User Tools

Site Tools


cerebellar_pilocytic_astrocytoma

Cerebellar pilocytic astrocytoma

Types

Children

Adult

Cerebellar pilocytic astrocytomas in adults should be treated with macroscopic complete surgical resection whenever possible. If this is achieved, long-term survival rates are excellent, whereas subtotal resection carries a high risk of tumour recurrence. Ki67 is less important prognostically than the extent of initial resection 1).

Diagnosis

Pilocytic astrocytomas range in appearance:

Large cystic component with a brightly enhancing mural nodule: 67%

non enhancing cyst wall: 21%

enhancing cyst wall: 46%

heterogeneous, mixed solid and multiple cysts and central necrosis: 16%

completely solid: 17%

Enhancement is almost invariably present (~95%). Up to 20% may demonstrate some calcification.

Haemorrhage is a rare complication.

MRI

Signal characteristics include:

T1: iso to hypointense solid component compared to adjacent brain

T2: hyperintense solid component compared to adjacent brain

Apparent diffusion coefficient (ADC) values have been shown to assist in differentiating cerebellar pilocytic astrocytomas and medulloblastomas. Previous studies have applied only ADC measurements and calculated the mean/median values.

The 25th percentile for mean (MD) yields the best results for the presurgical differentiation between pediatric cerebellar pilocytic astrocytomas and medulloblastomas. The analysis of other DTI metrics does not provide additional diagnostic value 2)

Treatment

Complete resection whenever possible

The pure neuroendoscopic technique was shown to be minimally invasive and a successful and secure method to treat cystic cerebellar tumors 3).

Complications

Acute hemorrhagic presentation in pilocytic astrocytomas (PAs) has become increasingly recognized. This type of presentation poses a clinically emergent situation in those hemorrhages arising in PAs of the cerebellum, the most frequent site, because of the limited capacity of the posterior fossa to compensate for mass effect, predisposing to rapid neurological deterioration.

Outcome

Nine percent of the children in a study underwent repeated surgery due to progressive tumor recurrence, and 15% were treated for persistent hydrocephalus 4).

The long-term functional outcome of low-grade cerebellar astrocytoma is generally favourable, in the absence of post-operative complications and brainstem involvement. No major impact of neurological deficits, cognitive functions and emotional disorders on academic achievement and independent functioning was observed 5).

The good long-term outcomes suggest that it may be appropriate to do incomplete resection rather than risk additional neurological deficit 6).

There is controversy about whether patients with tumor remaining after surgery should receive radiation therapy. It is also unclear whether only patients with incomplete resection require follow-up and for how long 7).

Complete resection

Complete resection of cerebellar astrocytoma is an important prognostic factor, indicating a more favorable prognosis than subtotal resection. This was also the conclusion of a much larger study by Villarejo et al. who reviewed 203 cases of low-grade cerebellar astrocytoma 8).

Loh et al., documented that patients with subtotal removal of cerebellar astrocytoma can have arrested tumor growth or spontaneous tumor regression during long-term follow-up. Following partial resection of pediatric cerebellar astrocytoma, they recommend that the patients be followed up a “wait and see” approach with surveillance using MRI. They found that several tumors treated with radiotherapy after surgery had malignant transformation and do not recommend adjuvant radiation treatment for children with cerebellar astrocytoma who have subtotal resection. More research is needed on the prognosis of patients with subtotal resection of cerebellar astrocytoma 9).

Pilomyxoid features and anaplasia

A subset may behave in a more aggressive fashion and clinically progress despite the use of conventional treatments. Histologic features associated with a more aggressive course include the presence of monomorphous pilomyxoid features (ie, pilomyxoid variant) and anaplasia in the form of brisk mitotic activity with or without necrosis 10).

Case series

2015

Two cases of fatal hemorrhagic cerebellar PAs: one of a child with a slowly growing stereotypical WHO Grade I PA with a 1-year period of symptomatology that preceded a rapid clinical deterioration, and another of an asymptomatic child having a PA variant, presenting with progressive obtundation following a presumed Valsalva event. These two scenarios parallel previous reports in the literature of either a setting of progressive expression of cerebellar dysfunction and transient episodes of raised intracranial pressure (ICP), or abrupt onset of features of increased ICP in a previously well child. The literature is further reviewed for a current understanding of the factors that predispose, initiate and propagate bleeding, with specific reference to the role of vascular endothelial growth factor and other angiogenic agents in the genesis and stability of the vasculature in PAs. In this context, we propose that obliterative vascular mural hyalinization with associated altered flow dynamics and microaneurysm formation was the pathogenesis of the hemorrhage in our first case. In the second case, large tumor size, increased growth rate, looseness of the background myxoid matrix, and thinness of the tumor blood vessels with calcospherite deposition predisposed to vascular leakage and bleeding concurrent with sudden increases in intravascular hydrostatic pressure. In that cerebellar PAs are common, this report underscores the importance of considering in the differential diagnosis the possibility of a spontaneous hemorrhage in a posterior fossa PA in a child presenting with a sudden neurological ictus and raised ICP 11).

1997

In a retrospective study of 73 patients operated on for cerebellar pilocytic astrocytomas, results of treatment, outcome and biological behaviour of residual tumour were analysed. Complete tumour resection proven by CT or MRI scans within 1 year after surgery was achieved only in 69% of cases. In 31% of cases the surgeon's opinion on the extent of surgical resection was not borne out by the result of postoperative neuroimaging. Progression of residual tumour or tumour recurrence appeared in 19% of patients. 1 patient showed metastatic spread along the craniospinal axis, and in 1 patient malignant degeneration appeared during follow-up. Stable residual tumour or regression of residual tumour was seen in 14% of patients. Outcome after surgical treatment, which was combined with irradiation in 10 patients (14%), was favourable in 80% and unfavourable in 20% of patients. This outcome of treatment was not influenced by a second operation for progression of residual tumour or recurrent tumour. Characteristics of patients with tumour progression after the first operation did not differ from those of the whole group. There were 17 reoperations for residual or recurrent tumour, 10 of which took place within 4 years after the initial surgical treatment. Surgery-related morbidity was 15% and mortality 4%. Irradiation to residual tumour in 8 patients was followed by complete regression in 1 patient, progression in 4 patients and no changes in 1 patient. For the remaining 2 patients the effect of irradiation on the residual tumour is unknown. Factors that determine the prognosis are discussed on the basis of this retrospective analysis and the data from the literature. It is concluded that optimal treatment for a cerebellar pilocytic astrocytoma does not consist solely in surgery with the aim of total tumour removal and careful tumour handling in order to avoid spread of tumour cells and subsequent metastases and additional radiation therapy is strictly selected cases, but also in posttreatment follow-up based on direct postoperative neuroimaging, preferably by MRI. An algorithm for postoperative follow-up management is presented 12).

Case reports

2018

A 47-year-old- lady with a posterior fossa pilocytic astrocytoma underwent surgical decompression. She developed multiple early local recurrences Along with malignant transformation of the cranial lesion she developed skeletal dissemination within a very short time frame. There were no features or family history of Neurofibromatosis type 1. She did not receive radiotherapy or chemotherapy prior to the recurrences 13).

2015

A 72-year old female with a right cerebellar pilocytic astrocytoma WHO grade I with an Isocitrate dehydrogenase 1 (IDH1) R132H mutation. The patient is recurrence-free 6 years after the initial diagnosis. Only one single case with strikingly similar clinicopathological features has been reported before. Otherwise, IDH1/2 mutations are not seen in pilocytic astrocytomas. The clinical implications of these findings are discussed 14).

1)
Wade A, Hayhurst C, Amato-Watkins A, Lammie A, Leach P. Cerebellar pilocytic astrocytoma in adults: a management paradigm for a rare tumour. Acta Neurochir (Wien). 2013 Aug;155(8):1431-5. doi: 10.1007/s00701-013-1790-1. Epub 2013 Jun 22. PubMed PMID: 23793962.
2)
Wagner MW, Narayan AK, Bosemani T, Huisman TA, Poretti A. Histogram Analysis of Diffusion Tensor Imaging Parameters in Pediatric Cerebellar Tumors. J Neuroimaging. 2015 Sep 2. doi: 10.1111/jon.12292. [Epub ahead of print] PubMed PMID: 26331360.
3)
Giannetti AV, Rocha MD, Rosseto RS, Pedrosa HA. Pure Neuroendoscopic Resection of Cystic Cerebellar Tumors. World Neurosurg. 2015 Sep;84(3):867.e7-867.e11. doi: 10.1016/j.wneu.2015.04.029. Epub 2015 Apr 23. PubMed PMID: 25913429.
4)
Due-Tønnessen BJ, Lundar T, Egge A, Scheie D. Neurosurgical treatment of low-grade cerebellar astrocytoma in children and adolescents: a single consecutive institutional series of 100 patients. J Neurosurg Pediatr. 2013 Mar;11(3):245-9. doi: 10.3171/2012.11.PEDS12265. Epub 2012 Dec 14. PubMed PMID: 23240848.
5)
Ait Khelifa-Gallois N, Laroussinie F, Puget S, Sainte-Rose C, Dellatolas G. Long-term functional outcome of patients with cerebellar pilocytic astrocytoma surgically treated in childhood. Brain Inj. 2014 Nov 10:1-8. [Epub ahead of print] PubMed PMID: 25383654.
6)
Steinbok P, Mangat JS, Kerr JM, Sargent M, Suryaningtyas W, Singhal A, Cochrane D. Neurological morbidity of surgical resection of pediatric cerebellar astrocytomas. Childs Nerv Syst. 2013 Aug;29(8):1269-75. doi: 10.1007/s00381-013-2171-z. Epub 2013 May 29. PubMed PMID: 23715810.
7)
Dirven CM, Mooij JJ, Molenaar WM. Cerebellar pilocytic astrocytoma: a treatment protocol based upon analysis of 73 cases and a review of the literature. Childs Nerv Syst. 1997;13:17–23. doi: 10.1007/s003810050033.
8)
Villarejo F, Diego JMB, Riva AG. Prognosis of cerebellar astrocytoma in children. Childs Nerv Syst. 2008;24:203–210. doi: 10.1007/s00381-007-0449-8.
9)
Loh JK, Lieu AS, Chai CY, Hwang SL, Kwan AL, Wang CJ, Howng SL. Arrested growth and spontaneous tumor regression of partially resected low-grade cerebellar astrocytomas in children. Childs Nerv Syst. 2013 Nov;29(11):2051-5. doi: 10.1007/s00381-013-2113-9. Epub 2013 May 1. PubMed PMID: 23632690; PubMed Central PMCID: PMC3825417.
10)
Rodriguez FJ, Scheithauer BW, Burger PC, Jenkins S, Giannini C. Anaplasia in pilocytic astrocytoma predicts aggressive behavior. Am J Surg Pathol. 2010;34(2):147–160.
11)
Wilson MP, Johnson ES, Hawkins C, Atkins K, Alshaya W, Pugh JA. Hemorrhagic presentations of cerebellar pilocytic astrocytomas in children resulting in death: report of 2 cases. J Neurosurg Pediatr. 2016 Apr;17(4):446-52. doi: 10.3171/2015.10.PEDS1580. Epub 2015 Dec 18. PubMed PMID: 26684764.
12)
Dirven CM, Mooij JJ, Molenaar WM. Cerebellar pilocytic astrocytoma: a treatment protocol based upon analysis of 73 cases and a review of the literature. Childs Nerv Syst. 1997 Jan;13(1):17-23. Review. PubMed PMID: 9083697.
13)
Konar SK, Nandeesh BN, Sandhya M, Chandana N, Devi BI, Bhat DI. Pilocytic astrocytoma with spontaneous malignant transformation with intracranial and skeletal dissemination: case report and review of the literature. Br J Neurosurg. 2018 May 10:1-4. doi: 10.1080/02688697.2018.1472214. [Epub ahead of print] PubMed PMID: 29745267.
14)
Behling F, Steinhilber J, Tatagiba M, Bisdas S, Schittenhelm J. IDH1 R132H mutation in a pilocytic astrocytoma: a case report. Int J Clin Exp Pathol. 2015 Sep 1;8(9):11809-13. eCollection 2015. PubMed PMID: 26617931; PubMed Central PMCID: PMC4637747.
cerebellar_pilocytic_astrocytoma.txt · Last modified: 2019/02/19 11:51 by administrador