Infratentorial Meningioma in an 8-Year-Old Child as First Sign of Neurofibromatosis Type 2

Abstract

Meningiomas are rare intracranial tumors in pediatric patients. In contrast to meningiomas in adults, childhood ones have a poorer prognosis because of their high growth potential and tendency to recur. Meningiomas are often associated with neurofibromatosis type 2 (NF2) which is an autosomal-dominant disorder. In contrast to adults who primarily present with symptoms due to vestibular tumors, the initial symptoms in children with NF2 are subtle skin tumors, posterior capsular cataracts, or neurological signs secondary to cranial nerve(s) schwannoma excluding vestibular nerve, and/or brainstem or spinal cord compression. Here we report on the clinical, radiological, and histological findings in an 8-year-old boy who was diagnosed with an isolated infratentorial meningioma and a novel splice site mutation in the NF2 gene. The same mutation was detected in the boy's mother who suffered from hearing loss and tinnitus due to a bilateral vestibular schwannoma. Our patient demonstrates the need for molecular testing for NF2 gene mutations even in isolated childhood meningiomas although they do not fulfill the clinical criteria of NF2.

Intraventricular metaplastic meningioma in a child: case report and review of the literature

“…7 NF2-associated meningiomas, on the other hand, do have a higher predisposition to rapid and malignant progression than sporadic adult counterparts. 1 One series of intraventricular meningiomas found that the primary risk for recurrence/progression is subtotal surgical resection, and this has been corroborated by others. 2,7 In summary, this is the first published case of an intraventricular myxoid meningioma arising in a child.…”

“…1 Furthermore, intraventricular meningiomas are rare, accounting for only 0.5-2.0% of all meningiomas. 2 Several histologic variants of grade I meningioma have been documented in the literature, including fibrous, meningothelial, transitional, microcystic, angiomatous, secretory, lymphoplasmacyte-rich and psammomatous.…”

Clear Cell Meningeoma of the Brainstem in a 3.5-year-old Child: Case Report and Review of the Literature

“…Our patient showed spastic paraparesis with ataxic gait disturbance and truncal ataxia as well as hyperrefl exia, particularly of the lower limbs, with positive Babinski ' s refl ex as signs of cerebellum and brainstem compression. Meningiomas in childhood and adolescence are frequently associated with NF [ 5,10,12,13,15,25,26,29,30,35,37,38] and radiation therapy. An association with NF complicates the neurological signs, because patients with this disease often have diff use meningiomatosis or other tumors [5,26,29,35,38] .…”

“…The signs and symptoms of raised intracranial pressure are therefore often the initial presenting signs in infants and younger children with meningiomas [12 -14, 20, 37, 38] . In infants, increased head circumference, tense fontanel and seizures are frequently sporadic cases [35] . Most children with NF-2 show only a single lesion initially.…”

“…Since the association of meningioma and NF-2 is known, patients and their family members should be investigated for NF-2 gene mutations because of the prognostic impact of the syndrome. Lifelong multiprofessional care in a qualifi ed medical centre according to screening protocols is recommended for all patients with NF-2 [5,26,35] . The boy ' s aunt had already been operated on for two intraspinal meningiomas.…”