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

Naylor, Ryan M.; Wohl, Anton; Raghunathan, Aditya; Eckel, Laurence J.; Keating, Gesina F.; Daniels, David J.

doi:10.3171/2018.4.peds17638

Cited by 8 publications

(5 citation statements)

References 36 publications

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“…The 2016 WHO classification of Nervous system categorized DIA and DIG together ( 18 , 19 ) and classified as grade I ( 3 ). DIA/DIGs are generally solitary and have a favorable prognosis with long-term disease-free survival after surgical resection ( 20 ). DIA/DIGs with multifocal lesions are uncommon, and little is known about their clinical features and management.…”

Section: Discussionmentioning

confidence: 99%

Multifocal Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIA/DIG): An Institutional Series Report and a Clinical Summary of This Rare Tumor

Wang

Cheng

et al. 2022

Front. Oncol.

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AimMultifocal desmoplastic infantile ganglioglioma/astrocytoma (DIA/DIG) has rarely been reported. Here, two cases have been presented, reviewing the literature and proposed treatment algorithms for this rare tumor.Patients and MethodsWe report two patients diagnosed with multifocal DIA/DIGs in West China Hospital. In addition, a literature review was performed, in October 2019, on case reports of DIA/DIGs with multifocal lesions. The clinical and radiological features, treatment, and outcome of this rare disease were discussed.ResultsDIA/DIGs with multifocal locations were rare, and only thirteen cases (including ours) had been reported. This series included 8 males and 5 females with a mean age of 31.4 ± 45.7 months (range, 3-144 months). The supratentorial hemisphere, suprasellar region, posterior cranial fossa, and spinal cord were frequently involved. Ten patients (76.9%) received surgical resection for the symptomatic lesions and three patients (23.1%) underwent biopsy. Seven patients received chemotherapy postoperatively. Six individuals had tumor recurrences during the follow-up period, while three patients had tumors that spontaneously regressed. Finally, two patients died of tumor progression and one patient died of respiratory insufficiency and hypothalamic dysfunction.ConclusionsMultifocal DIA/DIGs have more aggressive clinical behavior and poor outcome despite benign histology. DIA/DIGs should be included in the differential diagnosis of multifocal brain tumors in children. The mainstay of treatment is surgical resection; adjuvant treatment with chemotherapeutic drugs is unknown and requires additional research.

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Section: Discussionmentioning

confidence: 99%

Multifocal Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIA/DIG): An Institutional Series Report and a Clinical Summary of This Rare Tumor

Wang

Cheng

et al. 2022

Front. Oncol.

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“…Chaterjee et al presented two cases of the non-infantile variant of DIA with the canonical V600E mutation, describing this mutation as frequent in DIG-DIA tumors [ 12 ]. Two more cases were published in 2018, one negative for BRAF V600E mutation and the other positive [ 22 , 23 ]. In 2019, Guerreiro Stucklin et al established the relevance of ALK / ROS1 / NTRK / MET alterations in infant gliomas, especially in high-grade gliomas [ 37 ].…”

Section: Discussionmentioning

confidence: 99%

Desmoplastic infantile astrocytoma with atypical phenotype, PTEN homozygous deletion and BRAF V600E mutation

Megías

San-Miguel

Sánchez

et al. 2022

acta neuropathol commun

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Desmoplastic infantile astrocytoma (DIA) is rare, cystic and solid tumor of infants usually found in superficial cerebral hemispheres. Although DIA is usually benign, uncommon cases bearing malignant histological and aggressive clinical features have been described in the literature. We report a newborn patient who was diagnosed with a DIA and died postresection. Pathologic examination revealed that the main part of the tumor had benign features, but the internal region showed areas with a more aggressive appearance, with higher-proliferative cells, anaplastic GFAP positive cells with cellular polymorphism, necrosis foci, vascular hyperplasia with endothelial proliferation and microtrombosis. Genetic study, performed in both regions of the tumor, showed a BRAF V600E mutation and a homozygous deletion in PTEN, without changes in other relevant genes like EGFR, CDKN2A, TP53, NFKBIA, CDK4, MDM2 and PDGFRA. Although PTEN homozygous deletions are described in gliomas, the present case constitutes the first report of a PTEN mutation in a DIA, and this genetic feature may be related to the malignant behavior of a usually benign tumor. These genetic findings may point at the need of further and deeper genetic characterization of DIAs, in order to better understand the biology of this tumor and to obtain new prognostic approaches, a better clinical management and targeted therapies, especially in malignant cases of DIA.

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“…However, desmoplastic astrocytomas can also be found in adolescence. 4 DIAs usually are of large size, with acute and subacute clinical symptoms. Younger patients may present neurological symptoms early after birth, but often not further investigated, [5][6][7] which leads to the progression of the disease.…”

Section: Discussionmentioning

confidence: 99%

Purely Intraventricular Desmoplastic Infantile Astrocytoma

Nicoletti

Umana

Riolo

et al. 2021

Journal of Pediatric Neurology

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Desmoplastic infantile astrocytomas (DIAs) are rare pediatric tumors characterized by superficial brain cortex involvement, along with the meninges of the supratentorial compartment, and are classified as grade I neoplasms according to the 2016 World Health Organization. A 5-year-old female patient was admitted to our unit with abnormal decorticate posturing, bilateral mydriasis with weak pupillary light reflex, and brisk lower limbs reflexes. Her medical history was unremarkable. Magnetic resonance imaging of the brain revealed a massive lesion with bilateral intraventricular growth mainly prevailing on the left and involving the ipsilateral foramen of Monro. After external ventricular drainage positioning, Dandy's transfrontal transcortical approach to the left lateral ventricle, a meticulous ependymal microsurgical dissection of the lesion was performed, resulting in an excision of the cystic component of the left lateral ventricle. A gross total removal of the lesion was performed with an en bloc resection of the deeper cystic part. Thirty days after surgery, the patient presented with fluent speech, conserved axial, and extremity sensorimotor functions, except a mild central facial paresis which progressively improved. To the best of our knowledge, this is the first case of DIA characterized by purely intraventricular growth. Tumor recurrence, although considered rare, represents an unpredictable event. Therefore, an adequate follow-up must be reserved for each patient.

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

Cited by 8 publications

References 36 publications

Multifocal Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIA/DIG): An Institutional Series Report and a Clinical Summary of This Rare Tumor

Multifocal Desmoplastic Infantile Ganglioglioma/Astrocytoma (DIA/DIG): An Institutional Series Report and a Clinical Summary of This Rare Tumor

Desmoplastic infantile astrocytoma with atypical phenotype, PTEN homozygous deletion and BRAF V600E mutation

Purely Intraventricular Desmoplastic Infantile Astrocytoma

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