Desmoplastic infantile astrocytoma: recurrence with malignant transformation into glioblastoma: a case report

Abstract

Desmoplastic infantile astrocytoma (DIA) is an uncommon brain tumor of early infancy. The tumor is characterized by a lobar location, glial histology, and excellent prognosis after surgical removal. DIA and a similar tumor, desmoplastic infantile ganglioglioma (DIG) have been considered to be benign neoplasms, but the prognosis of DIA and DIG is currently under question as atypical and aggressive clinical features of the tumors have been reported. We encountered a patient who was diagnosed with DIA at the age of 22 months and exhibited tumor recurrence 8 years later. Surgical removal of the recurred tumor revealed that the tumor had transformed to overt glioblastoma. This case demonstrates that DIA is not an absolutely benign tumor and that careful clinical surveillance is needed during the follow-up period.

Radiological features of infantile glioblastoma and desmoplastic infantile tumors: British Columbia’s Children’s Hospital experience

“…[1][2][3][4]9,11,14,15,19,20,22,23,27,30,[37][38][39][40]43,44 A homogeneously and avidly enhancing solid nodule was the most common finding among those cases (27 of 32). 1,3,4,9,11,14,15,19,22,27,30,[38][39][40]43 Either no contrast was given or the pattern of enhancement was interpreted as heterogeneous in 7 cases. 2,15,20,23,37,44 Only 1 publication reported the features of DIG on DWI; 37 however, the description was vague.…”

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

“…Presently, both pathological entities are considered to be histological subtypes of desmoplastic infantile gliomas and are classified as benign (WHO grade I) tumors according to the WHO classification (2016) of central nervous system tumors [5] . Despite the fact that DIAs are classified as WHO grade I tumors with favorable prognosis, DIAs with aggressive, clinical, and pathological features have been described, albeit as case reports [1] . DIAs are primarily solitary tumors but have been reported, albeit rarely, to occur as multiple localizations in the central nervous system ( Table 1 ) [4, 6-8, 11, 12] .…”

“…DIAs typically present as large, solitary, solid-cystic, supratentorial tumors, located on the cortical surface and having a dural attachment; however, aggressive variants with multifocal localizations or leptomeningeal spread have been described, although these are extremely rare [1] . Gross total resection of solitary DIA usually results in a good outcome, but management of patients with multifocal DIAs remains a dilemma because of very limited experience worldwide [1,6] . In this report, we describe the sixth case of DIA with intracranial and intraspinal lesions and review the pertinent literature ( Table 1 ) [4, [6][7][8] .…”

“…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] .…”

Congenital Glioblastoma Multiforme: An Unusual and Challenging Tumor

“…Even though overexpression of either p53 or EGFR has been associated with worse prognosis in cases of pediatric GBMs, their correlation with the biological behavior and clinical outcome of congenital GBMs remains unclear. 2,7,11 It is interesting to note that there are reported cases that have raised the possibility of GBM differentiation to a less malignant histological type 49 and, on the contrary, of benign tumors recurring as GBM, 50 even one such case related with a TP53 mutation. 51 Despite the fact that similar cases including congenital brain tumors do not exist in the literature, there are questions arising about malignant brain tumors' natural history that have to be thoroughly investigated.…”