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CASE REPORT Table of Contents   
Year : 2009  |  Volume : 52  |  Issue : 3  |  Page : 383-385
Frontal recurrence of medulloblastoma five years after excision and craniospinal irradiation


National Neurosurgical Referral Centre, National Academy of Medical Sciences, Bir Hospital, Kathmandu, Nepal

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Date of Web Publication12-Aug-2009
 

   Abstract 

Medulloblastomas were originally classified under gliomas of the cerebellum until Bailey and Cushing in 1925 named these tumors as medulloblastoma. At present these tumors are classified under primitive neuroectodermal tumor. Surgical excision followed by craniospinal irradiation is the treatment of choice. A 13-year-old-girl operated for posterior fossa medulloblastoma 5 years ago presented with history of headache and vomiting on and off for 4 days in late August 2008. The MRI showed left frontal tumor which on excision was reported as medulloblastoma. Even after optimal treatment reports of recurrence abound in literature. The most common location is in the posterior fossa, followed by spinal, supratentorial, and uncommonly, systemic metastases. We conclude that medulloblastomas are highly aggressive tumor with high local recurrences if the initial excision is incomplete and that recurrence in the supratentorial area although uncommon is still a possibility. This mandates regular follow up of these children till adulthood to catch early recurrences and metastatic disease.

Keywords: Glioma, medulloblastoma, radiotherapy

How to cite this article:
Roka Y B, Bista P, Sharma G R, Adhikari D, Kumar P. Frontal recurrence of medulloblastoma five years after excision and craniospinal irradiation. Indian J Pathol Microbiol 2009;52:383-5

How to cite this URL:
Roka Y B, Bista P, Sharma G R, Adhikari D, Kumar P. Frontal recurrence of medulloblastoma five years after excision and craniospinal irradiation. Indian J Pathol Microbiol [serial online] 2009 [cited 2019 Oct 14];52:383-5. Available from: http://www.ijpmonline.org/text.asp?2009/52/3/383/55001



   Introduction Top


Medulloblastomas were originally classified under gliomas of the cerebellum until Bailey and Cushing in 1925 named these tumors as medulloblastoma and presently they are classified under primitive neuroectodermal tumor (PNET). [1] These tumors account for approximately 7-8% of all intracranial tumors and 30% of pediatric brain tumors. They are more common in males and two thirds in patients aged less than 9 years. Surgical excision followed by craniospinal irradiation is the treatment of choice. The extent of resection, presence or absence of metastases and the age, less than or more than 3 years influence the prognosis of these children. The 5-year progression-free survival rate is 70-80% for patients at low risk and 60-70% for patients at high risk. Medulloblastomas are very aggressive (WHO Grade IV) tumors and recurrence is common even after optimal treatment. Most of the recurrences occur within 2 years and mostly in the posterior fossa, although supra-tentorial and systemic metastases to bone and lymph nodes have also been reported in literature. Post radiation neo-tumors with various grades and locations have also been reported in literature. Reports on supra-tentorial metastases of medulloblastoma are infrequent and we report such a case of recurrence in the left frontal lobe 5 years after near total excision and craniospinal irradiation for a primary posterior fossa medulloblastoma.


   Case Report Top


A 13-year-old girl presented with history of headache and vomiting on and off for 4 days in late August 2008. There was no other significant history at present. She was operated in 2003 with ventriculoperitoneal shunt, followed by total excision of posterior fossa tumor [Figure 1]. The histopathology showed medulloblastoma with neuroblastic differentiation. The Magnetic Resonance Imaging (MRI) of the craniospinal axis did not show any deposits and she underwent craniospinal irradiation with dose of 50 Gy to posterior fossa and 30 Gy to the spinal axis. She was followed up with yearly MRI of the whole craniospinal axis to look for any deposits/recurrences.

On examination during this admission her higher mental function, cranial nerves, and motor examination were all normal. Computed Tomography and MRI showed a large, cystic, heterogeneously enhancing mass in the left frontal area with midline shift of less than 5 mm [Figure 2]. She underwent left frontal craniotomy and excision of the tumor with the histopathology showing features of medulloblastoma [Figure 3]. Postoperative imaging showed some residual blood in the cavity but no tumor locally or in the spine.


   Discussion Top


Medulloblastomas are WHO Grade IV tumors which are common in the pediatric age group less than 9 years, common in males and mainly in the posterior fossa. The prognosis depends on the excision of tumor, age of the patient, and presence or absence of metastases [Table 1]. [2]

Collin's law states that, if a tumor has not recurred in a period of time equal to age of patient at diagnosis plus 9 months, then that patient can be considered to be cured. [3] This law was first hypothesized for Wilms tumor and now has been extended to cover many pediatric tumors. It has been found to be a useful rule for prediction as confirmed by Paulino who studied 424 pediatric tumor cases, 59 of them medulloblastoma and concluded that Collin's law is a useful and simple way of predicting risk period for relapse in the tumor types they had studied. [3] Some studies by others seem to refute this conclusion. [4],[5]

Even after optimal treatment reports of recurrence abound in literature. The most common location is in the posterior fossa, followed by spinal, supratentorial, and uncommonly, systemic metastases. Few papers have described the sub frontal location as the common site of recurrence. [6],[7] In the series by Sun et al., [7] the most common site of recurrence out of 27 cases they reported, was in the posterior fossa (10 cases), followed by the sub frontal lobe (7 cases), spine (6 cases), and other areas (4 cases). [6] The possible explanation hypothesized are a geographic miss (cases in which the radiation source did not fully cover the injured area) in cribriform plate due to targeting deviation and the migration of tumor cells to the cribriform plate caused by the "face down position" of the patient during surgery. [7] In the series by Garcia et al . [8] of 5 cases of recurrence, they found intraventricular dissemination of the tumor in all the cases. [8] Post radiation induced secondary tumors in various locations have also been described. [9],[10] Majority of these are glioblastomas.

We conclude that medulloblastoma is a highly aggressive tumor with high local recurrences if the initial excision is incomplete and that recurrence in the supratentorial area although uncommon is still a possibility. This mandates regular follow-up of these children till adulthood to catch early recurrences and metastatic disease.

 
   References Top

1.Bailey P, Cushing H. Medulloblastoma Cerebelli: A common type of midcerebellar glioma of childhood. Arch Neurol Psychiatry 1925;14:192- 224.  Back to cited text no. 1    
2.Packer RJ, Cogen P, Vezina G, Rorke LB. Medulloblastoma: clinical and biologic aspects. Neuro Oncol 1999;1:232-50.  Back to cited text no. 2    
3.Paulino AC. Collin's law revisited: c0 an we reliably predict the time to recurrence in common pediatric tumors? Pediatr Hematol Oncol 2006;23:81-6.  Back to cited text no. 3    
4.Sure U, Berghorn WJ, Bertalanffy H. Collin's law. Prediction of recurrence or cure in childhood medulloblastoma? Clin Neurol Neurosurg 1997;99:113-6.  Back to cited text no. 4    
5.Friedberg MH, David O, Adelman LS, Heilman CB. Recurrence of medulloblastoma: Violation of Collins' law after two decades. Surg Neurol 1997;47:571-4.  Back to cited text no. 5    
6.Hardy DG, Hope-Stone HF, McKenzie CG, Scholtz CL. Recurrence of medulloblastoma after homogeneous field radiotherapy. Report of three cases. J Neurosurg 1978;49:434-40.  Back to cited text no. 6    
7.Sun LM, Yeh SA, Wang CJ, Huang EY, Chen HC, Hsu HC, et al . ­Postoperative radiation therapy for medulloblastoma-high recurrence rate in the subfrontal region. J Neurooncol 2002;58:77-85.  Back to cited text no. 7    
8.Garcνa Escrig M, Dνaz Guzmαn J, Soto Tιllez O, Simón de las Heras R, Mateos Beato F, Muρoz A, et al . Cerebellar medulloblastoma in ­childhood: s0 upratentorial metastasis. Neurologia 1993;8:131-4.  Back to cited text no. 8    
9.Cohen MS, Kushner MJ, Dell S. Frontal lobe astrocytoma following radiotherapy for medulloblastoma. Neurology 1981;31:616-9.  Back to cited text no. 9    
10.Osumi AK, Mclendon RE, Tien RD, Friedman HS, Graham M, ­Hockenberger B, et al . Well differentiated astrocytoma occurring nine years after radiation therapy for medulloblastoma. Clin Neuropathol 1994;13:281-5.  Back to cited text no. 10    

Top
Correspondence Address:
Y B Roka
National Neurosurgical Referral Centre, National Academy of Medical Sciences, Bir Hospital, Kathmandu
Nepal
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0377-4929.55001

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    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1]

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