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 Table of Contents  
Year : 2016  |  Volume : 4  |  Issue : 4  |  Page : 301-305

Rhabdoid meningioma with lung metastasis in a paediatric patient: A case report and literature review

1 Department of Neurosurgery, Prince Sultan Military Medical City, Riyadh, Saudi Arabia
2 Department of Pathology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia

Date of Web Publication12-Oct-2016

Correspondence Address:
Hussein Kheshaifati
Department of Neurosurgery, Prince Sultan Military Medical City, P. O. Box: 7897, Riyadh 11159
Saudi Arabia
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DOI: 10.4103/2468-6360.191915

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Meningioma is a common intracranial tumour which is usually benign. It is well-known to be high grade as atypical or anaplastic with grade II or III. Meningiomas are rarely found in paediatric patients. Extracranial metastasis from brain meningioma is rare but can occur in the lungs, pleura, liver, lymph node and bones. In this paper, we report a 16-year-old female with an extracranial metastasis of grade III meningioma to the lung. She underwent gross total resection along with chemo- and radiotherapy. The outcome and treatment modality would also be discussed.

Keywords: Meningioma, metastasis, rhabdoid

How to cite this article:
Kheshaifati H, Alhindi H, Homoud MM. Rhabdoid meningioma with lung metastasis in a paediatric patient: A case report and literature review. J Health Spec 2016;4:301-5

How to cite this URL:
Kheshaifati H, Alhindi H, Homoud MM. Rhabdoid meningioma with lung metastasis in a paediatric patient: A case report and literature review. J Health Spec [serial online] 2016 [cited 2021 Jan 19];4:301-5. Available from: https://www.thejhs.org/text.asp?2016/4/4/301/191915

  Introduction Top

Meningiomas are common tumours in adults but uncommon in the paediatric population. Rhabdoid meningioma in paediatric patients as subtype is a rare variety that can metastasise. [1],[2]

We will discuss a 16-year-old female who had a dural-based lesion which was diagnosed as rhabdoid meningioma after resection (Simpson Grade I), which metastasised to the lungs during chemo- and radiotherapy.

  Case report Top

A 16-year-old right-handed female was referred to our hospital in May 2011 after being treated in another facility for three weeks. The patient presented with a history of vomiting, generalised tonic colonic seizure, right temporal swelling and progressive deterioration of vision. Her seizures were controlled using phenytoin, but she was then started on dexamethasone with gastric protection.

On examination, the patient was conscious, alert and haemodynamically stable. Chest and abdomen examination was unremarkable. On central nervous system examination, pupils were 2 mm bilaterally reactive, but she was blind on the right eye with decreased visual acuity on the left eye. However, no other deficits were found overall. She had an elevated disc on fundus eye exam on the right side. There was a clear non-tender pulsatile swelling on the right temporal area with no skin changes or warmth.

Computed tomography (CT) and magnetic resonance imaging of the brain showed a right frontotemporal lesion with heterogeneous enhancement as well as necrosis invading the bone and muscle. Cerebral angiography with embolisation of the tumour feeders from external carotid artery was done [Figure 1] and [Figure 2].
Figure 1 (a and b): Axial computed tomography showed a right frontotemporal lesion, most likely a dural-based lesion, with skull and muscle invasion

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Figure 2 (a and b): Axial and coronal T1 with contrast showed a dural-based lesion at the frontotemporal area with heterogeneous rim enhancement, vasogenic oedema and midline shift. Invasion and destruction of the bone and muscle were also noted

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Right temporal craniectomy with gross total resection of the lesion was performed. In addition, removal of invaded muscle bone and dura followed by duraplasty and cranioplasty were done, [Figure 3].
Figure 3 (a-c): Axial computed tomography done on the 1st postoperative day, with and without contrast, demonstrating gross total resection using cranioplasty for the lesion

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The patient tolerated the procedure very well with no deficit. She was observed in the intensive care unit for the first 24 h postoperatively, where brain CT with contract was performed, which showed gross total resection without haematoma. She was discharged on the 7 th postoperative day in good condition.

On histopathology exam, haematoxylin and eosin stain showed sheet of discohesive cells with extensive necrosis and evidence of embolization. The cells had moderate to abundant cytoplasm and eccentric nuclei with variable nucleoli. Eccentric cytoplasmic inclusions were seen in many areas, which is characteristic of rhabdoid cells. Mitosis was frequent. Immunohistochemistry proved the tumour cells were positive for vimentin and S100 protien. Epithelial membrane antigen (EMA) was only focally expressed; this profile raised the suspicion for melanoma. However, other melanoma markers were negative, and cytokerative was also negative. Final diagnosis made was rhabdoid meningioma, [Figure 4].
Figure 4 (a-c): H and E stain showed necrosis and intravascular embolization material (onyx) and rhabdoid cells with their eccentric cytoplasmic inclusion with two mitotic figures

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The patent was admitted under Oncology for one month postoperatively, where she was started on radiotherapy and received one cycle of chemotherapy. The plan was to give her 30 Gy fractions of radiotherapy; however, she was found to have massive pleural effusion. Therefore, pleurodesis was done on one side and a drain was inserted on the other side, [Figure 5].
Figure 5 (a-c): Axial chest computed tomography with contrast showed multiple bilateral large nodules in the lungs with pleural effusion

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Chest CT done, before starting oncology treatment, showed multiple bilateral lung nodules [Figure 5]. Another chest CT done when her condition deteriorated in the last week of August 2011 showed progression of the lung nodules despite her receiving an additional cycle of chemotherapy and 18 - 19 Gy fractions of radiotherapy. Brain CT performed showed recurrence of the meningioma, [Figure 6].
Figure 6: (a-c) Axial brain computed tomography with contrast

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The patient's oxygen demand increased and general condition continued to deteriorate daily despite full supportive measures. Unfortunately, the patient died three months after the tumour resection.

Histopathological study of the pleural fluid showed malignant cells which suggested metastasis. Due to the patient's clinical condition, tissue biopsy could not be taken.

  Discussion Top

Meningiomas account for 30% of primary intracranial tumours. Usually, these occur in the middle-aged and elderly; however, it can also occur in children where it is found to behave more aggressively. Its recurrence is usually multifactorial and the grade of the tumour plays an important role in its say. Rate of recurrence in Grade I: 7 - 20%, Grade II: 29 - 40%, and Grade III: 50 - 70%. [3] Meningiomas account for 1 - 4% of intracranial tumours in children and rarely with Grade III type. [4]

Tumours with rhabdoid appearance was first described by Beckwith and Palmer in 1978 in a young child with renal tumour. Rhabdoid meningioma is known to be one of the subtypes of meningioma, which is listed under Grade III, first described by Kepes in 1998 and had a poor outcome. It was recently added to the World Health Organization's classification of tumours of the nervous system, in the year 2000. [5],[6],[7]]

One in 1000 meningiomas metastasise extracranially, and it usually occurs following surgery. Extracranial metastasis most often occur with anaplastic types and is associated with poor outcome, mostly metastasising to lungs, pleura, liver, lymph nodes and bone. [5] Only one case of metastasising meningioma was reported first by Cushing and Eisenhardt in 1938. [8]

The presence of rhabdoid cells is a clue for its diagnosis, with cytological features of abundant eosinophilic cytoplasm, eccentric nuclei, and intracytoplasmic hyaline inclusions throughout, or lesions in addition to the usual features of a meningioma where it also might be variable. [9],[10] Rhabdoid cells by themselves are not anaplastic. Rhabdoid meningioma anaplastic or atypical features as a whole will most likely have necrosis, high mitosis, atypia, sheeting, elevated MIB-1 and brain invasion. Staining varies in degrees in epithelial membrane antigen (EMA), vimentine and cytokeratine. [10]

In malignant meningioma, there is difficulty distinguishing them from primary meningeal sarcoma. In addition to the clinical and radiological clues, the role of positive EMA will suggest meningioma and sarcoma herringbone architecture. [10] This is why diagnosis would require a detailed study, and exclusion of other differentials is the clue in some cases.

Our literature review revealed 15 reported cases of metastasis from rhabdoid meningioma; three of which were found to be among the paediatric age group, [Table 1]. [2],[11],[12],[13],[14],[15],[16],[17],[18],[19],[20],[21],[22] We found it interesting that Mordechai et al., used BRFA inhibitor as a trial treatment for recurrence after genetic analysis, which showed regression in the lesion with clinical improvement.
Table 1: Summary of literature review of all patients with metastasis rhabdoid meningioma

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  Conclusion Top

Rhabdoid meningioma is a rare pathology that can occur in children with often metastasis after surgery. In general, the outcome is poor owing to both, recurrence and metastasis.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Morina A, Kelmendi F, Morina O, Pazanin L, Dragusha S, Ahmeti F, et al. Rhabdoid meningioma in an eight-year-old child. Med Arh 2010;64:123-4.  Back to cited text no. 1
Wang Z, Kong M, Li J, Xiao W, Zheng S. Intraspinal rhabdoid meningioma metastasis to the liver. J Clin Neurosci 2011;18:714-6.  Back to cited text no. 2
Buccoliero AM, Castiglione F, Rossi Degl′Innocenti D, Franchi A, Sanzo M, Cetica V, et al. Pediatric rhabdoid meningioma: A morphological, immunohistochemical, ultrastructural and molecular case study. Neuropathology 2011;31:59-65.  Back to cited text no. 3
Germano IM, Edwards MS, Davis RL, Schiffer D. Intracranial meningiomas of the first two decades of life. J Neurosurg 1994;80:447-53.  Back to cited text no. 4
International Agency for Research on Cancer. WHO classification of tumours. In: WHO Classification of Tumours of the Central Nervous System. 3 rd ed. Lyon, France: International Agency for Research on Cancer (IARC); 2007.  Back to cited text no. 5
Wu YT, Lin JW, Wang HC, Lee TC, Ho JT, Lin YJ. Clinicopathologic analysis of rhabdoid meningioma. J Clin Neurosci 2010;17:1271-5.  Back to cited text no. 6
Kepes JJ, Moral LA, Wilkinson SB, Abdullah A, Llena JF. Rhabdoid transformation of tumor cells in meningiomas: A histologic indication of increased proliferative activity: Report of four cases. Am J Surg Pathol 1998;22:231-8.  Back to cited text no. 7
Cushing H, Eisenhardt L. Meningiomas. Ilinois: Thomas, Springfield; 1938. p. 692.  Back to cited text no. 8
Bansal M, Pathak VP, Kishore S, Bansal KK. Rhabdoid meningioma: Rapid intraoperative diagnosis on squash smears. Diagn Cytopathol 2010;38:594-6.  Back to cited text no. 9
Burger PC, Scheithauer BW, Vogel FS, editors. Surgical Pathology of the Nervous System and its Coverings. 4 th ed. Churchill Livingstone: Hardcover; 2002.  Back to cited text no. 10
Perry A, Scheithauer BW, Stafford SL, Abell-Aleff PC, Meyer FB. "Rhabdoid" meningioma: An aggressive variant. Am J Surg Pathol 1998;22:1482-90.  Back to cited text no. 11
Bannykh SI, Perry A, Powell HC, Hill A, Hansen LA. Malignant rhabdoid meningioma arising in the setting of preexisting ganglioglioma: A diagnosis supported by fluorescence in situ hybridization. Case report. J Neurosurg 2002;97:1450-5.  Back to cited text no. 12
Parwani AV, Mikolaenko I, Eberhart CG, Burger PC, Rosenthal DL, Ali SZ. Rhabdoid meningioma: Cytopathologic findings in cerebrospinal fluid. Diagn Cytopathol 2003;29:297-9.  Back to cited text no. 13
Al-Habib A, Lach B, Al Khani A. Intracerebral rhabdoid and papillary meningioma with leptomeningeal spread and rapid clinical progression. Clin Neuropathol 2005;24:1-7.  Back to cited text no. 14
Wakabayashi K, Suzuki N, Mori F, Kamada M, Hatanaka M. Rhabdoid cystic papillary meningioma with diffuse subarachnoid dissemination. Acta Neuropathol 2005;110:196-8.  Back to cited text no. 15
Koenig MA, Geocadin RG, Kulesza P, Olivi A, Brem H. Rhabdoid meningioma occurring in an unrelated resection cavity with leptomeningeal carcinomatosis. Case report. J Neurosurg 2005;102:371-5.  Back to cited text no. 16
Santhosh K, Kesavadas C, Radhakrishnan VV, Thomas B, Kapilamoorthy TR, Gupta AK. Rhabdoid and papillary meningioma with leptomeningeal dissemination. J Neuroradiol 2008;35:236-9.  Back to cited text no. 17
Eom KS, Kim DW, Kim TY. Diffuse craniospinal metastases of intraventricular rhabdoid papillary meningioma with glial fibrillary acidic protein expression: A case report. Clin Neurol Neurosurg 2009;111:619-23.  Back to cited text no. 18
Eom KS, Kim HS, Kim TY, Kim JM. Intraventricular malignant meningioma with CSF-disseminated spinal metastasis: Case report and literature review. J Korean Neurosurg Soc 2009;45:256-9.  Back to cited text no. 19
Zhou Y, Xie Q, Gong Y, Mao Y, Zhong P, Che X, et al. Clinicopathological analysis of rhabdoid meningiomas: Report of 12 cases and a systematic review of the literature. World Neurosurg 2013;79:724-32.  Back to cited text no. 20
Parameshwaran Nair R, Vinod, Sarma Y, Nayal B, Kaur Dil S, Tripathi PK. Metastatic rhabdoid meningioma of the parotid - Mimicking primary salivary gland neoplasm. Int J Surg Case Rep 2015;6C: 104-6.  Back to cited text no. 21
Mordechai O, Postovsky S, Vlodavsky E, Eran A, Constantini S, Dotan E, et al. Metastatic rhabdoid meningioma with BRAF V600E mutation and good response to personalized therapy: Case report and review of the literature. Pediatr Hematol Oncol 2015;32:207-11.  Back to cited text no. 22


  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]

  [Table 1]


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