|Year : 2016 | Volume
| Issue : 4 | Page : 301-305
Rhabdoid meningioma with lung metastasis in a paediatric patient: A case report and literature review
Hussein Kheshaifati1, Hindi Alhindi2, Mohammed M Homoud1
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 Publication||12-Oct-2016|
Department of Neurosurgery, Prince Sultan Military Medical City, P. O. Box: 7897, Riyadh 11159
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 2019 Aug 19];4:301-5. Available from: http://www.thejhs.org/text.asp?2016/4/4/301/191915
| Introduction|| |
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. ,
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|| |
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].
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|| |
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%.  Meningiomas account for 1 - 4% of intracranial tumours in children and rarely with Grade III type. 
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. ,,]
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.  Only one case of metastasising meningioma was reported first by Cushing and Eisenhardt in 1938. 
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. , 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. 
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.  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]. ,,,,,,,,,,,, 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|| |
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]