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Table of Contents    
LETTERS TO EDITOR
Year : 2019  |  Volume : 67  |  Issue : 1  |  Page : 300-302

Recurrent atypical meningioma of the lumbosacral spine


1 Department of Histopathology, Apollo Speciality Hospital, Vanagaram, Chennai, Tamil Nadu, India
2 Department of Neurosurgery, Apollo Speciality Hospital, Vanagaram, Chennai, Tamil Nadu, India

Date of Web Publication7-Mar-2019

Correspondence Address:
Dr. N Sadiya
Department of Histopathology, Apollo Speciality Hospital, Vanagaram, Chennai - 600 095, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.253643

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How to cite this article:
Sadiya N, Pande A, Salapathi S, Ghosh S. Recurrent atypical meningioma of the lumbosacral spine. Neurol India 2019;67:300-2

How to cite this URL:
Sadiya N, Pande A, Salapathi S, Ghosh S. Recurrent atypical meningioma of the lumbosacral spine. Neurol India [serial online] 2019 [cited 2019 Mar 18];67:300-2. Available from: http://www.neurologyindia.com/text.asp?2019/67/1/300/253643




Sir,

Meningiomas are benign, slow growing tumors arising from arachnoid cap cells. Meningiomas of the spinal compartment are relatively rare when compared with those in the intracranial compartment. They account for approximately 1.2% of all meningiomas of the central nervous system and 25% of the primary spinal cord tumors. Atypical meningiomas account for 6%–8% of all meningiomas and have a higher recurrence rate. The majority of spinal meningiomas are intradural occurring predominantly in the thoracic spine. We present a case of a recurrent atypical meningioma of the lumbosacral spine in a young male. A review of literature reveals only a few reported cases of atypical meningioma of the lumbosacral spine. This case is presented in view of its rarity of its location, in a young male, and highlights its aggressive course.

Spinal meningiomas are tumors originating from arachnoid cap cells, located in the dural root sleeve,[1] and are most commonly situated in the intradural extramedullary compartment, respecting the pial layer of the spinal cord. Spinal meningiomas are rare and account for about 1.2% of all meningiomas and 25% of all spinal cord tumors.[1] The majority of the spinal meningiomas are located in the thoracic spine.[2] Spinal meningiomas are more common in women, which is thought to be influenced by factors such as sex hormones or other receptor types common to women.[3] They are slow growing tumors and, therefore, lead to symptoms only after reaching a distinct size due to significant spinal cord compression. Initially, local pain is a leading symptom; however, in a considerable number of patients, diagnosis is not confirmed until neurological deficits or gait disturbances are present. Total resection of spinal meningiomas is usually not difficult, but if the tumor is located ventral to the spinal cord and calcified, surgery becomes difficult. However, atypical meningiomas [World Health Organization (WHO) Grade II] represent a therapeutic challenge given the high recurrence rate and greater mortality compared with WHO Grade I meningiomas. Traditionally, treatment has entailed attempts at gross total resection with radiation therapy reserved for residual disease or recurrence.

A 28-year old, normotensive, euglycemic gentleman was admitted with complaints of severe low backache for the past 3 months which was sudden in onset, gradually progressive, aggravated on coughing and sneezing, radiating to both the lower limbs. He also complained of sacral area numbness over the past few days. Physical examination revealed a positive straight leg raising test, with tenderness over L5-S1 region. Magnetic resonance imaging (MRI) of the spine showed irregularly heterogeneously enhancing intradural lesion at L5-S1 with sacral nerve roots displaced laterally and showing compression [Figure 1]a, [Figure 1]b, [Figure 1]c. Electrophysiological monitoring of the external anal sphincter and somatosensory evoked potential of tibial nerve was normal. L4-S1 laminectomy with total excision of the lesion was done. At surgery, a firm gelatinous greyish-yellow soft tumor with evidence of earlier hemorrhages was seen intermingled with the roots. The tumor was dissected from the roots and total excision was done piecemeal under intraoperative electro-neurophysiological monitoring.
Figure 1: (a and b) MRI spine showing irregularly heterogenously enhancing intradural lesion at L5-S1 with sacral nerve roots displaced laterally. (c) MRI image showing compression of nerve roots

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A frozen section was requested and squash smears revealed a cellular neoplasm in a myxoid background in which were seen round-to-oval cells showing a mild anisonucleosis with coarse chromatin, inconspicuous nucleoli, and moderate cytoplasm [[Figure 2] inset]. A diagnosis of a probable myxopapillary ependymoma was rendered. The follow-up sections revealed portions of dura lined by flattened meningothelial cells, with a neoplasm arranged in sheets showing hypercellularity [Figure 3] with occasional whorling pattern [[Figure 4] inset]. The tumor showed the same cellular morphology as in squash smears except with foci of cystic changes. Foci of atypical mitosis rating to 15–17/10 hpf and focal necrosis were seen [Figure 4]. Also evident was intraluminal homogeneous eosinophilic material which was periodic acid–Schiff stain-positive and resistant to diastase digestion. The surrounding stroma showed edema, myxoid change, sparse lymphocytic infiltrate, and hemosiderin-laden macrophages.
Figure 2: Microphotograph of squash smear showing a cellular neoplasm with round to oval cells with mild anisonucleosis in a myxoid background

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Figure 3: Photomicrograph (x40) showing a hypercellular neoplasm arranged in sheets

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Figure 4: Microphotograph (x40) showing a cellular neoplasm with mitosis as depicted by arrows with occassional whorling pattern (inset)

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Immunohistochemistry done revealed positivity for epithelial membrane antigen (EMA) and vimentin, with negativity for cytokeratin, S100, estrogen receptor (ER), progesterone receptor (PR), and glial fibrillary acidic protein (GFAP). Cluster of differentiation (CD)-99 showed positivity in few foci. The Ki-67 proliferative index was 15.4% [Figure 5]. In view of the characteristic radiological and histological findings with positivity for EMA and a high proliferative index, a diagnosis of atypical meningioma was rendered. The patient's symptoms resolved completely postoperatively and the follow-up MRI did not show any residual tumor. But he presented 6 months later with low backache radiating to the right knee with numbness in the right sacral area. MRI done showed recurrence of the lesion at the postoperative bed at L5-S1 level. Total resection was done and histopathology was consistent with an atypical meningioma. The patient was advised radiotherapy.
Figure 5: Photomicrograph showing positivity for EMA,focal positivity for CD 99 and negativity for PR with a Ki-67 proliferative index of 15.4%

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Meningioma has been recognized as a tumor entity for nearly 200 years.[4] These tumors are more commonly seen in women and predominantly arise in the fourth to sixth decade. As far as spinal meningiomas are concerned, they are most prevalent in the thoracic followed by the cervical and lumbar regions.

MRI is the diagnostic modality of choice since it often delineates the characteristic dural origin of meningiomas. They are typically isointense or hypointense to grey matter on T1 and isointense or hyperintense on T2 weighted images. Spinal meningiomas display an avid homogeneous enhancement with contrast.[5] Enhancement of the adjacent dura or the dural tail is also characteristic of a meningioma.[6]

The treatment consists of total surgical resection of the tumor. In most cases, meningioma is well-distinguished from the spinal cord enabling easy removal of the tumor. Resectability of a spinal meningioma is generally related to tumor location with respect to the spinal cord. The resection of dural attachment is less radical and not routinely performed in contrast to the strategy in cerebral meningiomas. Complete resection of spinal meningiomas is more difficult when the tumor is located ventrally and ventrolaterally than is seen in dorsally located cases, and also in recurrent tumors due to arachnoid scarring and in calcified meningiomas. The major complication is recurrence. Benign meningiomas (WHO Grade I) have a recurrence rate of approximately 7%–20%, atypical meningiomas recur in 29%–38% of cases, and anaplastic meningiomas in 50%–78% of cases.[7] High cellularity is one of the predictors of recurrence in these tumors as suggested by Skullerud and Loken.[8]

Pravdenkova et al.,[9] observed that the expression of progesterone receptors (PRs) alone in meningiomas signifies a more favorable and biological outcome. They also found that either a lack of estrogen receptor and PR or the presence of estrogen receptors only in meningiomas correlated with a more aggressive clinical behavior, progression, and recurrence. In histopathological borderline cases, the PR status combined with a high proliferative index can provide more insight into the behavior of meningiomas with respect to recurrence. Gursan et al.,[10] have shown a strong inverse correlation between a high proliferative index and a negative PR status predicting recurrence in tumors with these features. A high recurrence is also noted in high-grade meningiomas. A substantial number of atypical and malignant meningiomas are mostly associated with a lack of expression of PRs.

The role of radiotherapy in the treatment of spinal meningiomas remains controversial because of the potential damage caused by radiation.[11] Gezen et al.,[12] have considered radiotherapy to be an adjuvant treatment in meningiomas with an early recurrence after total or subtotal resection.

Lumbosacral meningiomas are rare. As with other meningiomas, they occur more often in women than in men. A literature review of spinal meningiomas shows the thoracic spine to be the predominant location.[13] There is a low incidence in the lumbar spine. Similar results have been encountered by Solero et al.,[1] and Levy et al.[4] A critical review of 131 surgically treated cases of spinal meningioma by Erol et al.,[13] has reported only two cases of meningiomas in the lumbar spine.

To conclude, only very few cases of meningiomas of the lumbosacral spine have been reported in literature and a still lower incidence of atypical meningiomas of lumbosacral spine with recurrence exists. Atypical meningiomas have a higher recurrence rate, and complete excision of the tumor is crucial. A high cellularity, a negative PR status, and a high Ki-67 labeling index are predictors of an aggressive behavior and early recurrence.[14],[15] Radiotherapy is an adjuvant treatment in tumors with subtotal resection. This case is presented due to the rarity in its location and in a young male. Rapid recurrence of the tumor in this case despite the fact that a safe radical resection was achieved is also a surprising feature.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Solero CL, Fornari M, Giombini S, Lasio G, Oliveri G, Cimino C, et al. Spinal meningiomas: Review of 174 operated cases. Neurosurgery 1989;25:153-60.  Back to cited text no. 1
    
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Winn HR. Youmans and Winn Neurosurgical Surgery. Philadelphia, PA: Elsevier; 2011.  Back to cited text no. 2
    
3.
Lee JH. Meningiomas – diagnosis, treatment and outcomes. New York, Berlin, Heidelberg: Springer; 2011.  Back to cited text no. 3
    
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Levy WJ, Bay J, Dohn D. Spinal cord meningioma. J Neurosurg 1982;57:804-12.  Back to cited text no. 4
    
5.
Weber DC, Lovblad KO, Rogers L. New pathology classification, imagery techniques and prospective trials for meningiomas: The future looks bright. Curr Opin Neurol 2010;23:563-70.  Back to cited text no. 5
    
6.
Quekel LG, Versteege CW. “The dural tail sign” in MRI of spinal meningiomas. J Comput Assist Tomogr 1995;19:890-2.  Back to cited text no. 6
    
7.
Capodano AM. Nervous system tumors: Meningioma. Atlas Genet Cytogenet Oncol Haematol 2000.  Back to cited text no. 7
    
8.
Skullerud K, Loken AC. The prognosis of meningioma. Acta Neuropathol 1974;29:337-44.  Back to cited text no. 8
    
9.
Pravdenkova S, Al-Mefty O, Sawyer J, Hussain M. Progesterone and estrogen receptors: Opposing prognostic indicators in meningiomas. J Neurosurg 2006;105:163-73.  Back to cited text no. 9
    
10.
Gursan N, Gundogdu C, Albayrak A, Esref Kabalar M. Immunohistochemical detection of progesterone receptors and the correlation with Ki-67 labeling indices in paraffin embedded sections of meningioma. Int J Neurosci 2002;112:463-70.  Back to cited text no. 10
    
11.
Yoon SH, Chung CK, Jahng TA. Surgical outcome of spinal canal meningiomas. J Korean Neurosurg Soc 2007;42:300-4.  Back to cited text no. 11
    
12.
Gezen F, Kahraman S, Canakci Z, Beduk A. Review of 36 cases of spinal meningioma. Spine 2000;25:727-31.  Back to cited text no. 12
    
13.
Erol I, Anja H, Oliver M, Hischam B, Dietmar S, Siamak A. Spinal meningiomas: Critical review of 131 surgically treated patients. Eur Spine J 2008;17:1035-41.  Back to cited text no. 13
    
14.
Sah SK, Shi X, Sah SK, Yadav PK, Li Y. Pigmented intramedullary spinal cord meningioma mimicking a nervous system infection: An unusual report and review of the literature. Neurol India 2018;66:832-5.  Back to cited text no. 14
[PUBMED]  [Full text]  
15.
Shinde SV, Shenoy AS, Savant HV, Balasubramaniam SB. Coexistent intracerebral metastatic melanoma and meningioma. Neurol India 2017;65:110-2.  Back to cited text no. 15
[PUBMED]  [Full text]  


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



 

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