8/7/2019 0209 Case 1

1/4

C O M F E B R U A R Y C A S E 1 b p a _3 0 1 5 3 1 ..5 3 3

5-YEAR OLD MALE WITH AN INTERHEMISPHERIC FRONTAL

MASSbpa_301 531..534Laura An Bertolino, MD2; Pa Lpore, MD2; Mnica Carassai, MD2; Roberto Gonzlez, 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 revealedthat the intracranial lesion had doubled in size (Figure 2). It was

hyperintense on bothT1 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 MICROSCOPICPATHOLOGY

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 fibrillaryacidic protein). (Figure 9)

Figure 1.

Figure 2.

doi:10.1111/j.1750-3639.2009.00301.x

531Brain Pathology 19 (2009) 531534

2009 The Authors; Journal Compilation 2009 International Society of Neuropathology

8/7/2019 0209 Case 1

2/4

Figure 3.

Figure 4.

Figure 5.

Figure 6.

Correspondence

532 Brain Pathology 19 (2009) 531534

2009 The Authors; Journal Compilation 2009 International Society of Neuropathology

8/7/2019 0209 Case 1

3/4

Figure 8.Figure 9.

Figure 7.

Correspondence

533Brain Pathology 19 (2009) 531534

2009 The Authors; Journal Compilation 2009 International Society of Neuropathology

8/7/2019 0209 Case 1

4/4

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 acomponent 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 andhypo 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 patientpresented with three lesions: a subcutane-

ous frontal lipoma independent of the cranial vault, an intracranial

tubulonodular lipoma mixed with neuroglial tissues arising in the

interhemisphericfissure, and partialagenesis 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 astrocytesand 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-

ranial interhemispheric frontal mass with a mixture of mature

lipomatous and neuroglial tissues.

REFERENCES

1. Alzoum M, Alorainy IA, Husain M, Al Ruhainmi K (2002) Multiple

pericallosal lipomas in two siblings with frontonasal dysplasia. Am J

Neuroradiol23:7301.

2. Demaerel P, Van de Gaer P, Wilms G, Baert AL (1996)Interhemispheric lipoma with variable callosal dysgenesis:

relationship between embryology, morphology, and symptomatology.

Eur Radiol6(6):9049.

3. Donati F, Vassella F, Kaiser G, Blumberg A (1992) Intracranial

lipomas. Neuropediatrics 23(1):328.

4. Gastaut H, Regis H, Gastaut JL, Yermenos E, Low MD (1980)

Lipomas of the corpus callosum and epilepsy. Neurology

30(2):1328.

5. Gmez-Goslvez F, Menor-Serrano F, Tllez de Meneses-Lorenzo M,

AleuPrez-Gramunt M, Sala-Snchez AG, Rubio-SorianoA,

Carbonell-Nadal J, Mulas F (2003) Lipomas intracraneales en pedi-

atra: estudio retrospectivo de 20 pacientes.Rev Neurol37(6):51521.

6. Guye M, Gastaut JL, Bartolomei F (1999) Epilepsy and perisylvian

lipoma/cortical dysplasia complex. Epileptic Disord1(1):6973.

7. Haberland C, Perou M (1970) Encephalocraniocutaneous lipomatosis.Arch Neurol22:14455.

8. Hayashi T, Shojima K, Yamamoto M, Hashimoto T, Fukuzumi A,

Honda E (1983) Intracranial lipomasreport of six cases. No To

Shinkei 35(3):25768.

9. Ichikawa T, Kumazaki T, Mizumura S, Kijima T, Motohashi S,

Gocho G (2000) Intracranial lipomas: demonstration by computed

tomography and magnetic resonance imaging. J Nippon Med Sch

67(5):38891.

10. Kakita A, Inenaga C, Kameyama S, Masuda H, Ueno T, Honma J,

Shimohata M, Takahashi H (2005) Cerebral lipoma and the

underlying cortex of the temporal lobe: pathological features

associated with the malformation. Acta Neuropathol109(3):33945.

11. Kazner E, Stochdorph O, Wende S, Grumme T (1980) Intracranial

lipoma. Diagnostic and therapeutic considerations. J Neurosurg

52(2):23445.12. Kleihues P, Cavenee WK (2000) Pathology and Genetics of Tumours

of the Nervous System, IARCPress: Lyon.

13. Loddenkemper T, Morris HH 3rd, Diehl B, Lachhwani DK. (2006)

Intracranial lipomas and epilepsy. J Neurol253(5):5903.

14. Tart R, Quisling RG (1991) Curvilinear and tubulonodular varieties of

lipoma of the corpus callosum: an MR and CT study. J Comput Assist

Tomogr 15(5):80510.

15. Truwit C, Barkovich AJ (1990) Pathogenesis of intracranial lipoma:

an MR study in 42 patients. AJR Am J Roentgenol155(4):85564.

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 imagingalso 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.

Correspondence

534 Brain Pathology 19 (2009) 531534

2009 The Authors; Journal Compilation 2009 International Society of Neuropathology