J Korean Soc Geriatr Neurosurg > Volume 18(2); 2022 > Article
Ryu, Lee, Lee, Suh, Lee, and Cho: A rapidly growing intraparenchymal schwannoma in a geriatric patient: a case report and literature review

Abstract

Intraparenchymal schwannomas are extremely rare intracranial tumors. Although their occurrence has been reported in various age groups, they are most frequently seen in individuals under 30 years of age. A 65-year-old woman was brought to the emergency department with impaired consciousness due to spontaneous intracerebral hemorrhage in the right basal ganglia (BG). Approximately 3 months later, the patient was taken to another hospital due to decreased consciousness, and a subsequent brain computed tomography scan showed a mass-like lesion in the right BG. The tumor was removed, and the biopsy result revealed an intraparenchymal schwannoma with no involvement of the ventricular ependymal lining. An immunohistochemical analysis revealed a high Ki-67 labeling index, indicating rapid growth without malignancy.

Introduction

Intracranial schwannomas account for approximately 5% to 8% of primary intracranial tumors and most commonly originates from the 8th cranial nerve [1]. In fact, intracranial intraparenchymal schwannoma (IS) that are not derived from CN VIII have rarely been reported. Since the initial report by Gilbson et al. in 1966, only approximately 150 cases have been reported [2]. This type of schwannoma is slow growing and typically observed in young people under the age of 30; however, symptoms can manifest quickly in old age [3].

Case Report

A 65-year-old woman presented to the emergency department with impaired consciousness and underwent an emergency burr hole procedure for management of spontaneous intracerebral hemorrhage (ICH) with intraventricular hemorrhage in the right basal ganglia (BG) (Fig. 1). The ICH biopsy revealed a hematoma, not specific. The patient received treatment for hypertensive ICH and was transferred to another hospital. Approximately 3 months later, she was brought to another hospital due to decreased consciousness, subsequently, a brain computed tomography (CT) was performed which revealed a right BG mass-like lesion and was transferred to this hospital for further examination. The patient was in stupor at the time of discharge from the first hospitalization; however, she recovered well, apart from minor concerns of drowsiness and left hemiparesis.
A previously performed CT scan showed a 62×45 mm irregular mass with heterogeneous enhancement and approximately 14 mm midline shift (Fig. 2). In the brain magnetic resonance imaging performed later at our hospital the T1 weighted image (T1WI) showed slight gray matter hypointensity, and the T2 weighted image (T2WI) showed peritumoral edema and heterogeneously hyperintense lesions, while contrast-enhanced T1WI revealed intense ring enhancement of the mass. Based on these findings, the lesion was suspected to be glioblastoma multiforme (GBM).
Therefore, the patient underwent a craniotomy and tumor removal. Following right frontotemporoparietal craniotomy and dural incision, the mixed friable and hard, grayish, poorly demarcated, marginated mass (4.5×5×4 cm) was accessed using the transcortical approach (Fig. 3). It was then removed using a cavitron ultrasonic surgical aspirator since there was no involvement of the ependymal lining of the ventricles. Further, no sign of necrosis along with vascular proliferation was observed by analysis of the frozen biopsy sample, leading to a suspicion of glioma.
Upon histopathological examination, the cellular lesions consisted of a bundle of spindle cells arranged in a storiform pattern with palisading arrangement; however, no nuclear pleomorphism was seen. In addition, mild infiltration was of the mass into the brain parenchyma surrounding the tumor cells was observed, indicating the presence of a neurogenic tumor due to ovoid to spindle and partially wavy or buckled shaped cells. Immunohistochemical staining showed strong expression of both S-100 and glial fibrillary acidic protein (GFAP), while Ki-67 labeling index was 39.9% and the mitotic count was 3-4/10 high-power filed (Fig. 4).

Discussion

IS is an extremely rare intracranial tumor. Despite being prevalent in various age groups, from the youngest case being of a patient aged 6 months to the oldest case one being 84 year-old, it frequently occurs in individuals under 30 years of age (58.7%). Of these, 12.0% (18) of patients were over 60 years of age, which is unexpected. The incidence was higher in men in the age group under 30 years (male:female; 1.84:1); whereas, the pattern was reversed in the group above 60 years of age (male: female; 0.46:1) (Table 1) [4-8].
IS may originate anywhere in the intracranial region; however, appears to be more frequent in the supratentorial region during young age, whereas it is commonly located in the infratentorial region in individuals >60 years of age [4]. Although the pathophysiology of IS has not yet been clearly identified, 2 hypotheses have been proposed: (1) developmental theory, (2) non-developmental theory. The developmental theory assumes that mesenchymal pial cells in the brain parenchyma transform into Schwann cells. Alternatively, the non-developmental theory assumes that Schwann cells originate from adjacent organs, such as the meningeal branch of the perivascular nerve plexus and anterior ethmoidal and trigeminal nerves, where Schwann cells usually exist [9]. At present, it unclear clear which of these 2 hypotheses prevails.
The radiologic features of IS include cyst formation, calcification, and peritumoral edema, and it is difficult to find distinct differences from those of vestibular schwannomas [5,10]. However, only one similar case with intratumoral hemorrhage has reported so far, and even in vestibular schwannoma, the features shown in this case is known to be very rare, accounting for about 0.4% [6,11].
The diagnosis of intraparenchymal schwannoma by imaging is challenging, and is dependent on histopathological analysis and immunohistochemical findings. The histopathological appearance was characterized by Antoni A and B cells, while immunohistochemical staining showed positive expression of S-100 whereas epithelial membrane antigen and GFAP were absent in most cases [4,7].
Since intraparenchymal schwannoma presents with slow proliferation, Ki-67 labeling index is usually observed at less than 1% [1]. In this case, high Ki-67 labeling index was observed, indicating rapid growth, similar to a malignant intracerebral nerve sheath tumors (MINST). MINST is termed malignant peripheral nerve sheath tumors (MPNST) of brain parenchyma. MINST’s characteristics is similar to MPNST’s one except not associated of neurofibromatosis type Ⅰ. In histologically, MINST has pleomorphic cells with irregular nuclei. And that tumor also showed poorly differentiated malignant spindle cell. MINST is extremely rare, only one case was reported in the age group above 60 years [8]. The malignancy rate was approximately 5% in the age group above 60 years, which was slightly lower compared to other age groups [4].
IS are mostly benign tumors and show a good prognosis after total resection [1]. It is crucial to set an accurate surgical goal in the early stages of IS treatment for better clinical outcomes; however, there are certain limitations in the diagnosis of IS since it is difficult to differentiate from GBM or high-grade gliomas based on the imaging findings. Thus, hematoxylin and eosin staining is the only feasible option for examination of intraoperative frozen biopsy [7].

Conclusion

IS is a benign lesion characterized by slow growth in most cases and presents a good prognosis when treated with total resection. To the best of our knowledge, this is the first case of IS characterized by a rapid growth rate without evidence of malignancy. Since it is difficult to rule out GBM in the early stages, gross total resection and short-term follow-up will be required for management of these tumors.

NOTES

No potential conflict of interest relevant to this article was reported.

Fig. 1.
(A) Initial computed tomography (CT) scan showed intracerebral hematoma in the right basal ganglia. (B) The brain CT after 3 weeks revealed hematoma resolution and no mass like lesion. (C, D) Three months later brain CT revealed a large mass at right basal ganglia with rim enhancement. Written informed consent was obtained for publication of this case report and accompanying images.
jksgn-2022-00115f1.jpg
Fig. 2.
Brain magnetic resonance imaging revealed peritumoral edema and a heterogeneously hyperintense lesion on T2-weighted image (A) and slight hypointense to grey matter on T1-weighted image (T1WI) (B). On the contrast-enhanced T1WI showed ring enhancement of the mass (C). Written informed consent was obtained for publication of this case report and accompanying images.
jksgn-2022-00115f2.jpg
Fig. 3.
Intraoperatively, a mixed friable and hard, grayish mass (star) was confirmed. Written informed consent was obtained for publication of this case report and accompanying images.
jksgn-2022-00115f3.jpg
Fig. 4.
(A) The tumor shows a storiform pattern of spindle cells (H&E, ×100). (B, C) The tumor cell nuclei and cytoplasm are diffusely positive for S-100 protein and GFAP (×100). (D) The Ki-67 labeling is 39.9% (×100). Written informed consent was obtained for publication of this case report and accompanying images.
jksgn-2022-00115f4.jpg
Table 1.
Radiological and immunohistochemical features of intraparenchymal schwannoma in geriatric patients above 60 years old
No. Study Year Sex Age (yr) Location S-100 GFAP EMA Malignancy Cystic Calcification Hemorrhage Ki-67 (%)
1 Ghatak et al. 1975 Female 63 Parietal n/a n/a n/a × n/a n/a × ×
2 Solomon et al. 1987 Male 69 Medulla Negative n/a n/a × × × ×
3 Cervoni et al. 1988 Female 61 P-O n/a n/a n/a × n/a n/a × ×
4 Wilberger 1989 Female 62 Pituitary n/a n/a n/a × n/a n/a × ×
5 Tran-Dinh et al. 1991 Female 64 Cbll., brainstem n/a n/a n/a × × × ×
6 Casadei et al. [6] 1993 Female 79 Cbll. Positive Positive Positive × × × ×
7 Casadei et al. [6] 1993 Female 84 Temporal Positive Positive Positive × × × ×
9 Singh et al. 1993 Female 61 Cbll. Positive Negative n/a × × × ×
10 Weiner et al. 1993 Male 61 Brainstem n/a n/a n/a × × × ×
11 Weiner et al. 1993 Female 78 Brainstem Positive Negative Negative × × × ×
12 Ranjan et al. 1996 Female 65 Cbll. Positive Negative Negative × × × × ×
13 Tanabe et al. 1996 Female 68 Pons Positive Negative Negative × × × ×
14 Muzzafar et al. [5] 2010 Male 68 Brainstem n/a n/a n/a × × ×
15 Barnard et al. [8] 2011 Female 75 Frontal Positive Negative Negative × × ×
16 Khoo and Taki [7] 2012 Male 60 Frontal n/a Negative Negative × × × × <1
17 Luo et al. 2013 Male 72 P-O Positive Negative n/a × n/a n/a × ×
18 Luo et al. 2013 Male 64 Cbll. Positive Negative n/a × n/a n/a × ×
19 Anselmi et al. 2021 Male 74 Pons Positive Negative n/a × × ×
20 Present case 2020 Female 65 Basal ganglia Positive Positive Positive × × × 39.9

GFAP, glial fibrillary acidic protein; EMA, epithelial membrane antigen; P-O, parieto-occipital; Cbll., cerebellum; n/a, not available.

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