doi:10.1111/j.1750-3639.2009.00301.

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COM FEBRUARY CASE 1 b pa _ 3 0 1 531..533

5-YEAR OLD MALE WITH AN INTERHEMISPHERIC FRONTAL

MASS bpa_301 531..534

Laura Aón Bertolino, MD2; Pía Lépore, MD2; Mónica Carassai, MD2; Roberto González, MD3;
Gustavo Sevlever, MD, PhD1; Miguel A. Riudavets, MD1
1
Department of Neuropathology. Institute for Neurological Research, FLENI. Buenos Aires, Argentina.
2
Departments of Pathology, and 3 Neurosurgery. Hospital Churruca-Visca. Buenos Aires, Argentina.

CLINICAL HISTORY
A 1-year old male (with no significant past medical history) pre-
sented to the Department of Plastic Surgery for evaluation and
eventual resection of a subcutaneous frontal mass. Imaging of the
head revealed a second intracranial mass and he was referred to the
Department of Neurosurgery. Subsequently, Magnetic Resonance
Imaging (MRI) detected two T-1-hyperintense masses. One was the
extra-cranial frontal mass noted clinically and the other was an
intra-axial, interhemispheric frontal mass. Additionally, partial
agenesis of posterior corpus callosum was found. (Figure 1) At that
time, only the subcutaneous mass was resected and physical exami-
nation was normal. Four years later a follow-up MRI revealed
that the intracranial lesion had doubled in size (Figure 2). It was
hyperintense on both T1 and T2 (Figures 2 and 3). Craniotomy was
performed and a soft yellow mass was partially resected. Focal
attachment to the cingulate gyri was appreciated.
GROSS AND MICROSCOPIC
PATHOLOGY
Macroscopically, surgical fragments from both specimens were
yellow, lobulated and soft. Permanent sections from the extracra-
nial frontal mass are shown in Figure 4. Sections from the intracra-
nial lesion are shown in Figures 5, 6, 7 & 8. No normal cortex was
appreciated. Some areas were positive for GFAP (glial fibrillary
acidic protein). (Figure 9)

Figure 2.
Figure 1.

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Correspondence

Figure 5.

Figure 3.

Figure 6.

Figure 4.

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 Correspondence

Figure 7.

Figure 9.
Figure 8.

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Correspondence
DIAGNOSIS
Encephalic Lipoma (tubulonodular type) with neuroglial compo-
nent associated with partial agenesis of the Corpus Callosum. The
patient was discharged home without further deficit. Follow-up
MRI displayed remaining tumor in the location. The patient
remains under clinical observation for mass re-growth and other
systemic lesions.
DISCUSSION
Intracranial lipomas are infrequent Central Nervous System (CNS)
lesions with an incidence that ranges between 0.08 to 0.2% of
autopsied cases and between 0.06 to 0.3% among those found
during imaging, (3) but according to others this frequency can raise
up to 0.4%. (12) Although these lesions are considered grade I
mesenchymal, non-meningothelial tumors according to the World
Health Organization, (12) their origin is not clear and the idea of a
primary malformation is still accepted. In fact, their origin may
arise in the abnormal persistence and abnormal differentiation
of the primitive meninx. (5) These kind of lesions are more fre-
quently detected as a single entity, however, they can also be a
component of “encephalocraniocutaneous lipomatosis” a rare neu-
rocutaneous syndrome. (7)
By imaging studies intracranial lipomas are frequently located in
the midline, usually in the supratentorial compartment, and more
precisely they tend to be interhemispheric in the anterior corpus
callosum. (9, 12, 15) In fact, pericallosal lipomas as a single entity
are usually divided into two groups: curvilinear and tubulonodular;
(1, 2, 14, 15). The former are thin, posteriorly located in the corpus
callosum, and have low incidence of associated anomalies; (2,14).
On the other hand, tubulonodular lipomas are located anteriorly,
and are associated with a high incidence of facial defects and/or
encephaloceles. (14) On MRI, characteristically these lesions are
homogenous, and show hyperintensity on T1-weighted images and
hypo or isointensity on T2. (5)
Clinically patients can be asymptomatic, present with recurrent
headaches, (3) show mental retardation (8) or can present with
focal or generalized epilepsy, (4, 6) which has been regarded by
some as the most frequent presentation, (11) but this has more
recently been challenged. (13)
In our case, the patient presented with three lesions: a subcutane-
ous frontal lipoma independent of the cranial vault, an intracranial
tubulonodular lipoma mixed with neuroglial tissues arising in the
interhemispheric fissure, and partial agenesis of the posterior corpus
callosum. There was no history of mental retardation or epilepsy.
In terms of the histological assessment, this case showed typical
features of a mature lipoma intermingled with neuroglial tissue
imparted by the presence of neuropil showing reactive astrocytes
and mature neurons with glial satellitosis. Reports of lipomas
arising in that location with or without agenesis of the corpus
callosum are not abundant and most of the lesions described are
composed only of typical mature lipomatous tissue; (12) and these
lesions are usually sharply demarcated from cortex, (10) or but
others may show myelo-lipomatous differentiation (i.e., with
hematopoietic elements).
In conclusion, we report a case of an asymptomatic child with
partial agenesis of the corpus callosum and two lipomas, an extrac-
ranial one with mature lipomatous tissues only and a second intrac-
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ranial interhemispheric frontal mass with a mixture of mature
lipomatous and neuroglial tissues.
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ABSTRACT
We describe a 1 year-old male infant who presented to the Depart-
ment of Plastic Surgery with a subcutaneous frontal mass. Radio-
logical studies showed this to be an extracranial mass, but imaging
also demonstrated a second interhemispheric mass in the frontal
lobe as well as partial agenesis of the corpus callosum. Only the
frontal mass was resected at that time and it was a lipoma. At the
age of five, surgical excision of the second lesion was performed
since it had increased in size. The child had no history of seizures.
It showed lipomatous tissue with hyalinized connective areas inter-
mingled with a neuroglial component. The lesion was diagnosed as
an encephalic lipoma (tubulonodular type) associated with partial
agenesis of the corpus callosum. The patient is under clinical obser-
vation for mass re-growth and other systemic lesions.
Brain Pathology 19 (2009) 531–534