Bevacizumab is Effective for Recurrent Papillary Tumor of the Pineal Region: First Report

Abstract

Papillary tumor of the pineal region (PTPR) is a rare brain tumor that probably arises from ependymal cells. There are no known systemic treatments for PTPR once it is refractory to surgery and radiation. We present the first case of a durable radiographic and clinical response of a PTPR to bevacizumab, an antibody against vascular endothelial growth factor, despite multiple prior treatments. Bevacizumab may be an effective treatment for PTPR.

Keywords: Angiogenesis; Bevacizumab; Pineal tumor.

Fig. 1

HE-stained sections of the tumor demonstrate an epithelial tumor with papillary features (left; HE ×400). The tumor is strongly positive for keratin immunostaining (right; Cam5.2 ×600).

Fig. 2

MRI of the tumor 2 months before (a, b) and then again immediately before (c, d) bevacizumab treatment. a Magnetic resonance (MR) axial T2 image of the posterior fossa shows a large heterogeneous mass (white arrow) in the posterior cerebellum with surrounding hyperintense signal likely related to vasogenic edema and treatment effects. b Post-contrast T1-weighted image with fat saturation shows heterogeneous enhancement of the mass (white arrow) related to the patient's known metastatic disease from his PTPR. c MR axial FLAIR image of the posterior fossa shows an increase in the size of the heterogeneous mass (white arrow) in the posterior cerebellum with surrounding hyperintense signal likely related to vasogenic edema and treatment effects. d Post-contrast T1-weighted image with fat saturation shows an increase in heterogeneous enhancement of the mass (white arrow) related to the patient's known metastatic disease from his PTPR. The mass measured 4.6 cm. A linear focus of enhancement along the left fourth ventricle is related to the patient's metastatic disease.

Fig. 3

MRI of the tumor after bevacizumab treatment. a Magnetic resonance axial FLAIR image of the posterior fossa shows a decrease in the size of the heterogeneous mass (white arrow) in the posterior cerebellum with a decrease in the associated edema after bevacizumab. The extraventricular drain is partially visualized (black arrow). b Post-contrast T1-weighted image with fat saturation shows a decrease in the heterogeneously enhancing mass (white arrow) after bevacizumab. The mass measured 2 cm and was stable for 13 months. The extraventricular drain is partially visualized (black arrow).

Fig. 4

MRI of the tumor after 13 months of bevacizumab treatment. a Magnetic resonance axial FLAIR image of the posterior fossa shows an increase in the size of the heterogeneous mass (white arrow) in the posterior cerebellum with an increase in the associated edema. The extraventricular drain is partially visualized (black arrow). b Post-contrast T1-weighted image with fat saturation shows an increase in the heterogeneously enhancing mass (white arrow) after bevacizumab. The extraventricular drain is partially visualized (black arrow).

Fig. 5

MRI of the tumor after 5 months of bevacizumab and etoposide treatment. a Magnetic resonance axial FLAIR image of the posterior fossa shows stability in the size of the heterogeneous mass (white arrow) in the posterior cerebellum. The extraventricular drain is partially visualized (black arrow). b Post-contrast T1-weighted image with fat saturation shows stability in the heterogeneously enhancing mass (white arrow). The extraventricular drain is partially visualized (black arrow).