Malignant evolution of presumed benign lesions in the brain in neurofibromatosis: case report

Carella, Antonella; Medicamento, N.

doi:10.1007/s002340050483

Cited by 13 publications

(7 citation statements)

References 4 publications

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“…A similar suggestion was made earlier by Carella et al [26]. However, larger studies will be necessary to establish a firm correlation between lesions and development of high-grade astrocytomas in NF1 patients.…”

Section: Discussionsupporting

confidence: 82%

Glioblastoma Multiforme in the Posterior Cranial Fossa in a Patient with Neurofibromatosis Type I

Broekman

Risselada²,

Engelen-Lee³

et al. 2009

Case Reports in Medicine

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Patients with Neurofibromatosis type 1 (NF1) have an increased risk of developing neoplasms. The most common brain tumors, found in 15%–20% of NF1 patients, are hypothalamic-optic gliomas, followed by brainstem and cerebellar pilocytic astrocytomas. These tumors generally have a benign nature. NF1 patients are predisposed to a 5-fold increased incidence of high-grade astrocytomas, which are usually located in supratentorial regions of the brain. We present an NF1 patient who developed a high-grade astrocytoma in the posterior fossa and discuss possible pathophysiological mechanisms.

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Section: Discussionsupporting

confidence: 82%

Glioblastoma Multiforme in the Posterior Cranial Fossa in a Patient with Neurofibromatosis Type I

Broekman

Risselada²,

Engelen-Lee³

et al. 2009

Case Reports in Medicine

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“…Our patients with CGNF1 were also older than patients with SCG, despite a much smaller tumor volume. This suggests that the growth rate is slower in CGNF1 than in SCG, in contrast with earlier findings [2,4,10].…”

Section: Discussioncontrasting

confidence: 56%

“…It could be related, in both phakomatoses, to a disturbance of maturation and migration of the astrocytic precursor, since neurofibromin is involved in cell migration and differentiation, in addition to oncogene suppression [15]. Carella et al postulated that these tumors could result from the degeneration of white matter hamartomatous lesions known as unidentified bright objects (UBO) [2]. However, the region of the roof of the IV ventricle is not a common site for UBO [3,12], and the findings in our patients, with MRI performed soon after, or even before, the appearance of the tumor, do not substantiate this view.…”

Section: Discussionmentioning

confidence: 99%

“…Although less common, cerebellar gliomas are not exceptional, and occupy the second place in terms of frequency in the study of NF1-associated gliomas published by Ilgren et al [4]. The prognosis of cerebellar gliomas associated with NF1 (CGNF1) is not well established, but many authors consider that they are more malignant than sporadic cerebellar gliomas (SCG) [2,7,9]. However, the rare series reported in the literature are old and small [4,11], and both the pathology and the natural history of CGNF1 are still poorly understood [15].…”

Section: Introductionmentioning

confidence: 99%

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Cerebellar gliomas in children with NF1: pathology and surgery

Vinchon¹,

Soto-Ares

et al. 2000

Child's Nerv Syst

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Cerebellar gliomas associated with NF1 (CGNF1) are rarely reported in the literature, and they are considered to be malignant in a high proportion of cases. In an attempt to improve the definition of this disease and clarify its management, we reviewed our patients with CGNF1 and compared their tumors with sporadic cerebellar gliomas (SGC). We operated on six children with CGNF1, all but one of whom were asymptomatic. They represented one-tenth of all pediatric cerebellar gliomas, and one third of NF1-associated gliomas seen in our institution. CGNF1 appeared at a later age than SCG. They are seated near the roof of the IV ventricle and are not related to white matter hypersignal hamartomas. Most of these tumors showed radiological progression. They were four pilocytic astrocytomas, one ganglioglioma, and one malignant astrocytoma. One patient had tumor recurrence after 8 years, and the others are still disease free. The overall outcome appeared to be better for GCNF1 than for SCG. On account of the regular growth, uncertain pathology, and good surgical outcome, we advocate systematic resection of these tumors.

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“…There are few reports on the natural course of CNS tumors in NF1 19 . Spontaneous regression of optic gliomas has been reported, 20–23 while there seem to be few reports of malignant progression 24 …”

Section: Discussionmentioning

confidence: 99%

Neurofibromatosis type 1‐associated unusual pleomorphic astrocytoma displaying continual malignant progression

Yokoo

Kamiya

Sasaki

et al. 2001

Pathology International

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Patients with neurofibromatosis type 1 (NF1) often have gliomas as a complication, most of which are benign pilocytic astrocytomas which have arisen in optic pathways. In the present case, a 17-year-old girl (at death) with stigmata of NF1, initially had a bulky tumor mass in the left thalamus, developing into the lateral ventricle, at 13 years of age. Partially resected tissue samples showed pleomorphic astrocytoma with abundant xanthoma cells and degenerative structures such as Rosenthal fibers (RF) and eosinophilic granular bodies. Fine eosinophilic granules identical to RF, both immunophenotypically and ultrastructurally, were also seen. The residual tumor was subtotally resected 6 months later, and the tumor histology was essentially similar as before, accompanying the regenerative structures; this was believed to be a good prognostic indicator. However, several anaplastic features such as mitosis, necrosis and vascular proliferation appeared even in areas rich in the regenerative structures. After a 2-year, disease-free interval, multiple tumor relapse occurred in June 1997. Partially resected tumor tissues were composed of monotonous small anaplastic cells with prominent proliferative activity. Surprisingly, the tumor cells had retained eosinophilic granules within the cell bodies. Postoperative chemotherapy with procarbazine, MCNU and vincristine (PCV) suppressed the residual tumor dramatically, but the regrowing tumor finally became uncontrollable, leading to the patient's death. TP53 mutation was not detected, while p27 immunopositivity was constantly high during malignant progression, suggesting acquisition of proliferative activity to overcome p53 and p27 inhibitory functions. A review of previously published reports failed to reveal any cases of this type.

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Malignant evolution of presumed benign lesions in the brain in neurofibromatosis: case report

Cited by 13 publications

References 4 publications

Glioblastoma Multiforme in the Posterior Cranial Fossa in a Patient with Neurofibromatosis Type I

Glioblastoma Multiforme in the Posterior Cranial Fossa in a Patient with Neurofibromatosis Type I

Cerebellar gliomas in children with NF1: pathology and surgery

Neurofibromatosis type 1‐associated unusual pleomorphic astrocytoma displaying continual malignant progression

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