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CASE REPORT
Year : 2021  |  Volume : 16  |  Issue : 3  |  Page : 618-622

Extra-axial desmoplastic medulloblastoma in adult cerebellopontine angle: Case report and noninvasive molecular subgrouping utilizing magnetic resonance imaging-based radiomics nomogram


Department of Neurosurgery, Mayo Hospital, King Edward Medical University, Lahore, Pakistan

Date of Submission04-Dec-2020
Date of Decision01-Feb-2021
Date of Acceptance20-Mar-2021
Date of Web Publication14-Sep-2021

Correspondence Address:
Dr. Sundus Ali
Department of Neurosurgery, Mayo Hospital, King Edward Medical University, Lahore
Pakistan
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ajns.AJNS_525_20

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  Abstract 

Cerebellopontine angle (CPA) is an atypical site for adult medulloblastoma (MB) with only 12 cases reported in pure extra-axial location. None was predicted on preoperative imaging while the most common misdiagnosis was petrous meningioma. We add the 13th case to this list, attempting to reiterate the radiological features for preoperative prediction of this rare pathology on conventional magnetic resonance imaging (MRI). Molecular subtyping also is not yet reported for adult extra-axial CPA MB. We propose the routine use of MRI-based nomograms, in atypical CPA extra-axial masses, for noninvasive prediction of molecular subgroup, especially in resource-limited setups that lack the facility of genetic profiling.

Keywords: Adult, cerebellopontine angle, medulloblastoma, radiomics


How to cite this article:
Ali S, Qasim A, Tasdique S, Sarwar MR, Shams S. Extra-axial desmoplastic medulloblastoma in adult cerebellopontine angle: Case report and noninvasive molecular subgrouping utilizing magnetic resonance imaging-based radiomics nomogram. Asian J Neurosurg 2021;16:618-22

How to cite this URL:
Ali S, Qasim A, Tasdique S, Sarwar MR, Shams S. Extra-axial desmoplastic medulloblastoma in adult cerebellopontine angle: Case report and noninvasive molecular subgrouping utilizing magnetic resonance imaging-based radiomics nomogram. Asian J Neurosurg [serial online] 2021 [cited 2021 Dec 4];16:618-22. Available from: https://www.asianjns.org/text.asp?2021/16/3/618/324461


  Introduction Top


Adult medulloblastomas (MBs) are relatively rare and constitute 0.4%–1.0% of adult primary brain tumors.[1] Cerebellopontine angle (CPA) is an atypical site for MB with 42 cases reported in both pediatric and adult population, intra- and extraaxial locations combined.[2] Extraaxially, the tumors have been localized in two areas: the tentorial region and the CPA.[3] In adults, only 12 cases were found in extra-axial CPA location.[2],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13]Here, we report the 13th case of an extra-axial lesion in the CPA of a 27-year-old male, which was presumed to be meningioma on preoperative imaging but turned out to be desmoplastic MB.

As molecular subtyping is not available in resource-limited setups in low income countries, we utilized ''for the first time'' the MRI based nomogram as an alternative to expensive genetic profiling and propose its use in selective cases.


  Case Report Top


A 27-year-old male patient presented with headache, vomiting, left-sided ataxia, and left-sided mild hearing loss of 1-month duration. The vision was normal. Fundus examination showed papilledema. He had left-sided House-Brackman Grade 1 facial paresis, left-sided mild sensorineural hearing loss, and left cerebellar signs on physical examination.

Imaging

A computed tomography scan of the brain showed a left CPA mixed-density nonenhancing lesion (5.4 cm × 2.8 cm) with broad-based tentorial attachment and displacement of the fourth ventricle but not causing obstructive hydrocephalus. No evidence of calcification was noted. Magnetic resonance imaging (MRI) showed T1 hypo/T2 heterointense, extra-axial lesion in left CPA, which had a wide tentorial attachment and showed moderate heterogeneous enhancement with IV gadolinium. There was no meatal extension [Figure 1]a, [Figure 1]b, [Figure 1]c. Adjacent white matter edema in the left cerebellar hemisphere was noted [Figure 1]d.
Figure 1: (a) Axial T1 showing hypointense and axial T2 showing heterointense mass in left cerebellopontine angle (b) T2 Sagittal showing central hyperintensity (c) Coronal contrast images showing heterogeneous moderate enhancement and wide tentorial attachment (d) T2 FLuid Attenuated Inversion Recovery (FLAIR) showing adjacent cerebellar edema

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Treatment

He underwent a left retromastoid craniectomy. A moderately vascular, soft suckable, grayish pink lesion was found densely adherent to petro-tentorial junction laterally and to the inferior surface of left hemi tentorium till hiatus medially. This attachment was heavily coagulated [Figure 2]a. The tumor-brainstem interface was well defined, but the tumor-cerebellar interface was ill-defined. All cranial nerves from 4 to 11 were preserved [Figure 2]b. Gross total excision achieved in a piecemeal fashion [Figure 2]c. The central necrotic part corresponded to T2 hyperintensity on imaging.
Figure 2: (a) Perioperative image showing heavy coagulation of tentorial attachment (b) Cranial nerve 5 (yellow arrow), 7, 8 (green arrow), 9, 10 (Blue arrow), and 11 (Red arrow) seen intact following tumor resection (c) tumor specimen

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Histopathology showed a nodular arrangement of round-to-oval cells having hyperchromatic nuclei and scanty cytoplasm, with brisk mitotic activity suggestive of Desmoplastic MB–WHO Grade IV [Figure 3]a. Immunohistochemical staining showed patchy cytoplasmic staining of beta-catenin, wild type p53, and synaptophysin positive with background GFAP activity [Figure 3]b, [Figure 3]c, [Figure 3]d. Molecular subtyping is currently not available in our country.
Figure 3: (a) H and E stain illustrating the peculiar nodular arrangement (b) cytoplasmic reactivity of Beta catenin (c) wild type P53 (d) Synaptophysin positive (e) Axial T1 without contrast at 9-month FU (f) Coronal Contrast at 9-month FU showing no evidence of recurrence or mets

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Postoperatively, his headache and ataxia improved but his facial paresis transiently worsened to Grade 2 which improved to Grade 1 over 2 weeks. His craniospinal MRI with contrast was done after 4 weeks of surgery. Cerebrospinal fluid analysis from lumbar tap turned out to be negative for malignant cells. He was labelled as “standard risk” based on the size of residual <1.5 cm2, no metastasis, nonlarge cell histology, and age >3 years. The patient underwent craniospinal irradiation as well as chemotherapy with vincristine, cisplatin, and cyclophosphamide. Postadjuvant therapy, craniospinal imaging was clear for any metastasis or recurrence at 9 months [Figure 3]e and [Figure 3]f.


  Discussion Top


Adult MB is known to be lateral hemispheric. CPA is a rarely reported location, challenging to diagnose both clinically and radiologically. There have been only 12 reported cases of extra-axial MB in the adult literature [Table 1].[2],[4],[5],[6],[7],[8],[9],[10],[11],[12],[13]
Table 1: Summary of Previously Reported Extra.axial Medulloblastoma in Adult Cerebellopontine Angle

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A review of all reported cases shows that CPA MB is twice more common in males in their 2nd–3rd decade, with the classic variety seen in more than half of the cases. Ten out of 12 reports so far published considered meningioma as a provisional diagnosis based on broad tentorial attachment, despite heterogeneous enhancement. Meatal extension which is considered pathognomonic for vestibular schwannoma was only seen in one case.[13]

Our preoperative diagnosis was based on a broad base extra-axial lesion, without extension into the internal auditory meatus favoring meningioma as well as disapproving schwannoma. Although the lack of dural tail and heterogeneous enhancement was not classical of a meningioma, the short clinical course and white matter edema in the adjacent cerebellar lobe favored atypical meningioma preoperatively. Classical radiological features of adult MB are well delineated in the literature but not for atypical locations. All 12 cases reported so far were diagnosed after histopathology leaving MRI features to be focused more than ever before.

Molecular subtyping has not yet been reported for adult CPA MBs. We lack the facility in our setup but tried to speculate on the basis of location, histology, and immunohistochemistry (IHC) retrospectively. Moreover, in the era of molecular genetics, radiogenomics have provided sufficient data to define certain molecular subtypes noninvasively, although it is quite clear that no single imaging feature is pathognomonic of any particular subgroup, certain imaging characteristics are much more prevalent in one subgroup compared to others and may even be highly specific for an individual molecular subgroup.[14]

Here, we intend to emphasize the use of an integrated approach utilizing location, histology, IHC, and radiomics to predict molecular subgroup in adult cases of MB, in developing countries that lack the facility of genetic subtyping. A brief review of the correlation of molecular subgroup to location, histology, IHC, and radiomics is presented individually, which can help predict the molecular subgroup with a fair degree of accuracy when combined.

Location-molecular subtype correlation

Adult MBs unlike pediatric are reported to be of two molecular subtypes, instead of four, with Group 3 occurring as an exception and Group 4 restricted to midline.[15],[16] Lateral location is reserved for WNT and SHH tumors, with the former mostly seen in CPA and the latter in the cerebellar hemisphere. Furthermore, superior location abutting and/or reaching the tentorium is a specific imaging feature of SHH-subgroup MB seen in 48% of patients as opposed to 6% in other groups.[14]

Histology-molecular subgroup correlation

There is a consensus among researchers that all desmoplastic histology in adults belongs to the SHH subgroup, but the opposite is not true: Not all SHH tumors in adults are of desmoplastic histology.[15],[17],[18]Moreover, another study reports that 9% of adult SHH-MBs are located in CP/CPA.[19]

Immunohistochemistry-molecular subgroup correlation

Beta-catenin localization to cytoplasm instead of nucleus excludes WNT.[18] In addition, some markers such as GAP 1 and YAP1 are considered pathognomonic for SHH and WNT type, respectively.[20]

Radiomics-molecular subgroup correlation

Nomograms based on multiparametric MRI have suggested 95% predictive accuracy for SHH subgroup,[21] best among all other subgroups. We applied the SHH subgroup-specific nomogram to our case [Figure 4] and the probability of the tumor to be SHH type turned out to be 98%. As only two molecular subgroups can be expected in adult CPA, we propose to suggest WNT type in cases where SHH type seems less probable on the nomogram, combined with nuclear reactivity for beta-catenin.[20]
Figure 4: Nomogram for SHH used to predict the molecular subgroup (used with permission of Dasgupta et al.[21]

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In our case, CPA location was the only factor favoring WNT type, while tentorial attachment, desmoplastic histology, and lack of nuclear reactivity of beta-catenin were favoring SHH type. Hence, we used SHH specific nomogram in retrospect to predict the molecular subgroup with fair degree of accuracy. Desmoplastic histology, p53 wild type and SHH subgroup all confer good to intermediate prognosis in this case.


  Conclusion Top


The use of an integrated approach, based on location, appearance, histology, and demographics not only predicts molecular subgroup but also help defining prognosis. MRI-based nomograms in selected cases can substitute expensive genetic profiling in resource-limited setups.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Giordana MT, Schiffer P, Lanotte M, Girardi P, Chio A. Epidemiology of adult medulloblastoma. Int J Cancer 1999;80:689-92.  Back to cited text no. 1
    
2.
Ratha V, Karmarkar VS, Deopujari CE. Extra-axial cerebellopontine angle adult medulloblastoma: An unusual presentation at an unusual site. Indian J Neurosurg 2019;08:203-6.  Back to cited text no. 2
    
3.
Doan NB, Patel M, Nguyen HS, Janich K, Montoure A, Shabani S, et al. A rare extra-axial midline tentorial adult medulloblastoma with dural-tail sign mimicking a meningioma. Asian J Neurosurg 2018;13:475-7.  Back to cited text no. 3
[PUBMED]  [Full text]  
4.
Becker RL, Becker AD, Sobel DF. Adult medulloblastoma: Review of 13 cases with emphasis on MRI. Neuroradiology 1995;37:104-8.  Back to cited text no. 4
    
5.
Akay KM, Erdogan E, Izci Y, Kaya A, Timurkaynak E. Medulloblastoma of the cerebellopontine angle – Case report. Neurol Med Chir (Tokyo) 2003;43:555-8.  Back to cited text no. 5
    
6.
Singh SK, Kumar N. Extraaxial cerebellopontine angle medulloblastoma with multiple intracranial metastases in adult: A rare case report with a review of literature. Asian J Neurosurg 2020;15:695-8.  Back to cited text no. 6
  [Full text]  
7.
Gil-Salú JL, Rodríguez-Peña F, López-Escobar M, Palomo MJ. et al. Meduloblastoma de presentación extra-axial en el ángulo pontocerebeloso. Neurocirugia 2004;15:285-9.  Back to cited text no. 7
    
8.
Fallah A, Banglawala SM, Provias J, Jha NK. Extra-axial medulloblastoma in the cerebellopontine angle. Can J Surg 2009;52:E101-E102.  Back to cited text no. 8
    
9.
Furtado SV, Venkatesh PK, Dadlani R, Reddy K, Hegde AS. Adult medulloblastoma and the “dural-tail” sign: Rare mimic of a posterior petrous meningioma. Clin Neurol Neurosurg 2009;111:540-3.  Back to cited text no. 9
    
10.
Singh M, Cugati G, Symss NP, Pande A, Vasudevan MC, Ramamurthi R. Extra axial adult cerebellopontine angle medulloblastoma: An extremely rare site of tumor with metastasis. Surg Neurol Int 2011;2:25.  Back to cited text no. 10
[PUBMED]  [Full text]  
11.
Spina A, Boari N, Gagliardi F, Franzin A, Terreni MR, Mortini P. Review of cerebellopontine angle medulloblastoma. Br J Neurosurg 2013;27:316-20.  Back to cited text no. 11
    
12.
Bahrami E, Bakhti S, Fereshtehnejad SM, Parvaresh M, Khani MR. Extra-axial medulloblastoma in cerebello-pontine angle: A report of a rare case with literature review. Med J Islam Repub Iran 2014;28:57.  Back to cited text no. 12
    
13.
Goudihalli SR, Pathak A, Brar R, Mundi I, et al. Reappraisal of cerebellopontine angle medulloblastomas: Report of a fatal case and lessons learned. Interdiscip Neurosurg Adv Tech Case Manag 2018;12:20-3.  Back to cited text no. 13
    
14.
Dasgupta A, Gupta T. Radiogenomics of medulloblastoma: Imaging surrogates of molecular biology. J Transl Genet Genomics 2018;2:15. [doi: 10.20517/JTGG.2018.21].  Back to cited text no. 14
    
15.
Remke M, Hielscher T, Northcott PA, Witt H, Ryzhova M, Wittmann A, et al. Adult medulloblastoma comprises three major molecular variants. J Clin Oncol 2011;29:2717-23.  Back to cited text no. 15
    
16.
Zhao F, Ohgaki H, Xu L, Giangaspero F, Li C, Li P, et al. Molecular subgroups of adult medulloblastoma: A long-term single-institution study. Neuro Oncol 2016;18:982-90.  Back to cited text no. 16
    
17.
Kool M, Korshunov A, Pfister SM. Update on molecular and genetic alterations in adult medulloblastoma. Memo 2012;5:228-32.  Back to cited text no. 17
    
18.
Ellison DW, Dalton J, Kocak M, Nicholson SL, Fraga C, Neale G, et al. Medulloblastoma: Clinicopathological correlates of SHH, WNT, and non-SHH/WNT molecular subgroups. Acta Neuropathol 2011;121:381-96.  Back to cited text no. 18
    
19.
Zhao F, Li C, Zhou Q, Qu P, Wang B, Wang X, et al. Distinctive localization and MRI features correlate of molecular subgroups in adult medulloblastoma. J Neurooncol 2017;135:353-60.  Back to cited text no. 19
    
20.
Aljoghaiman M, Taha MS, Abdulkader MM. Cerebellar medulloblastoma in middle-to-late adulthood. Case Rep Pathol 2018;2018:5425398.  Back to cited text no. 20
    
21.
Dasgupta A, Gupta T, Pungavkar S, Shirsat N, Epari S, Chinnaswamy G, et al. Nomograms based on preoperative multiparametric magnetic resonance imaging for prediction of molecular subgrouping in medulloblastoma: Results from a radiogenomics study of 111 patients. Neuro Oncol 2019;21:115-24.  Back to cited text no. 21
    


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  [Figure 1], [Figure 2], [Figure 3], [Figure 4]
 
 
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