Endocrine-Related Cancer (1998) 5 303-310 A pseudo-endocrine suprasellar tumour: review of primitive neuroectodermal tumours in adults

J Berwaerts1, J Verhelst1, G Dua2, R Abs3, I Neetens4 and C Mahler1

Departments of 1Endocrinology, 2Neurosurgery and 4Pathology, General Hospital, Middelheim, Antwerp and 3Department of Endocrinology, University Hospital of Antwerp, Antwerp, Belgium (Requests for offprints should be addressed to J Berwaerts who is now at Department of Medicine and Therapeutics, University of Aberdeen, Polwarth Building, Foresterhill, Aberdeen AB25 2ZD, UK)

Abstract

Primitive neuroectodermal tumours (PNETs) are very rare malignant brain tumours mostly affecting children. We report a 55-year-old female with a PNET, presenting with the clinical picture of a large suprasellar tumour. The patient complained of visual field defects, and had personality changes but no endocrine deficiencies or signs of hypothalamic disturbance. The diagnosis of craniopharyngioma was initially suspected on computerised tomography scan, but was not confirmed by magnetic resonance imaging, because of the presence of some perilesional oedema. The tumour was removed in two phases: first through a right frontal craniotomy to remove the suprasellar part and two weeks later through a transsphenoidal approach to remove tumour tissue from the . The pathological examination of the first resected specimen revealed compact fields of small, rounded cells and hyperplasia of the interstitial connective tissue, compatible with an adamantinomatous craniopharyngioma. Pathological investigation after the second operation demonstrated perivascular pseudo-rosettes and immunoreactivity of tumour cells for synaptophysin, favouring the diagnosis of a PNET. After the surgery the patient retained a serious cognitive deficit. Staging examinations were not performed and the intention of systemic therapy was abandoned. She died six months later, in poor general condition, of a respiratory infection. Our case of PNET is the first to be reported with a suprasellar location in an adult patient and we suggest that this diagnosis should be included in the differential diagnosis of a hypothalamic tumour in adults. The epidemiology and therapy of this unusual type of tumour is reviewed in detail.

Endocrine-Related Cancer (1998) 5 303-310

Introduction rare in children: in the literature less than fifteen cases of Tumours with a suprasellar location in the adult a PNET with suprasellar location are mentioned (Altman population are most frequently pituitary adenomas with et al. 1985, Kingston et al. 1985, Tomita et al. 1988, suprasellar extension, , and craniopharyngi- Bejjani et al. 1996). No case has ever been reported in an omas (Dietemann et al. 1992, Tindall & Barrow 1996). adult. Suprasellar brain tumours occur more frequently in child- ren than in adults, as about 15% of all brain tumours at a Case report younger age originate from the suprasellar region (Sung 1982). Also, the type of pathology is different: in a series A 55-year-old female was admitted to the hospital because of 98 patients under the age of 15 years, 44% of supra- of personality changes and frequent falls. Her food intake sellar tumours appeared to be optic , another 44% was restricted to eating chips and drinking coffee and she craniopharyngiomas and 12% germ cell tumours (Sung had been found on several occasions to be sleeping 1982). Primitive neuroectodermal tumours (PNETs) are underneath a table. Furthermore, she was constantly

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the sella turcica, suggestive of a craniopharyngioma. Magnetic resonance (MR) examination demonstrated a large tumour with enclosed , extending into the sphenoid sinus and compressing the cavernous sinus (Fig. 1). Comparable sizes of suprasellar and intrasellar parts of the tumour and a shell of perilesional oedema were noted to be less characteristic of a craniopharyngioma (Fig. 2). Eye examination indicated a normal fundus but a dimin- ished visual acuity at the right side (right side 2/10 as compared with left side 10/10). In the right visual field a central scotoma and normal peripheral borders were noticed. Hormonal determinations showed a normal thyroid function and gonadotrophin levels in accordance with a post-menopausal state. and growth hormone levels appeared to be within normal limits. The morning serum cortisol level and 24-h urinary cortisol ex- Figure 1 MR examination shows a large, partially cretion were found to be elevated to 1090 nmol/l (normal calcified tumour, which is primarily located above the sella turcica, and therefore suggests a cranio- levels 166-773 nmol/l) and 993 nmol/day (normal pharyngioma. excretion 110-440 nmol/day) respectively. These ab- normal values were still considered to be compatible with quarrelling with her neighbours. The patient acknowl- a physiological reaction to stress. Furthermore, the basal edged only dizziness and some visual disturbance. On adrenocorticotrophin level was within normal limits. physical examination, the patient appeared normally The first operation, which lasted 15 h, was performed oriented but disinhibited. The acuity of the right eye was through a right frontal craniotomy. The tumour appeared approximately found to be decreased, but other neuro- to be tender, but enclosed by a firm capsule. It contained logical deficiencies were not apparent. several cysts, filled with a crystalloid material. As large as A diffuse increase of theta waves on an electro- 6 cm in diameter, it had surrounded both internal carotid encephalogram was interpreted as indicative of ‘general arteries and had bifurcations into the anterior and middle cerebral dysfunction’. On X-ray, the sella turcica appeared cerebral arteries, the optic chiasm and both optic nerves. to be grossly enlarged and the dorsum was eroded. A brain Since complete debulking of the sella turcica was not computerised tomography (CT) scan showed a 60 mm feasible, it was decided to remove the tumour intra- large, partially calcified tumour, which was located above capsularly and to perform a second operation through a

(a) (b)

Figure 2 MR examination demonstrates a large tumour with enclosed cysts, extending into the sphenoid sinus. Comparable sizes of suprasellar and intrasellar parts of the tumour and a shell of perilesional oedema are less characteristic for cranio- pharyngioma. (a) Sagittal; (b) coronal secretion.

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Figure 3 Light microscopic examination shows a dense Figure 4 Immunohistochemical examination shows population of small, rounded cells located as pseudo- immunoreactivity for synaptophysin. Original magnification: rosettes around blood vessels (arrows). The central oval objective ×40, ocular ×10. nuclei contain chromatin with a salt-and-pepper appearance and only small nucleoli. The number of mitoses is limited. through in the sphenoid sinus. The first tentative diagnosis Immunohistochemical staining for NSE; original of craniopharyngioma was therefore considered to be magnification: objective ×40, ocular ×10. incorrect, probably due to inadequate tissue sampling. transsphenoidal approach. Light microscopic examination After the operation, the patient presented a panhypo- showed an adamantinomatous craniopharyngioma, con- pituitarism and a substitution treatment with hydro- sisting of compact fields of cells with a basaloid cortisone (30 mg/day), levothyroxine (50 µg/day) and appearance and interstitial strips of hyaline connective desmopressin acetate (2 × 15 µg intranasally) was started. tissue. Dystrophic lime appeared in the form of psamm- A brain CT scan one month after the operation oma bodies next to clefts of washed out lipids. demonstrated a hypodense and inhomogeneous aspect of The second operation, through a transsphenoidal the tissue in the right frontal lobe, with limited contrast approach, was performed 2 weeks later as the clinical uptake, indicating local oedema as a postoperative sequel. picture had not improved in the meantime. The well A foggy appearance of the sphenoid sinus and a large vascularised tumour was shown to fill up the sphenoid amount of air in the sella turcica, perimesencephalic sinus. Light microscopical examination showed a dense cisterns and in both lateral ventricles were strongly population of small, rounded cells located as pseudo- suggestive of a ventriculo-sphenoidal fistula, which could rosettes around blood vessels. Iron-laden macrophages explain the clinical finding of cerebrospinal fluid leakage and extracellular deposits of orange-brown iron pigment from the nose. In addition, the broadening of both lateral were noted. The central oval cell nuclei contained ventricles was a clear sign of postoperative hydro- chromatin with a salt-and-pepper appearance and only cephalus. A reconstruction of the skull base was therefore small nucleoli. Even though only a limited number of attempted and led to the closure of the fluid leak through mitoses were found at light microscopical examination, a a small opening in the bottom of the sella turcica. Ki-67 immunohistochemical study showed a labelling An ophthalmological examination was repeated after index of about 10%. Immunohistochemical examination the operation but appeared invalid because of lack of co- showed immunoreactivity for neurone specific enolase operation from the patient. Neurolinguistic evaluation (NSE), synaptophysin and chromogranin, but not for pointed first to a postoperative confusional state and, later, neurofilament protein (NFP) or glial fibrillary acid protein as deficits in memory and frontal functions became more (GFAP), and for none of the pituitary hormones (Figs 3 apparent, to a demential syndrome. This was an additional and 4). All these findings were suggestive of a small-cell, reason not to perform the usual staging examinations and malignant tumour with neuroendocrine differentiation. to postpone radiotherapy until a better general state would The preferential diagnosis was a primitive neuroecto- have been attained. Moreover, in close consultation with dermal tumour (PNET) of cerebral origin, with break- her family, the patient was admitted to a long-term care

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Downloaded from Bioscientifica.com at 10/01/2021 04:23:24AM via free access J Berwaerts et al.: Suprasellar PNET in an adult facility. There she died of an intercurrent respiratory that of (Peterson & Walker 1995). The infection about six months after the operations. overall incidence of PNETs is estimated at 0.06/100 000 persons/year (Grant et al. 1988). Eighty percent of all Discussion patients with either a PNET or are less than 15 years of age (Albright et al. 1995, Peterson & Walker 1995). All in all, only 35 adult patients with a Definition PNET have been reported in the literature (Bellis et al. The definition of PNET has been a point of debate for a 1983, Gaffney et al. 1985, Kuratsu et al. 1986, long time (Tomita et al. 1988, Pigott et al. 1990, Robles et Shuangshoti 1986, Grant et al. 1988, Louis & Hochberg al. 1992). Hart and Earle (1973) introduced the term 1990, Pigott et al. 1990, Louwaege et al. 1993, Miyazawa PNET to describe largely undifferentiated et al. 1994, Selassie et al. 1994, Papierz et al. 1995, occurring in the cerebrum of young individuals and Peterson & Walker 1995, Pickuth & Leutloff 1996). consisting of more than 90% of undifferentiated cells Whereas PNETs are most often located in frontal and resembling the germinal matrix cells of the embryonic parietal lobes (Altman et al. 1985, Gaffney et al. 1985, neural tube. Up to 10% of the tumour cells may exhibit Grant et al. 1988, Tomita et al. 1988, Robles et al. 1992, evidence of differentiation along either neuronal or glial Albright et al. 1995, Dirks et al. 1996), about 10% of the lines. Nevertheless, Rubinstein (1985) argued in favour of tumours are found around the third ventricle (Grant et al. a classification of embryonic 1988). PNETs with a suprasellar location have been (CNS) tumours according to the stages of cytogenesis: reported previously only in children, either occurring in (neural tube cells), association with a , as part of a so-called (primitive neurones), polar spongioblastoma (primitive ‘trilateral retinoblastoma’ (in about 10 patients) (Bejjani et ) and ependymoblastoma (ependymal cells). al. 1996), or isolated (in 4 patients) (Altman et al. 1985, Even though, traditionally, a distinction is made between Kingston et al. 1985, Tomita et al. 1988). These neuroblastoma (cerebrum), medulloblastoma (cerebell- suprasellar tumours generally extend into the cerebral um), pineoblastoma () and retinoblastoma hemispheres and not into the sella turcica as in our case. (retina), Rorke et al. (1985) advocated a more general application of the term PNET irrespective of tumour Aetiology localisation. Embryonic CNS tumours, consisting of less In the World Health Organisation classification of CNS than 90% of undifferentiated cells and arising above the neoplasms, PNETs are grouped as embryonic tumours tentorium (i.e. not in the cerebellum) in patients of all ages, because of the resemblance of tumour cells to primitive, may thus be classified as PNET (Gaffney et al. 1985). undifferentiated neuroepithelial precursor cells (Tatter et PNETs resemble classical medulloblastomas in al. 1996). are also regarded as a sort of several respects, such as histological appearance, embryonic CNS tumour, closely related to PNETs (Bejjani occurrence in children and malignant behaviour (Gaffney et al. 1996). In the sporadic as well as hereditary cases of et al. 1985). Apart from the localisation respectively this tumour, a mutation is assumed in both alleles of the above and below the tentorium, differences between these retinoblastoma tumour suppressor gene, located on two tumours consist of the most prevalent type of cell chromosome 13q14. The deficient gene product acts as a differentiation (astrocytic in PNETs vs neuronal in negative regulator of the transcription factors that are medulloblastomas), the capacity to seed along the responsible for the entry of retinal precursor cells into the cerebrospinal axis (15% vs 50%) (Albright et al. 1995), DNA synthesis phase of the cell cycle (Tatter et al. 1996). and the response to surgical and (25% vs For PNETs a similar ‘PNET tumour suppressor gene’ may 50% 5-year survival rate) (Gaffney et al. 1985). In be assumed, the mutation or deletion of which would addition, there are arguments in favour of different genetic allow the malignant transformation of neuroepithelial effects involved in the aetiology of the two tumour types cells at the different stages of differentiation: neural tube (e.g. chromosome arm 17p deletion exclusively in cells, neuroblasts, spongioblasts or ependymal cells medulloblastomas and not in PNETs) (Burnett et al. 1997). (Robles et al. 1992). However, up to now no single gene has been mapped for PNETs and translocations or Epidemiology additions can be demonstrated, but no consistent deletions The information on the incidence of PNETs is usually have been found which would support the hypothesis of reported in conjunction with that of medulloblastomas. In tumour suppressor gene loss (Pigott et al. 1990). children the occurrence of PNETs is estimated at 3% of all brain tumours, as opposed to 25% for medulloblastomas (Albright et al. 1995, Dirks et al. 1996). In adult patients the occurrence of PNETs is believed to be less than 10%

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Pathology Papierz et al. 1995). Another purpose of immuno- Macroscopically, PNETs are large (7 cm in diameter on histochemistry is to aid the distinction from other types of average) soft, grey-white masses, with prominent cystic CNS tumours: undifferentiated secondary tumours, poorly and variable haemorrhagic components. The tumours are differentiated small-cell tumours native to the CNS (e.g. sharply delineated, enclosed by a pseudo-capsule even germinomas, lymphomas or carcinomas) though they appear microscopically invasive (Gaffney et (Gaffney et al. 1985, Grant et al. 1988, Pigott et al. 1990), al. 1985, Robles et al. 1992). or a as in our case. Furthermore, the cell Light microscopically, the tumours consist proliferation index derived from a Ki-67 immuno- predominantly of small, undifferentiated cells with ovoid histochemical study (range 1-13% for PNETs) may hyperchromatic nuclei and a high nucleo-cytoplasmic provide additional prognostic information (Bodey et al. ratio against a fine fibrillary background. Mitoses are 1997). In our case, the first biopsy had already shown a often abundantly present, whereas areas of necrosis, hyperplasia of the connective tissue stroma, but only the haemorrhage and calcification can occur to a variable second biopsy demonstrated perivascular pseudo-rosettes degree within the tumour (Gaffney et al. 1985, Grant et al. and an immunoreactivity of tumour cells for synapto- 1988, Robles et al. 1992). In practice, the proportion of physin, but not for NFP or GFAP. These findings may differentiated cells can range from 0% to more than 20% indicate some degree of neuronal and ependymal of all tumour cells (Gaffney et al. 1985, Pigott et al. 1990). differentiation within the PNET. PNETs lacking any evidence of differentiation are actually very rare and may be considered as a special subtype Symptoms (NOS=not otherwise specified) (Miyazawa et al. 1994). Patients of all ages may present with a clinical picture On the other hand, if differentiated tumour cells are consisting of symptoms and signs of raised intracranial present, often more than one type of differentiation is pressure. In children, these appear as increasing list- represented within a single tumour (Gaffney et al. 1985). lessness, nausea, , while in adults , On the basis of light microscopy, tumour cells show visual disturbances and possibly epilepsy are more evidence of astrocytic, neuronal and oligodendrocytic prominent. The history of the illness is usually relatively differentiation in, respectively, 61%, 50% and 39% of the short, between one week and three months (Pigott et al. cases, whereas ependymal differentiation is quite 1990). Apart from changes in personality, our patient also uncommon (6%) (Gaffney et al. 1985). When cells with presented with visual field loss due to the suprasellar neuronal differentiation predominate among differenti- location of the tumour. Endocrine deficiencies were not ated tumour cells and the PNET would thus histologically clinically or biochemically apparent. be classified as a neuroblastoma, a desmoplastic variant can be distinguished if concomitant hyperplasia of the connective tissue stroma is present (Robles et al. 1992, Radiology Tatter et al. 1996). Furthermore, perivascular pseudo- Radiography of the skull, when performed in patients with rosettes are considered to be a hallmark of ependymal PNET, can show calvarial asymmetry or bone erosions differentiation (Gaffney et al. 1985). (Robles et al. 1992). On CT scan a PNET appears as a By means of electron microscopy, characteristics of heterogeneous, iso- to hyperdense mass, containing areas neuronal differentiation (neurites, neurotubules, dense- of calcification and cysts, with little surrounding oedema core vesicles, ...) can be demonstrated in a majority of (Altman et al. 1985, Pigott et al. 1990, Robles et al. 1992, PNETs (70-87%) (Pigott et al. 1990, Papierz et al. 1995). Pickuth & Leutloff 1996). Although PNET can Immunohistochemistry also can indicate differentiation in occasionally present as an intracerebral haemorrhage areas of apparent morphological uniformity (Papierz et al. (Louwaege et al. 1993), bleeding within a tumour is not as 1995). Evidence of neuronal differentiation, such as readily distinguished by CT scan (Figueroa et al. 1989, immunoreactivity for synaptophysin or NFP, can be Pickuth & Leutloff 1996). Contrast enhancement is shown in a highly variable proportion of PNETs: 0-12% heterogeneous and generally demonstrates well-defined up to 91% (Grant et al. 1988, Pigott et al. 1990, Papierz et margins. Mass effect and/or can easily be al. 1995). Immunoreactivity for GFAP can be recognised on CT scan (Altman et al. 1985, Pigott et al. demonstrated in 87-92% of the tumours, indicating 1990, Robles et al. 1992, Pickuth & Leutloff 1996). At entrapped stromal astrocytes or astrocytic differentiation MR examination, PNETs exhibit characteristics similar to of tumour cells (Gaffney et al. 1985, Grant et al. 1988, those on CT scan, except of course for hypointensivity on Pigott et al. 1990, Papierz et al. 1995). On the other hand, T1- and hyperintensivity on T2-weighted images a particular differentiation for NSE or S-100 protein (Figueroa et al. 1989, Robles et al. 1992, Louwaege et al. cannot easily be gathered from immunoreactivity, because 1993, Pickuth & Leutloff 1996). Whereas MR imaging in of lack of specificity of these markers (Grant et al. 1988, general is thought to provide a better anatomical definition

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Downloaded from Bioscientifica.com at 10/01/2021 04:23:24AM via free access J Berwaerts et al.: Suprasellar PNET in an adult of tumour margins, the haemorrhagic component of spinal cord amount to 30-35 Gy and 5 weeks (Gaffney et PNETs is most apparent on T1-weighted and the lack of al. 1985). For the use of chemotherapy in the treatment of significant surrounding oedema on T2-weighted images PNET three indications may be considered: (1) palliative (Figueroa et al. 1989, Robles et al. 1992, Pickuth & treatment, (2) holding measure to delay the need for Leutloff 1996). In addition, MR imaging allows a better radiotherapy in children under the age of three years and detection of intracerebral, subarachnoidal and meningeal (3) adjuvant chemotherapy (Gaze et al. 1994). With the metastases. From the latter stems the need for an MR combination of nitrosurea and vincristine, some individual examination of the spinal cord in tumour staging (Robles cases of unusually long survival have been reported (7-11 et al. 1992, Pickuth & Leutloff 1996). Initial staging of years) (Gaffney et al. 1985) whereas, in general, no PNETs should furthermore include an examination of the significantly longer survival arose with adjuvant cerebrospinal fluid, but no routine systemic imaging in chemotherapy compared with treatment regimens without asymptomatic patients (chest X-ray, echography of the adjuvant chemotherapy (Tomita et al. 1988, Dirks et al. abdomen, whole body bone scan, ...), because of the negli- 1996). The combination of vincristine, cyclophos- gible prevalence of extraneural metastases at the time of phamide, carboplatin and etoposide (e.g. according to the diagnosis (Peterson & Walker 1995). In our case, a adapted International Society of Paediatric and craniopharyngioma was suspected on CT scan, because of UK Children’s Cancer Study Group III (SIOP-UKCCSG the location of a heterogeneous mass mainly above and III) protocol)) has been shown to involve a high response within the sella turcica. At MR examination, the presence rate (85%), a long time to relapse and relatively low of some surrounding oedema was noted as an argument toxicity (Gaze et al. 1994). As a result, the palliative against craniopharyngioma (Hald et al. 1995), but this was setting is considered to be a good indication for this type not immediately suggestive of a PNET. In the literature of chemotherapy (Gaze et al. 1994). In our case, no only one case has been reported of a suprasellar PNET that systemic therapy was applied in consultation with the was suspected preoperatively by means of imaging patient's family, given the state of dementia after tumour techniques (Altman et al. 1985). resection.

Therapy Prognosis Many authors argue in favour of a multimodal radical Survival rates after tumour excision and neuraxis treatment, which allows for a systemic therapy from the irradiation (and in some cases adjuvant chemotherapy) are outset (Bellis et al. 1983, Gaffney et al. 1985, Tomita et al. reported to be as high as 37-64% survival at 1 year, 12- 1988, Albright et al. 1995, Peterson & Walker 1995, Dirks 50% survival at 2 years and 6-33% survival at 5 years. The et al. 1996). An optimal treatment regimen is not yet average survival period has been estimated at 10-37 established because of a lack of clinical trials due to the months (Altman et al. 1985, Gaffney et al. 1985, Grant et very low incidence of PNET (Peterson & Walker 1995). al. 1988, Tomita et al. 1988, Pigott et al. 1990, Albright et Total tumour excision on its own has been shown to al. 1995, Dirks et al. 1996, Pickuth & Leutloff 1996). enhance survival in two reports (Gaffney et al. 1985, Dirks PNETs are generally acknowledged to involve a poorer et al. 1996), or to reduce local recurrence without prognosis compared with medulloblastomas (Gaffney et influencing survival in another report (Tomita et al. 1988). al. 1985, Tomita et al. 1988, Gaze et al. 1994, Albright et A major argument in favour of early systemic therapy is al. 1995, Peterson & Walker 1995, Dirks et al. 1996). that (intraneural) metastases, which are difficult to Figures for 5-year survival rate after multimodal treatment demonstrate at the time of diagnosis (in 5-15% of differ by as much as 25% and 50% (Gaffney et al. 1985). patients), are abundantly present in the end stage of the PNETs are claimed to entail a poorer prognosis in adults disease (in 45-100%) (Gaffney et al. 1985, Tomita et al. as compared with children. In one report the 5-year 1988, Albright et al. 1995, Dirks et al. 1996), whereas survival rate for adults is estimated at 14% (Gaffney et al. local recurrence after tumour excision can only be found 1985), whereas in another report considering only children in 45-50% of end stage patients (Gaffney et al. 1985, a 5-year survival rate of 33% is mentioned (Albright et al. Tomita et al. 1988). Although PNETs are generally 1995). Conversely, age less than three years is also considered to be less radiosensitive than medulloblas- associated with a poorer prognosis, mainly because of the tomas, tumour excision is often combined with neuraxis contraindication for radiotherapy (Albright et al. 1995, irradiation, because of some separate reports of unusual Dirks et al. 1996). Apart from age, the proportion of long survival and low morbidity (Gaffney et al. 1985). A differentiated cells within a PNET is another useful dose and duration of irradiation to the primary tumour site prognostic factor (longer survival if more than 10% of of 50-55 Gy and 8 weeks respectively have been tumour cells are differentiated) (Gaffney et al. 1985). recommended, whereas these figures for irradiation to the Survival does not appear to be dependent on the type of

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Downloaded from Bioscientifica.com at 10/01/2021 04:23:24AM via free access Endocrine-Related Cancer (1998) 5 303-310 differentiation (neuronal or glial) (Tomita et al. 1988, Hald JK, Eldevik OP & Skalpe IO 1995 Craniopharyngioma Albright et al. 1995, Dirks et al. 1996). A last important identification by CT and MR imaging at 1.5 T. Acta indicator of poor prognosis is the presence of metastases Radiologica 36 142-147. at the initial staging (Albright et al. 1995), which can Hart MN & Earle KM 1973 Primitive neuroectodermal tumours actually be found in 5-15% of the patients as mentioned of the brain in children. Cancer 32 890-897. above (Gaffney et al. 1985, Tomita et al. 1988, Albright et Kingston JE, Plowman PN & Hungerford JL 1985 Ectopic al. 1995). intracranial retinoblastoma in childhood. British Journal of Ophthalmology 69 742-748. 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