Desmoplastic non-infantile ganglioglioma: A low-grade tumor, report of two patients

Khubchandani, Shaila; Chitale, Arun R.; Doshi, Paresh K

doi:10.4103/0028-3886.59482

Neurol India

2009

DOI: 10.4103/0028-3886.59482

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Desmoplastic non-infantile ganglioglioma: A low-grade tumor, report of two patients

Shaila Khubchandani

Arun R. Chitale

Paresh K Doshi

Abstract: Desmoplastic infantile gangliogliomas (DIGG) are low-grade (WHO Grade I) tumors of early childhood rarely occurring in older children and young adults. The mainstay of treatment is surgical. We report two older children with DIGG, both showing atypical radiology. One case was treated with radiation. The recurrence treated with only surgical excision showed excellent response to treatment.

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“…Besides, herein as in our case, the solid component of the tumor was medial to its cystic portion. Likewise, two more cases have been described by two different groups, in which the lesions were also centrally localized and more precisely in the left peritrigonal region, with the only difference to ours being the absence of a cystic component in both instances [9,10].…”

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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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Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature

2011

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Desmoplastic gangliogliomas (DIG) are rare primary neoplasms that comprise 0.5-1.0% of all intracranial tumors. Clinically, there are two forms of DIG, the infantile and the non-infantile. These tumors invariably arise in the supratentorial region and commonly involve more than one lobe, preferentially the temporal and frontal. On neuroimaging are seen as large hypodense cystic masses with a solid isodense or slightly hyperdense superficial portion. The histologic diagnosis is characterized by the presence of three different cell lines: astrocytic, neuronal, and primitive neuroectodermal marker sites, which were demonstrable. The treatment of choice is radical surgical excision, and if this is done, achieved complete healing of the patient does not require additional treatment. A literature review of DIG was compiled through Medline/Ovid using the keywords "desmoplastic infantile ganglioglioma", "desmoplastic non-infantile ganglioglioma" covering the years 1984-2009. We present a review of a total of 113 cases of infantile (94) and non-infantile gangliogliomas (19) published to date, examining the clinical, radiologic, surgical, and pathological aspects, as well as the outcome. Desmoplastic gangliogliomas represent a rare tumor group with two well-defined age groups, the children and non-children. Desmoplastic infantile gangliogliomas are the most common and occur in children below 5 years of age, and the large majority of them present within the first year of life. Surgery is the treatment of choice and no complementary treatment is needed in cases of complete tumor resection.

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Desmoplastic infantile ganglioglioma: A rare entity

Diyora¹,

Chhajed²,

Dhall³

et al. 2023

Journal of Pediatric Neurosciences

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Desmoplastic infantile ganglioglioma (DIG) is a very uncommon intracranial tumor with an incidence of 0.1%–1.25%. It is most often seen in the first 2 years of life. We describe two cases of this unusual entity in a 5-month-old boy and a 15-month-old child, both of whom had a history of convulsions and were diagnosed as DIG on histopathology after surgery. Gross total excision resulted in a successful treatment outcome without the requirement of any additional treatment.

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Desmoplastic non-infantile ganglioglioma: A low-grade tumor, report of two patients

Cited by 3 publications

References 9 publications

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

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

Desmoplastic infantile and non-infantile ganglioglioma. Review of the literature

Desmoplastic infantile ganglioglioma: A rare entity

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