningiomas account for only 0.5% to 2.4% of all brain tumors in this age group.1-4 They form a heterogeneous group of mostly benign tumors that arise from meningothelial cells and include meningothelial, atypical, papillary and anaplastic (malignant) meningioma.5 Whereas atypical meningiomas correspond histologically to grade II malignancy, papillary and anaplastic meningiomas are aggressive and frequently recur. They are therefore classified as tumors of grade III malignancy.5 Most cases of childhood meningiomas are diagnosed at the end of the first or at the beginning of the second decade of life.3,4 In contrast to adults, children with meningiomas are considered to have a poorer prognosis because of high rates of local relapse and malignant transformation.4 Surgical resection is the standard treatment for intracranial meningiomas, with total removal of the tumor usually resulting in complete long-term remission.3,6 Malignant meningiomas, in contrast, have an unfavorable prognosis, despite surgical resection and radiotherapy.6 However, the extent of surgical resection and the administration of adjuvant radiotherapy are important prognostic parameters.6 Patients with malignant meningiomas who undergo subtotal tumor resection have a 5-year disease-free survival rate of only 28% without radiotherapy, compared with 57% with radiotherapy.6 The present case demonstrates the dramatic clinical course of an intracranial childhood anaplastic meningioma. In this patient, the prerequisite for a successful treatment, ie, tumor resection, could not be achieved due to the extent of the tumor and the imminent bleeding risk during surgery.
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