NEUROLOGY 2008;70:e95
© 2008 American Academy of Neurology
Resident and Fellow Section
Teaching NeuroImage: Hemorrhagic ependymoma in the elderly
A rare cause of headache and gait imbalance
N. Montano, MD,
P. De Bonis, MD,
F. Doglietto, MD,
A. Cianfoni, MD,
R. Pallini, MD,
L. Lauriola, MD and
G. Maira, MD
From the Institute of Neurosurgery (N.M., P.D.B., F.D., R.P., G.M.), Institute of Radiology (A.C.), and Institute of Pathology (L.L.), Catholic University School of Medicine, Rome, Italy.
Address correspondence and reprint requests to Dr. Pasquale De Bonis, Institute of Neurosurgery, Catholic University School of Medicine, Largo Agostino Gemelli, 8, 00168 Rome, Italy debonisvox{at}gmail.com
A 73-year-old man presented with headaches, vomiting, and gait imbalance. Neurologic examination demonstrated bilateral papilledema and positive Romberg sign. Neuroimaging showed a posterior fossa lesion with calcifications and intratumoral hemorrhage associated with obstructive hydrocephalus (figure 1, A through D). Gross total resection was performed (figure 2, A and B). Histologic examination documented ependymoma (figure 2, C through E).
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Figure 1 Brain neuroimaging
Nonenhanced CT (A), MRI with axial T2-WI (B), sagittal non-enhanced (C) and enhanced (D) T1-WI, showing a heterogeneous, solid-cystic, hemorrhagic and calcific mass in the cisterna magna and in the inferior portion of the fourth ventricle. A blood-fluid level is visible in the cystic portion (arrow) and contrast enhancement is evident in the solid intraventricular portion. The mass obstructed the fourth ventricle with resulting hydrocephalus.
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Figure 2 Surgical and histologic images
(A, B) Intraoperative view confirming the hemorrhagic component of the lesion (A) and documenting the floor of the fourth ventricle free of disease at the end of tumor removal (B). (C–E) Histologic examination showed monomorphic cells with regular round nucleus and dispersed chromatin; tumor cells of the perivascular area were arranged radially around the vessels, forming perivascular pseudorosettes, with prominent perivascular immunoreaction for glial fibrillary acid protein (D) and epithelial membrane antigen (E). Mitoses and necrosis were absent. Diagnosis: WHO grade II ependymoma.
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Ependymoma typically affects children and rarely occurs in elderly patients.1 It is most frequently located in the posterior fossa and presents with cerebellar compression and intracranial hypertension. On MRI it appears as a T1-hypointense, T2-hyperintense, homogeneous contrast-enhancing mass that may fill and expand the fourth ventricle; heterogeneous enhancement due to calcifications and cystic components may be seen.2 Intratumoral hemorrhage has been reported and rarely causes an acute presentation.3 Hemorrhagic ependymoma should be considered in the differential diagnosis of hemorrhagic posterior fossa lesions in adults.
Disclosure: The authors report no disclosures.
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REFERENCES
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- Lyons MK, Kelly PJ. Posterior fossa ependymomas: report of 30 cases and review of the literature. Neurosurgery 1991;28:659–664.[Medline]
- Maksoud YA, Hahn YS, Engelhard HH. Intracranial ependymoma. Neurosurg Focus 2002;13:e4.[Medline]
- Honda M, So G, Kaminogo M, Abe K, Nagata I. Massive intratumoral hemorrhage of ependymoma of the fourth ventricle. Childs Nerv Syst 2005;21:926–929.[Medline]
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