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Clinical Neurology and Neurosurgery 110 (2008) 416419

Case report

Histologically benign intraventricular meningioma with concurrent


pulmonary metastasis: Case report and review of the literature
Daniel H. Fulkerson a, , Terry G. Horner b,1 , Eyas M. Hattab c,2
aDepartment of Neurosurgery, Indiana University School of Medicine, 545 Barnhill Drive, Emerson Hall, Suite #139,
Indianapolis, IN 46202, United States
b Indianapolis Neurosurgical Group, 1801 N. Senate Boulevard, Suite 535, Indianapolis, IN 46202, United States
c Department of Pathology and Laboratory Medicine, Indiana University School of Medicine, Immunohistochemistry Laboratory,
Clarian Pathology Laboratory, 350 W. 11th Street, Room 4040, Indianapolis, IN 46202, United States
Received 20 August 2007; received in revised form 19 December 2007; accepted 25 December 2007

Abstract
Only 12% of all meningiomas are intraventricular in location. Metastasis from a histologically benign meningioma is a rare, but well-
documented event. However, there are only four reported cases in the literature of metastatic spread from a purely intraventricular meningioma.
The tumors in these reports had a frankly malignant histology or were associated with surgical manipulation and recurrence of the primary
lesion. In this report, the authors present a rare case of the concurrent presentation of a histologically benign intraventricular meningioma and
a solitary lung lesion which proved to be metastatic meningioma.
2008 Elsevier B.V. All rights reserved.

Keywords: Meningioma; Benign; Intraventricular; Metastasis

1. Introduction 2. Case report

Meningiomas are generally benign intracranial tumors. The patient is a non-smoking 54-year-old male who
Only an estimated 0.1% metastasizes [10]. Meningiomas presented with a generalized seizure. Magnetic resonance
located exclusively within the ventricles are also rare, com- imaging (MRI) showed a right contrast-enhancing intraven-
prising 12% of the total. Tumors with malignant histological tricular tumor with surrounding edema (Fig. 1). The patient
features have a higher rate of recurrence and metastasis; had an unremarkable past medical history with no previous
however, there are reports of metastasis from benign seizures. His neurological exam was normal.
meningiomas. There are very few reports of metastasis of A pre-operative computed tomography (CT) scan of the
intraventricular meningiomas. All previously published cases chest demonstrated a 2 cm, well-circumscribed right lung
of metastatic spread occurred in tumors with histologically lower lobe mass (Fig. 2). A fine needle aspiration of the
malignant features or recurrence of the primary. The authors lung mass was performed and initially interpreted as poorly
present a case of a patient with an intraventricular menin- differentiated non-small cell carcinoma.
gioma with benign histology and a concurrently discovered The intraventricular tumor was then excised via a right
metastasis in the lung. parietal-occipital craniotomy. The tumor was extremely
fibrous. It was contained in the ventricle and had no
Abbreviations: WHO, World Health Organization; EMA, epithelial gross brain invasion. Pathological examination revealed a
membrane antigen; CT, computed tomography; MRI, magnetic resonance meningioma with both a meningothelial and fibroblastic com-
imaging. ponents (Fig. 3). There was marked collagen deposition.
Corresponding author. Tel.: +1 317 274 5723; fax: +1 317 274 7351.
Conforming to a World Health Organization (WHO) grade
E-mail addresses: dfulkers@iupui.edu (D.H. Fulkerson),
THorner@ing.md (T.G. Horner), ehattab@iupui.edu (E.M. Hattab).
I meningioma, this tumor lacked atypical features, had a
1 Tel.: +1 317 396 1300; fax: +1 317 924 8472. mitotic count of less than one per ten high power fields, and
2 Tel.: +1 317 491 6363; fax: +1 317 491 6419. was characterized by a low (23%) Ki-67 labeling index.

0303-8467/$ see front matter 2008 Elsevier B.V. All rights reserved.
doi:10.1016/j.clineuro.2007.12.025
D.H. Fulkerson et al. / Clinical Neurology and Neurosurgery 110 (2008) 416419 417

Fig. 1. MR axial and coronal T1 with contrast views showing enhancing, intraventricular mass.

Eighteen days later, a thoracotomy was performed for pre-


sumed lung cancer. The pathological specimen was again
characteristic of meningioma demonstrating a predominantly
transitional morphology (Fig. 4). Similar to its intracranial
counterpart, this tumor lacked atypical features, had a mitotic
count of one per ten high powered fields and a low (23%)
Ki-67 labeling index consistent with a WHO grade I menin-
gioma. The tumor was positive for epithelial membrane
antigen (EMA) and progesterone receptor further confirm-
ing the diagnosis of meningioma. It was negative for CD 34
and Factor XIIIa. All resected thoracic lymph nodes were
negative for malignancy.
The patient recovered well from both surgeries. There has
been no evidence of recurrence in MRI or positron emission
tomography (PET) for over 1 year after surgery.

Fig. 3. Photomicrographs of the intracranial specimen showing a char-


acteristic transitional pattern (A) and a significant fibroblastic collagen
deposition (B) indicative of fibrous meningioma. H&E, original magnifica-
Fig. 2. CT scan of chest with arrow indicating a well-circumscribed, solitary
tion 25.
mass.
418 D.H. Fulkerson et al. / Clinical Neurology and Neurosurgery 110 (2008) 416419

reported age range for metastasis is 1 week to 83 years,


with 75% of patients between 40 and 60 years old [30]. The
location of meningioma metastases depends on the route of
dissemination. Most spread is hematogenous, especially in
tumors that invade dural sinuses. The most common extracra-
nial location of metastasis is the lungs, involved in 60% of
patients [14,32]. Other sites include liver and abdominal vis-
cera, lymph nodes, and bones [26,29]. There are rare reports
of metastases to the kidney, bladder, thyroid, breast, thymus,
heart, skin, vulva, adrenal gland, and ocular fundus [1]. A
second route of dissemination is through the cerebrospinal
fluid, leading to tumor within the neuraxis [8]. Approximately
75% of patients with metastasis from either route had previ-
ous surgery on the primary lesion [26]. It has been theorized
that surgical manipulation releases tumor from its normally
cohesive state into the bloodstream or cerebrospinal fluid.
However, spread remains a rarity, despite the relatively large
number of surgical resections.
The discovery of metastasis often occurs after recurrence
of the primary tumor. The 5-year recurrence rate for menin-
giomas with benign histological features is approximately 3%
[28]. However, the recurrence rate is 3878% for tumors with
atypical characteristics including nuclear pleomorphism,
necrosis, elevated mitotic activity, loss of whorl formation
or sheeting, small cell change, macronucleoli, brain invasion
and complex karyotypes [6,7,2224,27]. Tumors with clearly
malignant features have a higher metastatic rate [12]. Certain
histological subtypes are inherently prone to recur or spread,
including the WHO grade II clear cell and chordoid vari-
Fig. 4. Photomicrographs from the lung lesion showing an H&E of transi- ants and the WHO grade III rhabdoid and papillary variants.
tional meningioma morphology similar to the intracranial tumor (A) and a
Of these, the papillary variant carries the highest risk of late
positive EMA staining (B). Original magnification 40.
distant metastases [5,18,21,28].
Unfortunately, the clinical behavior of meningiomas does
3. Discussion not always correlate with histological features. Despite the
incidence of up to 9% of histologically malignant features
Meningiomas are generally benign, slow growing lesions in meningiomas, only about 0.1% shows metastases [10,28].
that comprise 1326% of intracranial tumors. Their origin is There are reports of metastases in tumors with a benign
from meningothelial cells found in arachnoid villi, arachnoid histology or subtype [2,6,9,17,20,24,28,30]. In Kepes review
membrane, tela choroidea and choroid plexus [5]. These last of all metastatic meningiomas reported through 1982, 4 of the
two locations explain the rare occurrence of purely intraven- 85 cases had completely benign morphology [15].
tricular tumors, which account for approximately 12% of all The number of purely intraventricular meningiomas with
meningiomas [3,11]. Intraventricular meningiomas are more metastasis is decidedly small. We were able to find only
prevalent in patients with neurofibromatosis and in children four other case reports [13,16,25,31]. The cases presented
[3]. by Strenger et al. and Kamiya et al. were tumors with frankly
There is no pathognomonic constellation of signs or malignant histological features including hypercellularity,
symptoms for intraventricular meningiomas. Non-specific high mitotic index, pleomorphisms, and invasion of the brain.
headache is the most common symptom and is present in The case presented by Kleinschmidt-DeMasters and Avakian
approximately 80% of patients. Other symptoms include was a tumor with rapid local recurrence, three excisions over
vision and gait disturbances, memory difficulty, loss of 12 months, and dissemination throughout the neuraxis. All
energy, and cognitive changes [4,19]. Seizures are the pre- these cases had metastatic spread via the cerebrospinal fluid
senting symptom in 27% [11]. after prior surgery [13,16,31]. The last case presented by
Meningiomas rarely metastasize. The male to female ratio Ramakrishnamurthy et al. was a tumor with benign histol-
of metastasis is 3:2, despite the fact that meningiomas are ogy; however, spread through the spinal fluid was found after
24 times more likely to occur in females [30]. The time recurrence of the primary [25].
from diagnosis of the primary tumor to discovery of metas- Our case is unique in the fact that the intraventricular
tasis is approximately 6 years in previous series [17]. The tumor had a benign histology, spread presumably by the
D.H. Fulkerson et al. / Clinical Neurology and Neurosurgery 110 (2008) 416419 419

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