Desmoplastic ganglioglioma: report of a non-infantile case

Romero, Flávio Ramalho; Listik, Sérgio; Fabris, P

doi:10.1590/s0004-282x2011000200022

Cited by 2 publications

(3 citation statements)

References 4 publications

(14 reference statements)

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“…The characteristic clinical presentation of DNIGs, as previously described, involves in the majority of the cases seizures, followed by headaches and other less common symptoms [3][4][5][6]. The lesions are mainly localized within the temporal lobes followed by parietal and frontal and to a lesser extent within the occipital lobes [3].…”

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confidence: 72%

“…Although these tumors were thought initially to appear only in infants, after the first publication by Kuchelmeister et al in 1993, who presented two such neoplasms in older individuals (15 and 25 years of age), it became obvious that they could also arise in other age groups [2]. Therefore, the name desmoplastic non-infantile ganglioglioma (DNIG) has been used to encompass these cases and since then a total of 25 cases has been published [3][4][5][6][7][8].…”

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confidence: 99%

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Desmoplastic Non-Infantile Ganglioglioma; An Incidental Finding with Unusual Localization

Kanakis¹

2017

TNN

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Desmoplastic non-infantile ganglioglioma (DNIG) is a rare tumor entity and only a small number of cases have been published until now in the literature. Although its infantile counterpart (desmoplastic infantile ganglioglioma [DIG]) has been already recognized and included together with desmoplastic infantile astrocytoma (DIA) in the WHO Classification of CNS tumors, DNIG has not been accepted as a distinct tumor entity. The reason for this is obviously the scarcity of the neoplasm and subsequently the absence of substantial information regarding its pathogenesis and its association to the infantile form.Herein we present an interesting case with an unremarkable clinical presentation, which came to medical attention after the performance of imaging investigations, for staging purposes of a multifocal papillary thyroid carcinoma. Although the tumor showed the typical biphasic nature, consisting of a solid and a cystic component, its localization in the left ventricular trigonum, was indeed uncommon. Finally, both the histomorphological and the immunohistochemical findings were consistent with the diagnosis of a desmoplastic non-infantile gangioglioma.The occurrence of DNIG with the various clinical manifestations as well as the diverse imaging features, underlines the need of histopathological examination, in order to establish the final diagnosis.

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Desmoplastic Non-Infantile Ganglioglioma; An Incidental Finding with Unusual Localization

Kanakis¹

2017

TNN

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“…Gross total resection is always associated with far superior outcomes in solitary DIA/DIG ( 25 , 26 ). But the treatment of multifocal DIA/DIGs can be more complex due to the multifocal location.…”

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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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Desmoplastic ganglioglioma: report of a non-infantile case

Cited by 2 publications

References 4 publications

Desmoplastic Non-Infantile Ganglioglioma; An Incidental Finding with Unusual Localization

Desmoplastic Non-Infantile Ganglioglioma; An Incidental Finding with Unusual Localization

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

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