doi:10.1111/j.1750-3639.2010.00422.

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C A S E O F M O N T H M AY 2 0 1 0 bpa_422 987..988

35 YEAR-OLD MAN WITH FALCINE TUMOR

Eytan Raz1; Manila Antonelli2; Angelo Pichierri1; Arturo Consoli1; Felice Giangaspero2; Marco Fiorelli1
1
Department of Neurological Sciences—Sapienza University of Rome—Italy
2
Department of Pathology—Sapienza University of Rome—Italy

pathological examination is not routinely performed during

CLINICAL HISTORY AND
NEUROIMAGING
The patient is a previously healthy 35-year-old man who was admit-
ted to our hospital for evaluation after experiencing vomitus, dizzi-
ness and headache for one month. A neurological examination
elicited no abnormalities. CT and MR imaging were performed
and revealed a 3.5 ¥ 3.2 cm solitary, well-delineated, extra-axial
midline mass arising from the frontal falx cerebri. The lesion was
isointense to the cortex on T2 (Figure 1A) and hypointense on T1,
with a marked homogenous enhancement after intravenous contrast
administration (Figure 1B, 1C). Based on these findings, the radio-
logical diagnosis was meningioma of the falx.At surgery, the tumour
appeared as an extra-axial lesion and was removed via a left midline
frontal craniotomy. The excision was macroscopically complete.
MACROSCOPIC FINDINGS
Macroscopically, the surgical specimen was whitish, soft, well
circumscribed and measured 1.6 cm in diameter. Intraoperative
removal of meningiomas.
MICROSCOPIC FINDINGS
Microscopic features showed a neoplasm with high cellularity. The
cells had irregular nuclei, the majority of which were naked, and
in their midst were gemistocytic cells as well as pleomorphic
cells with vescicular nuclei and abundant, eosinophilic cytoplasm.
(Figure 2A). Mitotic figures were seen (3/10HPF), but without
necrosis or microvascular proliferation. These cells laid in a fibril-
lary background. On immunohistochimical examination, the neo-
plasm was reactive for glial fibrillary acidic protein (GFAP—
Figure 2B); MIB-1 index was about 15% (Figure 2C). P53 protein
was overexpressed (Figure 2D).
What is the diagnosis?

A B C

Figure 1.

A B

C D

Figure 2.

Brain Pathology 20 (2010) 987–988 987

© 2010 The Authors; Journal Compilation © 2010 International Society of Neuropathology
Correspondence
DIAGNOSIS
Primary intracranial solitary leptomeningeal astrocytoma (WHO
grade 3).
DISCUSSION
Primary intracranial solitary leptomeningeal astrocytoma (PLA) is
very rare; it usually arises in the leptomeninges of the brain or
spinal cord with no involvement of intraparenchymal tissue. PLA
represent nests of glial tissue separated from the bulk of the
CNS, which remain trapped in the leptomeningeal sheets, during
embryogenesis, following an aberrant migration (1, 4). This form
of astrocytoma differs from the more common secondary leptom-
eningeal astrocytoma, which refers to secondary spread from an
intra-axial glioma. PLA can present in two ways: a diffuse form
that is macroscopically similar to meningitis, and a localized soli-
tary form, that mimics other, more common, extra-axial lesions,
namely meningiomas.
Cooper and Kernohan criteria for a diagnosis of PLA are as
follows: no attachment of meningeal tumor to the brain, no evi-
dence of neoplasia within the brain, presence of leptomeningeal
encapsulation around the tumour. All three criteria were satisfied in
this case.
Concurrent adjacent meninigoma and astrocytoma has been
described (2), but a retrospective analysis of the removed piece
failed to detect any meningiomatous component of the lesion.
Fifteen cases of PLA are reported in the literature (3); the rarity
of this pathological entity make this diagnosis a challenge on a
neuroradiological basis. Notwithstanding, this challenge is of great
importance since the diagnosis of astrocytoma versus meningioma
implicates a notable difference both in prognosis and therapy. Ret-
rospective neuroradiological analysis of this case failed to detect
any findings to help in the differential diagnosis, thus confirming
the fundamental role of the neuropathologist even in what can
appear to be straightforward radiological diagnoses.
988
© 2010 The Authors; Journal Compilation © 2010 International Society of Neuropathology
REFERENCES
1. Cooper IS, Kernohan JW (1951) Heterotopic glial nests in the
subarachnoid space; histopathologic characteristics, mode of origin and
relation to meningeal gliomas. J Neuropathol Exp Neurol 10:16–29.
2. Davis GA, Fabinyi GC, Kalnins RM, Brazenor GA, Rogers MA (1995)
Concurrent adjacent meningioma and astrocytoma: a report of three
cases and review of the literature. Neurosurgery 36:599–604.
3. De Tommasi A, Occhiogrosso G, De Tommasi C, Luzzi S, Cimmino A,
Ciappetta P (2007) A polycystic variant of a primary intracranial
leptomeningeal astrocytoma: case report and literature review. World J
Surg Oncol 23(5):72.
4. Popoff N, Feigin I (1964) Heterotopic central nervous tissue in
subarachnoid space. Arch Pathol 78:533–539.
ABSTRACT
A 35-year-old man presented with one month history of vomitus,
dizziness and headache. CT and MR imaging revealed a
3.5 ¥ 3.2 cm solitary extra-axial midline mass arising from the
frontal falx cerebri; radiological findings were diagnostic of menin-
gioma of the falx. At surgery, the tumour appeared as an extra-axial
lesion and was removed via a left midline frontal craniotomy. Mac-
roscopically, the surgical specimen was whitish, soft, well circum-
scribed and measured 1.6 cm in diameter; microscopic features
showed a neoplasm with high cellularity, presence of mitotic
figures, without necrosis or microvascular proliferation; the neo-
plasm was reactive for glial fibrillary acidic protein and MIB-1
index was about 15%. Given the localization, microscopic features
were diagnostic of primary intracranial solitary leptomeningeal
astrocytoma (PLA), WHO grade 3. PLA is a very rare lesion that
arises in the leptomeninges of the brain or spinal cord with no
involvement of intraparenchymatous tissue. Fifteen cases of PLA
are reported in the literature. Retrospective neuroradiological
analysis of this case failed to detect any findings to help in the
differential diagnosis, thus confirming the fundamental role of the
neuropathologist even in what can firstly appear to be a straightfor-
ward radiological diagnosis.
Brain Pathology 20 (2010) 987–988