10.1007/s00381-007-0323-8

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Child's Nervous System

=A9 Springer-Verlag 2007

Original Paper

Posterior fossa tumors in = children: how=20 long does it take to establish the diagnosis?

Lutz D=F6rner¹, Michael J. Fritsch²,=20 Andreas M. Stark¹ and=20 Hubertus Maximilian Mehdorn¹

(1)	Department of Neurosurgery,=20 Universit=E4tsklinikum Schleswig-Holstein, Campus Kiel, = Schittenhelmstr. 10,=20 24105 Kiel, Germany

(2)	Department of Neurosurgery,=20 Universit=E4tsklinikum Greifswald, Moritz-Arndt Universit=E4t = Greifswald,=20 Greifswald, Germany

Lutz D=F6rner=
Email:=20 doernerl@nch.uni-kiel.de

Received:=20 15 December 2006 Revised:=20 29 December 2006 Published online: = 12 April 2007

Abstract

Objective The aim of this study is to = evaluate,=20 for our patient population, the time interval from the first = chart-documented=20 symptom to the radiological diagnosis in children and infants with = posterior=20 fossa tumors.

Materials and=20 methods We retrospectively analyzed 50 consecutive = children=20 (36 men, 14 women) with posterior fossa tumor treated at our department = between=20 January 1999 and December 2003. The mean age at time of diagnosis was=20 98 months (6 months=9616 years). The mean follow up was=20 27 months (6=9661 months). The diagnoses included astrocytoma=20 (n =3D 17), medulloblastoma = (n =3D 15), ependymoma (n =3D 6), = and=20 other tumors (n =3D 12).

Results The mean time interval = between onset of=20 symptoms and radiographic diagnosis was 142 days = (5=96535 days), the=20 median was 59 days. The mean time for Grade I and II tumors was=20 238 days (n =3D 19) and for tumors Grade III and = IV 117 days=20 (n =3D 31). The most common presenting symptoms were = headache, nausea,=20 vomiting, ataxia, and oculomotor deficits. Approximately half of the = patients=20 were initially diagnosed and treated for other diseases = (gastrointestinal=20 infection, appendicitis, psychological behavioral problems, cervical = spine=20 strains, different ophthalmologic entities). Specialists = (ophthalmologists,=20 orthopedics) tended to diagnose and treat their specific diagnoses = (e.g.,=20 strabism, torticollis). Parents play a significant role in the process = of=20 establishing the correct diagnosis.

Conclusion We conclude that further = information=20 and education regarding symptomatology and diagnosis of posterior fossa = tumors=20 in children is necessary. Communication has to be improved between = parents and=20 referring physicians of all specialties and neurosurgeons. =

Keywords Posterior=20 fossa tumor - Patient management - Diagnostic=20 delay - Diagnostic interval - Pediatric brain tumor =

Introduction

Intracranial tumors are the most common solid neoplasm in = children=20 occurring in 2.4 to 4 per 100,000 [1=965].=20 Posterior fossa tumors present approximately 60=9670% of all brain = tumors in=20 children. Clinical symptoms include headaches, nausea and vomiting, = reduced=20 level of consciousness, ataxia, or cranial nerve deficits. Pediatricians = and=20 general practitioners have significant responsibility in the process of=20 identification of clinical signs that could point toward a posterior = fossa=20 tumor. Among our patients, there were several with long lasting symptoms = before=20 the diagnosis was confirmed. Therefore, we wanted to evaluate the = diagnostic=20 process in our patients retrospectively.

Issues that we wanted to present and discuss in this paper = are: How=20 long does it take in a highly developed medical system to establish the=20 diagnosis? What are the main presenting symptoms in our patient = population? What=20 are the most misleading symptoms? What are the main referring = specialties? What=20 are the differences in regard of diagnostic workup and referral pattern = among=20 the specialties? What are the differences regarding the diagnostic = interval=20 between low-grade and high-grade tumors? What is the parent=92s role? =

Materials and methods

We performed a retrospective chart review and included 50 = children=20 with posterior fossa tumors treated in our department between January = 1999 and=20 December 2003.

Patients=92 data are summarized in Tables 1=20 and 2.=20

Table 1 = Epidemiological=20 data concerning our patient group of pediatric patients with posterior = fossa=20 tumors seen in our hospital (1999=962003)

Patient=92s data
Number of patients	50
Gender (male/female)	36/14
Mean age (months)	98 (6 months=9616 years)
Mean follow-up (months)	27 = (6=9661 months)

Table 2 = Histopathology of=20 the posterior fossa tumors in pediatric patients seen in our hospital=20 (1999=962003)

Pathology	Number of patients
Astrocytoma	17
Medulloblastoma	15
Ependymoma	6
Other	12
	50

Tumor localization was cerebellar hemispheres = (n =3D 23);=20 fourth ventricle (n =3D 11); midbrain, pons or = medulla oblongata=20 (n =3D 12), and cerebellopontine angle = (n =3D 4).

We evaluated the patient charts for the following = information: first=20 chart-documented symptom (including outpatient charts and letters from = referring=20 physicians), time interval from first symptom to established = radiological=20 diagnosis, presenting symptoms, specialties involved in diagnostic = evaluation,=20 specialty referring the patient to our department, misleading diagnoses = and=20 treatment plans before established diagnosis of a posterior fossa tumor, = and=20 histological diagnosis.

Results

Our study group is of similar epidemiological consistency = as=20 comparable groups in the literature: a mean age of 98 months, a = dominance=20 of male patients with a ratio of 36:14 to female patients, and the=20 histopathological differentiation is similar to previously studied = patient=20 collectives [3,=20 4].=20

The mean time interval from the first chart-documented = symptom to=20 the day of radiological diagnosis was 142 days, the median was=20 59 days.

The mean time interval for tumors Grade I and II was = 238 days=20 (n =3D 19 patients) and for tumors Grade III and IV = 117 days=20 (n =3D 31 patients).

Seventeen patients were diagnosed within the first = month; seven=20 patients were diagnosed within 2 months, six patients within = 3 months,=20 13 patients within 4=969 months, and in 7 patients, diagnosis took = longer=20 than 9 months (Fig. 1).=20

Fig. 1 Number = of patients=20 diagnosed within a certain time period (months) after onset of symptoms =

Most frequent symptoms were headache, nausea and vomiting, = reduced=20 level of consciousness, ataxia, cranial nerve deficits, and torticollis. = In 78%=20 of the patients (39/50), hydrocephalus was diagnosed.

Before being transferred to our department, the patients = were=20 seen, on average, by two physicians of different specialties. The = children were=20 mainly transferred to our department from pediatric hospitals, = pediatricians in=20 practice, general practitioners, neurologists, ophthalmologists, and = orthopedic=20 surgeons (Table 3).=20

Table 3 = Specialties being=20 involved in the diagnostic regimen before the admission to our hospital = with the=20 diagnosis of a posterior fossa tumor

Referring specialty	Number of patients
Pediatrics (hospital)	23
Pediatrics (praxis)	9
ENT	2
General practitioner	2
Ophthalmologist	3
Neurologist	4
Orthopedics	2
Others	5
	50

Case examples

Case 1

A 6-year-old girl developed ataxia shortly after starting = her first=20 year in school. The school physician diagnosed a =93developmental = delay=94 and=20 prescribed physical therapy. Symptoms improved, and no further workup = was done=20 at this point.

Two months later, ataxia got worse and was interpreted as=20 psychological in nature, caused by the school environment and stress. = The=20 following 3 weeks, dysarthria developed. No further workup was = done. The=20 patient was admitted by the parents through the pediatric emergency room = with=20 nausea, vomiting, and lethargy. Magnetic resonance imaging (MRI) = revealed a=20 tumor of the pons. The diagnostic time interval was 3 months and=20 3 weeks. Histology revealed an anaplastic astrocytoma (WHO III). =

Case 2

The mother of a 5-month-old girl noticed a torsion and = tilting of=20 the head to the right. The pediatrician prescribed chiropractic therapy. = The=20 following weeks, symptoms improved. The torticollis reappeared = 7 months=20 after the first symptoms. The parents presented the girl to the = pediatric=20 emergency room after the baby started vomiting and appeared less = conscious. MRI=20 revealed a tumor of the fourth ventricle with infiltration of the brain = stem and=20 the cerebellopontine angle on the left side. The diagnostic time = interval was=20 8 months and 10 days. Histology was ependymoma. =

Discussion

How long does it take?

In our patient series, it took 142 days, on average, = to=20 establish the diagnosis of a posterior fossa tumor.

What are the possible explanations?

Despite the fact that posterior fossa tumors are among the = most=20 significant solid tumors in childhood age, they are still relatively = rare and=20 tend to be misdiagnosed for more common differential diagnoses. Patients = in our=20 study have been diagnosed and treated for gastrointestinal infection=20 (n=3D9), appendicitis (n=3D1), psychological behavioral = problems=20 (n=3D4), cervical spine strains (n=3D9), and different = ophthalmologic=20 entities (n=3D5).

The awareness of childhood brain tumors within the = population is=20 low; the knowledge among physicians is limited. The presentation is = often=20 unspecific, especially in infants and younger children [6].=20 Posterior fossa tumors do not cause seizures as occasionally seen with=20 supratentorial tumors.

What are the most misinterpreted symptoms?

Torticollis has a wide range of differential = diagnoses:=20 spasmodic torticollis, hemorrhage into the sternocleidomastoid muscle = with=20 subsequent contracture, cervical infection/adenitis, syringomyelia, and=20 cerebellar tumor [7=969].=20 Torticollis may also present as a secondary correction to reduce = diplopia.

A number of children (n=3D8) already had a = torticollis for some=20 time until signs of intracranial pressure occurred and finally the = correct=20 diagnosis was made. Retrospectively, they often had mild neurological = deficits=20 from the beginning but were missed by the pediatricians and orthopedics = probably=20 because of their focused view on orthopedic causes of the symptom.

Diplopia and other oculomotor abnormalities were = not always=20 thoroughly evaluated by obtaining an imaging study of the brain. Some = patients=20 were treated for strabism conservatively for several weeks.

Who refers early and who refers late?

Most patients were seen and referred by pediatricians and=20 neuropediatricians either in a hospital setting or in private praxis and = by=20 general practitioners.

There were delays in establishing the diagnosis, on the = one hand,=20 when pediatricians did not interpret the symptoms correctly and, on the = other=20 hand, when specialties like orthopedics and ophthalmologists focused on = their=20 specific group of differential diagnoses.

Pediatricians and general physicians as the group of = physicians=20 seeing pediatric patients on a regular base and following their physical = development should be sensitized for the problems of the posterior fossa = tumors.=20

What are the differences regarding the diagnostic = interval=20 between low-grade and high-grade tumors?

The differences seen between benign and malignant tumors = were=20 expected and are easily understandable. Malignant tumors have a faster = growth=20 pattern. This way, they cause a faster developing hydrocephalus or=20 space-occupying effect, which the brain is not able to compensate = resulting in=20 early signs of intracranial pressure or neurological symptoms. There = were some=20 cases of malignant tumors with a longer time interval. We assume they = were=20 primarily of a lower grade followed by a secondary malignant = transformation.=20

Is a secondary hydrocephalus predictive for an = early=20 diagnosis of the tumors?

The majority of tumors have caused a secondary = hydrocephalus at the=20 time of diagnosis. Many tumors with a long history of symptoms finally = resulted=20 in a hydrocephalus. The symptoms of a raised intracranial pressure led = faster to=20 the diagnosis, but were not predictive for a short symptomatic period in = general.

What is the parent=92s role?

Overlooking our data, we had several cases in which only = the=20 parent=92s persistence led finally to the correct diagnosis of a = posterior fossa=20 tumor. Thus confirming the belief that parents are the best in = interpreting=20 their children=92s health.

Conclusion

Physicians seeing pediatric patients need to be further = sensitized=20 for the symptoms of posterior fossa tumors. The indication for cranial=20 radiological diagnostics especially MRI should be seen wider as in the = adult=20 patient, even for rather unspecific symptomatology.

Particularly, neurosurgeons and neuropediatricians should = more=20 actively teach about posterior fossa tumors and their symptoms, = diagnostics,=20 therapy, and outcome.

We need to pay attention to the parents who present their = children=20 with specific complaints and symptoms as well as parent who state that = there is=20 just something wrong with their child.

References

1.	Packer RJ (1999) Brain tumors in children. = Arch Neurol=20 56:421=96425

2.	Albright AL (1993) Pediatric brain tumors. CA = Cancer J=20 Clin 43:272=96288

3.	Mazzucco A, von der Weid N, Godoy N (1996) = Brain=20 tumors of the posterior fossa in childhood. An overview of the = patients of=20 the medical university hospital=92s pediatric department Berne in = the years=20 1990=961994. Schweiz Rundsch Med Prax 85:1001=961004

4.	Nomura S, Nishizaki T, Yamashita K, Ito H = (1998)=20 Pediatric brain tumors in a 10-year period from 1986 to 1995 in = Yamagushi=20 prefecture: epidemiology and comparison with adult tumors. Pediatr = Neurosurg 28:130=96134

5.	Pollack IF (1999) Pediatric brain tumors. = Semin Surg=20 Oncol 16:73=9690

6.	O=92Brien DF, Caird J, Kennedy M, Roberts GA, = Marks JC,=20 Allcutt D (2001) Posterior fossa tumors in childhood: evaluation = of=20 presenting clinical features. Ir Med J 94:52=9653

7.	Ballock RT, Song KM (1996) The prevalence of=20 nonmuscular causes of torticollis in children. J Pediatr Orthop=20 16:500=96504

8.	Turgut M, Akalan N, Bertan V, Erbengi A, = Erymilmaz M=20 (1995) Acquired torticollis as the only presenting symptom in = children=20 with posterior fossa tumors. Childs Nerv Syst 11:86=9688

9.	Gupta AK, Roy DR, Conlan ES, Crawford AH = (1996)=20 Torticollis secondary to posterior fossa tumors. J Pediatr Orthop=20 16:505=96507

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