Rhabdoid meningioma

Rhabdoid meningioma

Rhabdoid meningiomas are rare aggressive variant, adopted in WHO classification of CNS neoplasm (2000) and belonged to WHO Grade III subtype 1) 2).

Outcome

The behavior of rhabdoid meningiomas otherwise lacking malignant features remains unknown as most of the originally reported aggressive cases showed anaplastic histologic features independently of rhabdoid phenotype.

Rhabdoid meningioma is a malignant subtype of meningioma that occurs very rarely in pediatric patients. Additionally, rhabdoid meningioma, when it does occur in pediatric patients, has a high tendency to recur. Radical surgical resection with adjuvant radiotherapy is essential to prolonging survival.

Khairy et al.,reported the first case with extracranial extension to the mediastinal veins and heart 3).

Case series

44 patients with rhabdoid meningiomas lacking anaplastic features. Median age at diagnosis was 48.6 years (range 10-79). Location was supratentorial in 28 (63.6%), skull base in 15 (34.1%), and spinal in 1 (2.3%). Tumor grade was otherwise World Health Organization grade I (n = 22, 50%) or II (n = 22, 50%). Rhabdoid cells represented <20% of the tumor in 12 cases (27.3%), 20% to 50% in 18 (40.9%), and >50% in 14 (31.8%). Median clinical follow-up, available for 38 patients, was 5.0 years (range 0.17-14.2). Recurrence occurred in 9 patients (5-year recurrence-free survival, 73.7%) with a significantly higher risk in subtotally resected tumors (p = 0.043). Rhabdoid cell percentage was not associated with recurrence. Six patients died (4 of disease, 2 of unclear causes); 5-year overall survival was 86.7%, a mortality in excess of that expected in grade I-II meningiomas but much lower than originally reported. Review of 50 similar previously reported cases confirmed this findings. The authors suggest that rhabdoid meningiomas be graded analogously to nonrhabdoid tumors, with caution that some may still behave aggressively and close follow-up is recommended 4).

Case reports

References

1)

Kar A, Rath G, Mohanty L, Behera P, Mohapatro S, Lenka A, et al. Rhabdoid meningioma-An uncommon and aggressive variant. J Cancer Sci Ther. 2013;5:343–5.
2)

Kakar V, Elsmore A. Rhabdoid meningioma: Rare and highly aggressive. Internet J Neurosurg. 2005;3:1–5.
3)

Khairy S, Al-Ahmari AN, Saeed MA, Azzubi M. Pediatric Rhabdoid Meningioma with extension to the heart, a first case report and literature review. World Neurosurg. 2019 Jun 24. pii: S1878-8750(19)31669-9. doi: 10.1016/j.wneu.2019.06.117. [Epub ahead of print] PubMed PMID: 31247353.
4)

Vaubel RA, Chen SG, Raleigh DR, Link MJ, Chicoine MR, Barani I, Jenkins SM, Aleff PA, Rodriguez FJ, Burger PC, Dahiya S, Perry A, Giannini C. Meningiomas With Rhabdoid Features Lacking Other Histologic Features of Malignancy: A Study of 44 Cases and Review of the Literature. J Neuropathol Exp Neurol. 2016 Jan;75(1):44-52. doi: 10.1093/jnen/nlv006. Epub 2015 Dec 7. PubMed PMID: 26705409.

Meningioma differential diagnosis

Meningioma differential diagnosis

The differential for dural lesions is extensive and includes 1):

Dural metastases (e.g. breast cancer)

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) and meningioma exhibit similar radiographic features, however, they differ in their prognoses. Preoperative differentiation between them is important for determining the treatment and follow-up plan.

Age and myo-inositol level calculated from MRS are useful factors for distinguishing SFT/HPC from meningioma preoperatively 2).


Gliosarcomas

Leiomyosarcomas

Melanocytomas

Hodgkin lymphoma

Plasmacytomas

Inflammatory pseudotumors

Neurosarcoidosis

Plasma cell granulomas

Rosai Dorfman disease

Castleman disease

Xanthomas

Rheumatoid nodules

Tuberculomas


In the setting of hyperostosis consider:

Paget’s disease

Fibrous dysplasia

Sclerotic metastases (e.g. prostate and breast carcinoma)


Specific location differentials include:

Cerebellopontine angle

Vestibular schwannoma

Pituitary region:

Base of the skull:

References

1)

Johnson MD, Powell SZ, Boyer PJ, Weil RJ, Moots PL: Dural lesions mimicking meningiomas. Hum Pathol 33:1211–1226, 2002
2)

Ohba S, Murayama K, Nishiyama Y, Adachi K, Yamada S, Abe M, Hasegawa M, Hirose Y. Clinical and radiographic features for differentiating solitary fibrous tumor/hemangiopericytoma from meningioma. World Neurosurg. 2019 Jun 21. pii: S1878-8750(19)31646-8. doi: 10.1016/j.wneu.2019.06.094. [Epub ahead of print] PubMed PMID: 31233926.

Convexity meningioma surgery

Convexity meningioma surgery

Surgical safety checklist

Preoperative antibiotic prophylaxis

Skin Preparation

Positioning

For convexity meningioma, the head is positioned so that the center of the tumor is uppermost, the same position as described for parasagittal tumors or for tumors close to the midline.

Skin incision – Burr Holes – Dura mater opening

The incision and bone flap must be large enough to allow for excision of a good margin of dura around the tumor attachments.

The meningeal arteries are occluded as they are exposed.

Technical issues

These tumors can be removed intact by placing gentle traction on the dural attachment and working circumferentially around the tumor to divide the attachments to the cortex. However, if the surface of the tumor cannot be easily visualized without placing significant retraction on the cortex, internal decompression of the tumor is done and the capsule is reflected into the area of decompression.

In a situation where the tumor arises over the frontal temporal junction and grows into the sylvian fissure, the medial capsule and the dural attachment may extend down onto the lateral floor of the anterior fossa and anterior wall of the middle fossa, and the medial capsule of the tumor can be attached to branches of the middle cerebral artery.

A study showed that meningioma recurrence was unlikely when autologous cranioplasty was done with refashioned hyperostotic bone. This could be done in the same setting with meningioma excision. There was no recurrence at a mean of 5-year follow-up in convexity meningiomas 1).

Videos

1)

Lau BL, Che Othman MI, Fakhri M, San Liew DN, San Lim S, Bujang MA, Hieng Wong AS. Does putting back hyperostotic bone flap in meningioma surgery causes tumor recurrence? An observational prospective study. World Neurosurg. 2019 Mar 26. pii: S1878-8750(19)30863-0. doi: 10.1016/j.wneu.2019.03.183. [Epub ahead of print] PubMed PMID: 30926555.
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