Medicine: A Rare Subtype of Meningioma
Medicine: A Rare Subtype of Meningioma
Medicine: A Rare Subtype of Meningioma
OPEN
Abstract
Rationale: Anaplastic meningioma, a rare subtype of meningioma, has malignant morphological characteristics and a World Health
Organization (WHO) grade of III.
Patient concerns: In this report, we present findings from 6 cases of anaplastic meningioma.
Diagnoses: Pathological examination of the tumors, including hematoxylin and eosin staining and immunohistochemical staining,
was performed. Of the six cases of anaplastic meningioma, two were recurrent tumors from original seminoma with a WHO grade of I.
Histologically, three cases had carcinoma-like morphology, one case had sarcoma-like morphology, and two had two kinds of tissue
structures: carcinoma-like tumor cell nests and areas with spindle tumor cells. Necrosis was detected in most cases (5/6). Ki67 index
was high and varied from 20% to 70%.
Interventions: All the patients received surgery. 3 patients received adjuvant radiotherapy. 1 patient received chemotherapy.
Outcomes: 4 patients had no recurrence at follow-up of 19, 30, 46 and 54 months after the last surgery. 1 patient had recurrence 3
months after the last surgery. 1 patient died 12 days after the last surgery.
Lessons: This malignant subtype can be secondary to a WHO grade I meningioma after a long quiescent period. Necrosis was
common in the tumor tissues, and Ki67 index was usually high. For patients with a history of meningioma, including benign cases,
regular physical examination is important for early detection of tumor recurrence and malignant transformation.
Abbreviations: CT = computed tomography, EMA = epithelial membrane antigen, HE = hematoxylin and eosin, MRI = magnetic
resonance imaging, WHO = World Health Organization.
Keywords: anaplastic meningioma, case series, meningioma
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2.1.3. Case 3. A 77-year-old male patient was referred to our 2.1.5. Case 5. A 91-year-old man had undergone surgery for a
hospital because he had developed weakness of the right limbs WHO grade I meningioma 20 years before he visited our
and memory impairment 20 days prior. The symptoms hospital. The tumor recurred 13 years prior, and he underwent a
progressively worsened. The patient had no headache, nausea, second surgery. He experienced sudden-onset convulsions
vomiting, or convulsions. 6 months prior, and repeated convulsions for 25 days before
his visit.
2.1.4. Case 4. The patient was a 33-year-old man with a
6-month history of right eyelid swelling and a 1-month history of 2.1.6. Case 6. The patient was a 65-year-old man with a 1-week
discontinuous headaches. CT performed in a local hospital history of weakness of the lower limbs, somnolence, and
revealed a right intracranial mass. He visited our hospital for dizziness, with symptom aggravation 3 days before he visited
further diagnosis and treatment. our hospital.
Figure 1. MRI and CT findings. A mass with short T1 and long T2 signals (A, B) was detected inside and outside the right frontotemporal skull plate. The mass was
closely attached to the adjacent frontotemporal bone, and invaded the right temporal bone and the lateral orbital wall. The mass was also detected by CT. It showed
slightly low density and invaded the right frontotemporal bone, sphenoid bone, and lateral orbital wall (C). A mass in the right frontal lobe near the frontal bone was
detected in another patient by MRI. It had a short T1 signal and invaded the right frontal bone. CT = computed tomography, MRI = magnetic resonance imaging.
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Figure 2. Gross features of the tumor. The mass, measuring about 4 cm 3.0 cm 2.5 cm, was cut into pieces. The surface of the tumor is rough, with adhesion
between the mass and the surrounding tissue (A). The cut surface is gray yellow, with relative tenacious texture (B).
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slightly low density, invading the right frontotemporal bone, Necrosis was detected in all cases except case 1 (C, E, I, N, O).
sphenoid bone, and lateral orbital wall (Fig. 1C). An MRI scan Large patchy necrosis was found in 4 cases: Cases 2, 3, 4, and 6.
showed a mass with short T1 signal in the right frontal lobe near
the frontal bone in Case 6 (Fig. 1D), invading the right frontal
4.3. Immunophenotype
bone. Figure 2 shows the gross tumor features of Case 6. A near
globular mass, measuring approximately 4 cm 3.0 cm 2.5 cm, Results of tumor immunostaining are shown in Figure 4. Tumors
was cut into pieces. The surface of the mass was focally smooth. from all 6 cases were positive for epithelial membrane antigen
A local adhesion was observed between the mass and the (EMA) and vimentin immunostaining, which indicated bi-
surrounding tissue, which had a rough surface (Fig. 2A). The cut directional tumor differentiation. EMA immunostaining was
surface of the mass was gray yellow, with a relatively tenacious generally focally positive and strong in 2 cases (Cases 2 and 6)
texture (Fig. 2B). and weak in 4 cases (Cases 1, 3, 4, and 5). Vimentin
immunostaining was generally diffuse and strong, except in
Case 3, where vimentin immunostaining was relatively weak. The
4.2. Microscopic features
Ki67 index varied from 20% to 70%, and was 30%, 70%, 20%,
Histopathological features of the tumors are listed in Figure 3. 30%, 50%, 60% in Cases 1–6, respectively (Fig. 4 and Table 1).
Tumor tissues of Case 1 (A) and Case 5 (L) were composed of 2
different areas: spindle cells and focal carcinoma-like cell nests.
5. Discussion
The tumor cells of Case 1 had large nucleoli (B) and those of Case
5 had medium-sized nucleoli (M). Tumor cells of Cases 2, 3, and Most anaplastic meningiomas occur in adults, although some
4 formed cell nests with carcinoma-like morphology (C, E, G), rare cases have been reported in children.[9] In the present study,
and the tumor cells had medium-sized nucleoli (D, F, H). The the patients were all adults, with ages ranging from 33 to 91
tumor cells of Case 4 invaded bond and muscle tissues (J, K). years. Anaplastic meningiomas are usually highly invasive and
Tumor cells of case 6 had sarcoma-like morphology and invaded grow rapidly.[10] Ahmeti et al[10] reported a case of an anaplastic
brain tissues (N). The tumor cells had medium-sized nucleoli (P). meningioma that destroyed most of the calvarial bone. In a case
Figure 3. Microscopic features of the tumors. Tumor tissues of Case 1 and 5 had 2 kinds of tumor cells: carcinoma-like cells and spindle cells (A, L). The tumor cells
in Case 1 had large nucleoli (B). Tumor cells of Cases 2, 3, and 4 formed nests with carcinoma-like morphology (C, E, G). Necrosis was observed in the tumor tissues
in 5 cases: Cases 2, 3, 4, 5, and 6 (C, E, I, L, and O, respectively). The tumor cells of Cases 2, 3, 4, 5, and 6 had medium-sized nucleoli (D, F, H, M, P). In Case 4, the
tumor cells invaded bone (J) and muscles (K). In Case 6, the tumor cells invaded brain tissues (N). (A, C, E, K, N, O: magnification 100; G, L, I, J: magnification
200; B, D, F, H, M, P: magnification 100).
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Figure 4. Results of tumor immunostaining. The tumor cells in all cases were positive for epithelial membrane antigen (EMA) and vimentin. EMA immunostaining
was focal and weak in Cases 1, 3, 4, and 5, and focal and strong in Cases 2 and 6. Vimentin immunostaining was generally diffuse and strong, with weak staining
only in Case 3. The Ki67 index was high in all cases: 30%, 70%, 20%, 30%, 50%, and 60% in Cases 1–6, respectively (magnification 200). EMA=epithelial
membrane antigen.
reported by Güngör et al,[11] a giant intracranial tumor invaded recurrence rates of 50% to 80%.[12] Kawahara et al[12] reported a
extracranial soft tissue and formed a cutaneous mass. Consistent rapid recurrence of this tumor < 2 months after surgery for
with these previous reports, the tumors in the current study removal of the primary tumor. However, distant metastasis of
were aggressive, invading brain, bone, and muscle tissues. The anaplastic meningioma is rare. In a study of 168 cases of atypical
recurrence of anaplastic meningioma is very common, with and anaplastic meningioma, Kessler et al[2] found that the rate of
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Table 1 the present study, EMA immunostaining in the tumor cells was
Histopathological features of the 6 cases of anaplastic menin- generally focal and weak in most cases (4/6).
gioma. Anaplastic meningioma can be a result of malignant
transformation of meningioma with a WHO grade of I or
Features
case Mitosis Necrosis Nucleolus Ki67 II.[26] Rammo et al[22] reported a meningioma progressing from
WHO grade I to III after 12 years. In the present study, 2 patients
1 >10/10HPF None Single, large 30% had a history of WHO grade I meningioma, supporting the
2 >20/10HPF Large patchy necrosis Single, medium sized 70%
possibility of malignant transformation.
3 >5/10HPF Large patchy necrosis Single, medium sized 20%
4 >1/10HPF Large patchy necrosis Single, medium sized 30%
5 >10/10HPF Small patchy necrosis Single, medium sized 50% 6. Conclusion
6 >1/10HPF Large patchy necrosis Single, medium sized 60%
Anaplastic meningioma is a malignant meningioma subtype with
malignant morphological features. Necrosis and a high Ki67
index are common features in this rare tumor subtype, which may
extracranial metastases was about 3%. Corniola et al[13] reported be secondary to a WHO grade I meningioma. Our findings
a case of anaplastic meningioma with pulmonary metastasis indicate that regular physical examination is important in
2 years after surgery, resulting in the patient’s death. In a case patients with a history of meningioma, including benign cases,
reported by Lambertz et al,[14] a patient with anaplastic to enable early detection of recurrence and malignant transfor-
meningioma developed multiple metastases in both lungs after mation of meningioma.
the first surgery; this patient also had liver metastases. Nishida
et al[5] reported an anaplastic meningioma in a very rare site, the
spinal cord, with subsequent metastasis to the mediastinal lymph Author contributions
nodes. Moubayed et al[15] also reported a case of anaplastic Conceptualization: Hongyi Cao, Biying Jiang, Chuifeng Fan.
meningioma metastasizing to the mediastinal lymph nodes. The Data curation: Hongyi Cao, Biying Jiang, Yang Zhao, Chuifeng
patient had a cervical lump, which was found to be a metastatic Fan.
anaplastic meningioma after removal of the primary intracranial Formal analysis: Yang Zhao.
tumor. The prognosis of anaplastic meningioma is generally poor Investigation: Biying Jiang, Yang Zhao.
because of frequent recurrence and occasional metastasis, and Resources: Hongyi Cao.
the median survival time is <2 years.[12,16] Supervision: Chuifeng Fan.
The primary treatments for anaplastic meningioma are Validation: Chuifeng Fan.
resection and adjuvant radiotherapy.[17] A study by Orton Writing – original draft: Hongyi Cao, Biying Jiang, Yang Zhao.
et al[7] indicated that adjuvant radiotherapy improved survival in Writing – review & editing: Chuifeng Fan.
patients with anaplastic meningioma. Another study by Garzon-
Muvdi et al[18] also indicated an association between radiother-
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