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

Abstract

<jats:sec> <jats:title>OBJECT</jats:title> <jats:p>Two of the more common infantile brain tumors, glioblastoma multiforme (GBM) and desmoplastic infantile tumors (DITs), can be difficult to distinguish on MRI. Both tumors occur in the supratentorial compartment and both have solid and cystic components. Differentiating between the 2 on MRI studies could assist the surgeon in discussions with family and child management. The authors report on their institutional experience with both tumors, focusing on radio-graphic features, especially the diffusion studies, which might be useful in distinguishing between infantile GBM and DIT.</jats:p></jats:sec> <jats:sec> <jats:title>METHODS</jats:title> <jats:p>A retrospective review was undertaken of all infantile brain tumors treated at British Columbia’s Children’s Hospital between 1982 and 2012, and cases of GBM and DIT were recorded. Only cases that had imaging were included in the study. A literature review was completed to identify reported cases of infantile GBM and DIT. Only reports that described or included radiological studies (particularly MRI) of the tumors were included. Certain radiographic features of the tumors were reviewed, including location, size, consistency, pattern of enhancement, and features on MR diffusion studies.</jats:p></jats:sec> <jats:sec> <jats:title>RESULTS</jats:title> <jats:p>Of 70 cases of infantile brain tumors, 2 GBM cases and 3 DIT cases (all 3 of which were desmoplastic infantile gangliogliomas [DIGs]) met the inclusion criteria. The radiological studies obtained in all 5 cases were reviewed by a neuroradiologist. All 5 patients had supratentorial tumors with cystic-solid consistency. Diffusion MRI studies showed restricted diffusion in the 2 GBM cases, but no evidence of restricted diffusion in the DIG tumors. The GBM tumors were heterogeneously enhancing, and the DIG tumors showed avid and homogeneous enhancement. The literature review revealed 29 cases of infantile GBM and 32 cases of DIG/DIT that met the inclusion criteria. The tumors were large in both groups. The tumors were cystic-solid in consistency in 10 of 30 (33%) of GBM cases and 28 of 32 (87.5%) of DIT cases. The contrast enhancement was heterogeneous in 9 of 30 (30%) GBM cases, and it was homogeneous and avid in 27 of 32 (84%) of DIT cases. Diffusion studies were recorded in 2 published infantile GBM cases, and in both of them diffusion was restricted. The authors only found 1 report that discussed DIG tumor features on MR diffusion studies, but the interpretation was difficult and unclear.</jats:p></jats:sec> <jats:sec> <jats:title>CONCLUSIONS</jats:title> <jats:p>Magnetic resonance imaging, especially diffusion-weighted imaging, may be a useful aid in distinguishing between infantile GBM and DIT tumors, with infantile GBM demonstrating restricted diffusion.</jats:p></jats:sec>

Perinatal (fetal and neonatal) astrocytoma: a review

“…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).…”

“…The initial benign histology and clinical findings in the ten patients in this review are more consistent with a grade I–II tumor rather than a high-grade one. Nevertheless, it should be mentioned that some DIAGAs reported in some older children and adults have been diagnosed malignant [2, 62]. …”

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

“…17,33,39 Males are more commonly affected by DIA/DIG than females (1.5–1.7:1), and patients almost always present with rapidly enlarging head circumference and, sometimes, vomiting and seizures. 3,17,29,34 Classically, DIA/DIGs have been described as massive cystic lesions that emanate from firm nodules that are invariably supratentorial and often attached to the overlying dura. 2–4,8,13,23,36,39 The tumor nodules tend to be homogeneously enhancing, hypodense on T2-weighted imaging, and are not associated with significant vasogenic edema.…”

“…2–4,8,13,23,36,39 The tumor nodules tend to be homogeneously enhancing, hypodense on T2-weighted imaging, and are not associated with significant vasogenic edema. 3 Although there are several studies demonstrating multifocal disease, progression, metastasis, and/or malignant transformation to glioblastoma, 7,8,16,21,24,32,37 these more aggressive forms of DIA/DIGs tend to be exceptions rather than the rule. 17 Generally, DIA/DIGs are considered benign and are often cured by resection alone.…”

“…1,5,34 Resection is curative, and chemotherapy and/or radiation therapy is unnecessary in the majority of cases. 1,3,26,34,35 Indeed, DIA/DIGs are now categorized together in the 2016 WHO classification, are designated grade I, and generally have a good prognosis with long disease-free survival. 1,5,15,26,32,34,35,39 How- ever, there have been a few case reports of DIA/DIGs with atypical presentations, including primary vermian localization, 1 primary spinal localization, 25 metastatic spread, 32 multiple cerebral lesions, 1,18,28,32 and even occurrence in patients older than 6 years.…”