Desmoplastic Infantile Ganglioglioma

Munnynck, K. De; Gool, SW Van; Calenbergh, Frank Van; Demaerel, P.; Uyttebroeck, A; Buyse, Gunnar; Sciot, Raf

doi:10.1097/00000478-200211000-00015

Cited by 71 publications

(7 citation statements)

References 12 publications

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“…2–4,8,13,23,36,39 Accordingly, these tumors were large, peripheral supratentorial tumors with a primary cystic component and a cortical-based enhancing tumor nodule (Fig. 1).…”

Section: Resultsmentioning

confidence: 99%

“…3,17,29,34 Classically, DIA/DIGs have been described as massive cystic lesions that emanate from firm nodules that are invariably supratentorial and often attached to the overlying dura. 2–4,8,13,23,36,39 The tumor nodules tend to be homogeneously enhancing, hypodense on T2-weighted imaging, and are not associated with significant vasogenic edema. 3 Although there are several studies demonstrating multifocal disease, progression, metastasis, and/or malignant transformation to glioblastoma, 7,8,16,21,24,32,37 these more aggressive forms of DIA/DIGs tend to be exceptions rather than the rule.…”

Section: Discussionmentioning

confidence: 99%

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

Naylor¹,

Wohl²,

Raghunathan³

et al. 2018

Journal of Neurosurgery: Pediatrics

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OBJECTIVEThe 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).METHODSData 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.RESULTSTwo 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.CONCLUSIONSPostoperative 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.

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“…2–4,8,13,23,36,39 Accordingly, these tumors were large, peripheral supratentorial tumors with a primary cystic component and a cortical-based enhancing tumor nodule (Fig. 1).…”

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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

Naylor¹,

Wohl²,

Raghunathan³

et al. 2018

Journal of Neurosurgery: Pediatrics

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“…On the other hand, there are cases of DIG with unpredictable behavior. It has been argued that the outcome seems to be dependent on the location of the tumor, and that a suprasellar location may be associated with more aggressive behavior [14, 15]. The role of adjuvant chemotherapy and radiotherapy in cases of incomplete resection is dubious.…”

Section: Discussionmentioning

confidence: 99%

“…The disease-free survival has been reported to be 8–20 years [1, 15]. An accurate diagnosis is of paramount importance because DIG have a completely different prognosis from other malignant brain tumors that have almost the same neuroradiological and histological findings.…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic Infantile Ganglioglioma

Alexiou¹,

Stefanaki

Sfakianos³

et al. 2008

Pediatr Neurosurg

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Desmoplastic infantile gangliogliomas (DIG) are rare intracranial tumors that typically occur in infants and involve the cerebral cortex and the leptomeninges. They are usually very large in size and partially cystic. Total resection, if possible, is the treatment of choice, without the need for further adjuvant therapy. We report 2 cases of DIG. In both cases, computed tomography and magnetic resonance imaging revealed supratentorial mixed cystic and solid tumors, which presented as large cystic components with intense contrast enhancement of a mural nodule. The tumors were completely removed by surgery. The histological diagnosis was DIG. On follow-up examinations no tumor recurrence was noted.

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“…Although gross total resection is typically curative, tumor location may prevent complete tumor excision. Additionally, tumor recurrence, progression, and rarely leptomeningeal dissemination have been reported [ 2 , 9 ], underscoring the need for adjuvant treatment.…”

mentioning

confidence: 99%