Brain Tumors

Tortori-Donati, Paolo; Rossi, Andrea; Biancheri, Roberta; Garrè, Maria Luisa; Cama, Armando

doi:10.1007/3-540-26398-5_10

Cited by 10 publications

(6 citation statements)

References 179 publications

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“…If also histology was considered, the vermis remained as the most frequent tumor site for most of the variants (100% for CMB, 83% for MBEN, and 80% for other variables) with the exception for DMB tumors where 6 of 10 tumors (60%) originated from the hemispheres. Seven of 12 cases (58%) with MBEN showed the typical MRI appearance described as ''grape like'' by Giangaspero et al (2); and reported by other authors (15,25).…”

Section: Resultsmentioning

confidence: 65%

Medulloblastoma Variants: Age-Dependent Occurrence and Relation to Gorlin Syndrome—A New Clinical Perspective

Garrè¹,

Cama²,

Bagnasco³

et al. 2009

Clinical Cancer Research

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110

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Purpose: We aimed to test the hypothesis that medulloblastoma (MB) variants show a different age distribution and clinical behavior reflecting their specific biology, and that MB occurring at very young age is associated with cancer predisposition syndromes such as Gorlin syndrome (GS). Experimental Design: We investigated the frequency, age distribution, location, response to treatment, outcome, and association with familial cancer predisposition syndromes in a series of 82 cases of MB in patients ages <14 years diagnosed at the Giannina Gaslini Children's Hospital, Genoa, between 1987 and. Results: Desmoplastic MB and MB with extensive nodularity (MBEN), were present in 22 of 82 cases (27%) and were more frequent in children ages V3 years (13 of 25; 52%). In this age group, MBEN was significantly more frequent than desmoplastic MB and classic MB (P < 0.001) and had a good prognosis. MBEN was associated with GS in 5 of 12 cases. Overall, 8 cases occurred in the context of familial tumor predisposition syndromes (5 GS, 1 each NF1, LiFraumeni, and Fragile X) and 7 of these patients were ages V3 years at diagnosis. Desmoplastic histology and a more intensive treatment represented independent favorable prognostic factors in multivariate analysis (P = 0.003 and P = 0.0139, respectively). Metastasis was a predictor of bad outcome (P = 0.0001). Conclusions: Our data indicate that biologically different MB entities warrant risk-adapted treatment and that MBEN is strongly associated with GS. Patients, ages V3 years, with MB and their families should be investigated for tumor predisposition syndromes such as GS. Medulloblastoma (MB) with extensive nodularity (MBEN)occurring in young children is a rare but well-defined entity (1 -3). It is believed to be related to, but distinct from, desmoplastic MB (DMB). However, many aspects of this peculiar entity are still unclear, including its frequency, why it occurs at a very young age, why its prognosis is good, and its association with Gorlin syndrome (GS) or nevoid basal cell carcinoma syndrome. Most of the largest cooperative trials that have been published on MB in infants do not report on the relative frequency of this distinct entity compared with other variants and/or comment on the effect of the diagnosis of this variant on subsequent follow-up and treatment strategies (4 -6). A large prospective study by Eberhart reported that 73 of 330 (22%) cases had various degrees of desmoplasia and 14 of 330 cases were MBEN (4.2%; ref. 7). A more recent, extensive neuropathogical review focused on the morphologic features and biological behavior of 2 large contemporaneous cohorts of patients with MB and showed that DMB represents 5% of all MB and up to 57% of cases in children ages <3 years (3). A recent prospective, cooperative trial reported that desmoplastic histologic variants have a clearly favorable effect on prognosis in infants with MB who are treated with chemotherapy (CT) as first-line therapy. However, it did not mention the frequency of Imaging, Diagnosis, Pro...

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Section: Resultsmentioning

confidence: 65%

Medulloblastoma Variants: Age-Dependent Occurrence and Relation to Gorlin Syndrome—A New Clinical Perspective

Garrè¹,

Cama²,

Bagnasco³

et al. 2009

Clinical Cancer Research

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110

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“…Radiologically, teratomas usually present as large heterogeneous masses with a variable amount of solid and cystic masses. A CT Scan will show missed density lesion with hypodense fatty components and hyperdense calcified portion indicating teeth or bone [6]. The presence of fat, calcification and solid components is highly suggestive of a teratoma.…”

Section: Discussionmentioning

confidence: 99%

Congenital Infantile Intracranial Teratoma with Orbital Extension

Rafay¹,

Shaikh²,

Sharif³

2018

Surgery Curr Res

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Background: Mature intracranial teratoma in the pediatric population is a rare entity. They are sometimes associated with genetic syndromes but complete surgical excision is associated with good outcome and prognosis. Case Presentation:A 3 months old baby girl presented in the clinic with left eye proptosis, MRI brain showed temporal and retro-orbital multiple cystic lesions, which were excised and biopsy showed mature teratoma. The patient was discharged and followed in the clinic. Conclusion:This case underlies that mature brain teratoma may present at an early age even at birth. They are rare intracranial tumors and may be associated with other genetic syndromes. A high index of suspicion should be present when coming across such complex lesions in neonates. Although the overall outcome of these tumors is generally poor, among the children that survive, complete microsurgical excision of the tumor is associated with good outcome and prognosis. In cases of mature teratomas, we advocate that complete removal should be carried out. In cases of the residual disease, reoperation is indicated to achieve good results.

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“…10 The cystic components present variable signal intensity in relation to protein or methe-moglobin content, ranging from hypointense to hyperintense on T1-weighted images, while being hyperintense on T2weighted images. 107 To explain the possible discrepancy on prenatal and postnatal imaging, it is suggested that CPs start out in the fetus as primarily solid lesions and subsequently degenerate, producing cystic components and calcifications (►Fig. 9).…”

Section: Congenital Craniopharyngiomasmentioning

confidence: 99%

“…116 On MRI, the cystic component is isointense to CSF, with unenhancing walls and may contain septations. [122][123][124][125] The solid component is hypointense on T2-weighted images, due to the intense desmoplastic reaction producing numerous fibrocollagenous bands 107,121,125 a feature that helps to differentiate this tumor from cystic astrocytoma (►Fig. 10).…”

Section: Desmoplastic Infantile Tumorsmentioning

confidence: 99%

Congenital Brain Tumors

Nikas

Themistocleous

Vraka

et al. 2016

J Pediatr Neuroradiol

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Congenital brain tumors, unlike those of older children and adults, are a rare entity, accounting for less than 2% in terms of incidence, of all childhood brain tumors. They differ in their location, histology, biological behavior, and the therapeutic response compared with those of older children. In this article, we review the most important types of congenital brain tumors mainly focusing on their imaging findings.

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Brain Tumors

Cited by 10 publications

References 179 publications

Medulloblastoma Variants: Age-Dependent Occurrence and Relation to Gorlin Syndrome—A New Clinical Perspective

Medulloblastoma Variants: Age-Dependent Occurrence and Relation to Gorlin Syndrome—A New Clinical Perspective

Congenital Infantile Intracranial Teratoma with Orbital Extension

Congenital Brain Tumors

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