Desmoplastic Infantile Ganglioglioma

Alexiou, George Α.; Stefanaki, Kalliopi; Sfakianos, George; Prodromou, Neofytos

doi:10.1159/000149913

Cited by 23 publications

(5 citation statements)

References 25 publications

(15 reference statements)

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“…The number of patients included in each reviewed paper ranged from a single patient in a case report to 250 patients in a literature review 24,25 . These 44 papers yielded 468 cases of intracranial tumors in children 6 months of age and less 1,3–5,8–10,12,13,15,17–19,21,26,27,29–31,33–35,37,38,40–46,51–55,57–59,61,62,65–67 . There was significant variability in brain tumor histologies (Table 2).…”

Section: Resultsmentioning

confidence: 99%

Surgical Treatment of Brain Tumors in Infants Younger than Six Months of Age and Review of the Literature

et al. 2012

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Object Brain tumors are rare in infants under 6-months of age. These tumors can be challenging to treat surgically. We analyzed a modern series of patients treated by a multidisciplinary team at a tertiary care center and performed a literature review of this unique population. Methods Retrospective clinical data was collected for patients surgically treated for intracranial mass lesions at The Children’s Hospital of Philadelphia from 1998 to 2007. Dermoid cysts and other skull-based lesions were excluded from the analysis. Results Sixteen patients under 6-months of age underwent surgery for primary intracranial mass lesions. The median age of the patients at surgery was 5.2 months (range 1.4 to 6 months of age). Children most often presented with a bulging fontanelle, hydrocephalus, or macrocephaly (7 patients). Vomiting was seen in 5 patients, cranial nerve palsies in 1 patient, and seizures in 3 patients. All patients had tumor resections and post-operatively were monitored in the intensive care unit. The final pathology consisted of atypical teratoid/rhabdoid tumor (3 cases), primitive neuroectodermal tumor/medulloblastoma (3 cases), choroid plexus papilloma (2 cases), astrocytoma (2 cases), ganglioglioma (2 cases), desmoplastic infantile ganglioglioma (2), glioblastoma multiforme (1), and choroid plexus carcinoma (1). Two intra-operative deaths occurred. Of the surviving 14, a gross total resection was achieved in 4. Adjuvant therapy was determined by a multidisciplinary team composed of neuro-oncology, neurosurgery, and radiation oncology. Seven patients were treated with chemotherapy, 1 patient had proton beam therapy. Five-year overall survival was 45%. The eight surviving patients had neurological sequelae, and developmental outcome was variable. Conclusions Brain tumors are uncommon in children under 6-months of age. Patients present with a variety of tumor pathologies. Children who survive have neurological sequelae. More studies are necessary to understand the impact that different treatment options, tumor pathology, and tumor location have on neurological outcome.

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Section: Resultsmentioning

confidence: 99%

Surgical Treatment of Brain Tumors in Infants Younger than Six Months of Age and Review of the Literature

et al. 2012

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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%

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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“…Most patients with DIG present with new onset seizures [2,5,6], with or without focal neurological signs such as hemiparesis. Increased intracranial pressure and lethargy might be the only findings [7]. CT scans and MR images show large cystic tumors with enhancing solid components [6-9].…”

Section: Discussionmentioning

confidence: 99%

“…CT scans and MR images show large cystic tumors with enhancing solid components [6-9]. The frontal and temporal lobes are the most common sites of occurrence [2,6,7], wherein the tumor usually abuts the meningeal surface [10] and attaches to the dura. The tumors are firm and avascular with a dense desmoplastic component.…”

Section: Discussionmentioning

confidence: 99%

Mixed neuronal-glial tumor in the temporal lobe of an infant: a case report

et al. 2013

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BackgroundTumors that arise in the temporal lobes of infants and spread to the neural system are limited to several diagnoses. Herein, we present an infantile case of a temporal tumor showing neuronal and glial differentiation.Case presentationThe patient was a 9-month-old boy with low body weight due to intrauterine growth retardation. At 9 months after birth, he presented partial seizures. Computed tomography scanning revealed a mass (35 * 40 mm) in the left temporal lobe. Isointensity was noted on magnetic resonance T1-weighted images and fluid attenuation inversion recovery images. The tumor was heterogeneously enhanced with gadolinium. Positron emission tomography showed high methionine uptake in the tumor. During surgery, the tumor, which was elastic and soft and bled easily, was gross totally resected. A moderately clear boundary was noted between the tumor and normal brain parenchyma. Histologically, the tumor mainly comprised a ganglioglioma-like portion and short spindle cells at different densities. The former was immunohistochemically positive for some kinds of neuronal markers including synaptophysin. The spindle cells were positive for glial fibrillary acidic protein, but desmoplasia was not observed.DiscussionThe tumor contained both neuronal and glial elements; the former were the main constituents of the tumor and included several ganglion-like cells. Because neuronal elements gradually transited to glial cells, a mixed neuronal-glial tumor was diagnosed.Virtual SlidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/2045126100982604

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Desmoplastic Infantile Ganglioglioma

Cited by 23 publications

References 25 publications

Surgical Treatment of Brain Tumors in Infants Younger than Six Months of Age and Review of the Literature

Surgical Treatment of Brain Tumors in Infants Younger than Six Months of Age and Review of the Literature

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

Mixed neuronal-glial tumor in the temporal lobe of an infant: a case report

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