Novel suprasellar location of desmoplastic infantile astrocytoma and ganglioglioma: a single institution's experience

Abstract

Objective: The aim of this study was to describe the clinical presentation, imaging appearance, and differential outcomes based on tumor location in 7 patients with desmoplastic infantile astrocytoma and desmoplastic infantile gangliogliomas (DIA/DIG).

Methods: Data of 7 patients with histopathology-proven DIA/DIGs and preoperative imaging were retrospectively reviewed, and age, sex, clinical presentation, imaging characteristics, tumor location, surgical procedure, postoperative morbidity, and overall mortality were recorded.

Results: Two subgroups of patients with DIA/DIGs were found to exist based on whether their tumor was located in the cerebral hemispheres or suprasellar region. Nearly all patients presented with rapidly enlarging head circumference regardless of tumor location. However, ocular abnormalities, including nystagmus and preference for downward gaze, were specific for patients with suprasellar disease. These patients experienced significant postoperative complications and had poor long-term outcomes. In contrast, patients with hemispheric tumors underwent more extensive resection than patients with suprasellar tumors, had uneventful postoperative courses, and had no documented long-term comorbidities.

Conclusions: Postoperative course and long-term outcome for patients with DIA/DIGs were correlated to the anatomical location and radiographic appearance of their tumor at presentation, despite having histologically and molecularly indistinguishable, WHO grade I tumors.

Keywords: DIA = desmoplastic infantile astrocytoma; DIG = desmoplastic infantile ganglioglioma; desmoplastic infantile astrocytoma; desmoplastic infantile ganglioglioma; neuroradiology; oncology; outcomes; pediatric neurosurgery.

FIG. 1.

A–C: Coronal postcontrast T1-weighted MR images of classically described hemispheric DIGs showing massive cysts with cortically enhancing peripheral nodularity. D–F: Coronal postcontrast T1-weighted MR images of suprasellar DIGs showing large, homogeneously enhancing tumors with small associated cysts.

FIG. 2.

Case 1. A and B: Preoperative coronal (A) and axial (B) postcontrast T1-weighted images obtained in a 3-month-old girl with a large, homogeneously enhancing suprasellar tumor, which presented with rapidly enlarging head circumference, nystagmus, and bilateral optic nerve atrophy. The patient underwent subtotal tumor resection and subsequently received chemotherapy and radiation therapy. C and D: Coronal (C) and axial (D) postcontrast T1-weighted images obtained in the same patient 8 years after the first resection, with recurrent tumor growth and cystic transformation. A second partial resection was subsequently performed.

FIG. 3.

Case 6. A and B: This 4-month-old girl presented with a rapidly enlarging head circumference. Axial (A) and coronal (B) postcontrast T1-weighted images showing a large cystic tumor with an enhancing peripheral cortical-based nodule in the right parietal lobe. C: Intraoperative image obtained in the same patient after opening the dura and wide exposure of right parietal lobe. The solid part of the tumor appears as an extra-axial superficial cortical component (black arrowhead). The cystic portion is also visible (white arrowhead). D: Intraoperative image obtained in the same patient after removal of the tumor. There is significant collapse of the right brain hemisphere. Also seen is the medial wall of the bed of the tumor (black arrowhead). E and F: Axial (E) and coronal (F) postcontrast T1-weighted images obtained in the same patient 1 year after surgery. Neither the cystic or peripheral nodular components are present. Figure is available in color online only.