Desmoplastic infantile ganglioglioma ? clinicopathological and immunohistochemical study of four cases

Rout, Pritilata; Santosh, Vani; Mahadevan, Anita; Kolluri, V. R. Sastry; Yasha, T C; Shankar, S K

doi:10.1007/s00381-002-0610-3

Cited by 32 publications

(22 citation statements)

References 22 publications

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“…Desmoplastic cerebral astrocytoma/desmoplastic cerebral ganglioglioma of infancy ( DIAGA ) occurs most often during the first year of life than at any other time [2, 4, 20, 25, 30, 45, 47, 62, 71, 81, 83, 86]. The diagnosis was made in this review in nine neonates and in one fetus (Table 6).…”

Section: Resultsmentioning

confidence: 99%

Perinatal (fetal and neonatal) astrocytoma: a review

Isaacs

2016

Childs Nerv Syst

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Introduction The purpose of this review is to document the various types of astrocytoma that occur in the fetus and neonate, their locations, initial findings, pathology, and outcome. Data are presented that show which patients are likely to survive or benefit from treatment compared with those who are unlikely to respond. Materials and methods One hundred one fetal and neonatal tumors were collected from the literature for study. Results Macrocephaly and an intracranial mass were the most common initial findings. Overall, hydrocephalus and intracranial hemorrhage were next. Glioblastoma (GBM) was the most common neoplasm followed in order by subependymal giant cell astrocytoma (SEGA), low-grade astrocytoma, anaplastic astrocytoma, and desmoplastic infantile astrocytoma (DIA). Tumors were detected most often toward the end of the third trimester of pregnancy. Conclusion A number of patients were considered inoperable since their tumor occupied much of the intracranial cavity involving large areas of the brain. High-grade astrocytomas were more common than low-grade ones in this review. Fetuses and neonates with astrocytoma have a mixed prognosis ranging from as low as 20 % (GBM) to a high of 90 %. The overall survival was 47/101 or 46 %.

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Section: Resultsmentioning

confidence: 99%

Perinatal (fetal and neonatal) astrocytoma: a review

Isaacs

2016

Childs Nerv Syst

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“…6,17,26,27,38,39 While DIG and DIA are considered to be the same entity clinically, 14,39 they differ histologically in that the neoplastic cells in DIAs appear restricted to astrocytes, while DIGs include a neuronal component or variable maturity DIAs. 6,38,39 Distinguishing DIG and DIA requires demonstration of a neoplastic neuronal component.…”

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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“…Desmoplastic infantile astrocytomas (DIAs) are rare, benign, infantile brain tumors representing 15.8% of infantile intracranial tumors and 1.5% of all childhood intracranial tumors [1,2] . They are most frequently seen in children under 18 months of age and have a predilection for males [3] .…”

Section: Introductionmentioning

confidence: 99%

Unusual Occurrence of Multifocal Desmoplastic Infantile Astrocytoma: A Case Report and Review of the Literature

Narayan

Savardekar²,

Mahadevan³

et al. 2017

Pediatr Neurosurg

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Desmoplastic infantile gliomas are rare, benign tumors of the early infancy period. Two histological subtypes - desmoplastic infantile astrocytoma (DIA) and desmoplastic infantile ganglioglioma - have been described. The characteristic features of DIAs are lobar location, glial histology, and excellent prognosis after complete surgical excision. DIAs usually present as solitary, cortical-surfacing, solid-cystic neoplasms; however, atypical, aggressive, and multifocal variants of DIA have been reported in the literature. These rare DIAs presenting with multiple lesions pose a diagnostic as well as a therapeutic dilemma. We report an unusual case of an 8-month-old female infant diagnosed with multifocal (cranial and spinal) DIA and obstructive hydrocephalus and discuss the radiological and histological features of this rare variant of DIA. The patient underwent ventriculoperitoneal shunt placement to relieve the hydrocephalus, excisional biopsy from a surfacing lesion in the right frontal lobe, and multiple cycles of chemotherapy; however, the lesions continued to progress, and the patient is likely to have an unfavorable outcome.

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Desmoplastic infantile ganglioglioma ? clinicopathological and immunohistochemical study of four cases

Cited by 32 publications

References 22 publications

Perinatal (fetal and neonatal) astrocytoma: a review

Perinatal (fetal and neonatal) astrocytoma: a review

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

Unusual Occurrence of Multifocal Desmoplastic Infantile Astrocytoma: A Case Report and Review of the Literature

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