doi:10.1111/j.1750-3639.2009.00310.

x
COM JUNE 2009 CASE 2

A 58 YEAR OLD WOMAN WITH A CORPUS CALLOSUM

NODULE AT AUTOPSY bpa_310 743..744

Susanne K Jeffers, MD ; T. David Bourne, MDa; M. Beatriz S. Lopes, MD, PhDa

a

University of Virginia Health System, Department of Pathology, Charlottesville, VA.

HMB-45, and myogenin were negative. The remaining analysis of

CLINICAL HISTORY the brain was remarkable only for hypertensive vascular changes.
The patient was a 58-year-old African American woman with Of note, there was no evidence of other malformative lesions and
severe static encephalopathy and cerebral palsy both presumptively no signs of chronic hypoxic-ischemic damage.
related to prenatal/perinatal brain injury. Additional neurological
diagnoses included an ill-defined seizure history, bipolar affective
disorder, and medication-related tardive dyskinesia. The patient’s
other co-morbidities were non-contributory. She was a permanent
resident of a long-term care facility, where she expired unexpect-
edly. An autopsy was requested.

NEUROPATHOLOGY FINDINGS
The 1120 gram brain was grossly normal on external examination.
However, coronal sections demonstrated an ill-defined nodule
within the right side of the corpus callosum composed of heteroge-
neous areas of firm, white tissue and soft, yellow areas (Figure 1).
Microscopically, the nodule consisted of mature adipose tissue,
collagen, and blood vessels surrounded by callosal fibers
(Figure 2). Trichrome stain confirmed the presence of collagen
(Figure 2B). Immunohistochemical stains including SMA, Figure 1.

Figure 2.

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© 2009 The Authors; Journal Compilation © 2009 International Society of Neuropathology
Correspondence
DIAGNOSIS
Lipoma of the corpus callosum.
DISCUSSION
Intracranial lipomas are thought to be benign, slow-growing, con-
genital hamartomatous conditions, which are very rare (2). Since
the original description of these tumors in 1856 by Rokitansky (6),
approximately 200 cases have been reported (3). The frequency of
these lesions ranges from 0.46 to 1% of all intracranial tumors (10).
The most common location for these neoplasms is at or near the
midline. In the largest study of intracranial lipomas to date,
these tumors were found most frequently in the pericallosal-
interhemispheric region (45%), followed by the quadrigeminal
cistern (25%), and the suprasellar/interpeduncular cistern (14%)
(9).
The diagnosis of intracranial lipoma is commonly rendered in
the pediatric and young adult population, either incidentally on
neuroimaging or due to the symptoms they elicit which lead to
imaging studies. In older patients, intracranial lipomas are often
found incidentally at autopsy. Radiographically, a lipoma of the
corpus callosum is easily identified on plain skull films. Specifi-
cally, a radiolucent zone, which corresponds to the fatty tumor, is
surrounded by curvilinear calcifications (2). MRI demonstrates a
homogenous hyperintense mass on T1 weighted images and an iso-
to hypointense lesion on T2 weighted images (3). In addition,
CT scans and MRI are helpful in identifying other associated
malformations.
According to Gerber et al. (2), fifty percent of patients with
intracranial lipomas are asymptomatic. Common presenting symp-
toms include epilepsy, headache, behavioral changes, and cranial
nerve paralysis (10). However, the association between epileptic
seizures and intracranial lipomas remains controversial (2, 3).
Gastaut et al (1) reasoned that the symptomatic nature of corpus
callosum lipomas is dependent on the interruption of the callosal
fibers, which are replaced by the neoplasm. Such disconnection is
responsible for each hemisphere to develop epileptic discharges.
More specifically, EEG findings in case reports of patients with
callosal lipomas and epilepsy found seizure foci located in the
temporal lobe. The details of this association remain unclear (2).
Unfortunately in the present case we were unable to retrieve and
review any EEG performed on the patient. However, we believe
that the corpus callosum lipoma in this autopsy case may have been
a contributing factor to the patient’s history of seizures.
The precise etiology of intracranial lipomas is still a topic of
discussion. Historically, theories regarding the histogenesis of
these lesions include: a) hypertrophy from pre-existing fatty tissue
of the meninges, b) metaplastic meningeal connective tissue, c)
heterotopic malformation of dermal origin, and d) tumor-like mal-
formation derived from primitive meninx (7). Another theory pro-
poses that lipomas are midline defects due to the improper closure
of the neural tube. This theory is supported by case reports in which
lipomas are associated with other midline defects such as spina
bifida and meningomyeloceles (4). The school of thought that sug-
gests that lipomas are the result of meningeal maldifferentiation
proposes that lipomas are linked genetically to other midline
defects (11). Other features associated with the presence of intrac-
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© 2009 The Authors; Journal Compilation © 2009 International Society of Neuropathology
ranial lipomas include agenesis of the corpus callosum (most
common), webbed toes, cleft lip, mongolism, funnel chest, facial
asymmetry, high arched palate, ventricular septal defect, agenesis
of the cerebellar vermis, pituitary tumors, acoustic schwannomas,
and other intracranial lipomas most commonly located in the
choroid plexus of the lateral ventricles (1). Today, intracranial
lipomas are accepted to be due to the abnormal differentiation of
the persistent meninx primitiva, which is a region that constitutes
the inner level of the pia, arachnoid, and dura (10).
Macroscopically, lipomas vary in size from subcentimeter
nodules to large masses. They have a bright yellow, lobulated
appearance. Microscopically, lipomas consist of mature adipose
tissue with varied amounts of collagen, blood vessels, and
calcifications (2, 5).
Anticonvulsant therapy is the treatment modality of choice in
symptomatic lipomas. Surgical management proves to be challeng-
ing due to the high vascularity and adherence of the lesion to the
surrounding parenchyma and should only be pursued if the patient
fails medical management (2, 10).
REFERENCES
1. Gastaut H, Regis H, Gastaut JL, Yermenos E, Low MD (1980)
Lipomas of the corpus callosum and epilepsy. Neurology 30:132–138.
2. Gerber S, Plotkin R (1982) Lipoma of the corpus callosum. Journal of
Neurosurgery 57:281–285.
3. Loddenkemper T, Morris HH, Diehl B, Lachhwani DK (2006)
Intracranial lipomas and epilepsy. Journal of Neurology
253:590–593.
4. Patel, AN (1965) Lipoma of the corpus callosum: a nonsurgical
entity: nosology, diagnosis, management. North Carolina Medical
Journal 26: 328–335.
5. Paulus W, Scheithauer BW, Perry A (2007) Mesenchymal,
non-meningothelial tumours. World Health Classification of Tumours.
IARC. 4th edition,173–174.
6. Rokitansky C. (1856) Lehrbuch der Pathologischen Anatomie,
Vienna, Braumuller, 468–478.
7. Sukthomya C, Menakanit V (1973) Lipoma of the nervous system.
Australasian Radiology 17:256–260.
8. Takeya, T, Hamano K,Takita H (1995) Corpus callosum lipoma and
complex partial seizures. Brain & Development 17:365–366.
9. Truwit CL, Barcovich AJ (1990) Pathogenesis of intracranial lipoma:
an MRI study in 42 patients. American Journal of Radiology
38:1031–1035.
10. Yilmaz N, Unal O, Kiymaz N, Yilmaz C, Etlik O (2006) Intracranial
lipomas—a clinical study. Clinical Neurology and Neurosurgery
108:363–368.
11. Zettner A, Netsky MG (1960) Lipoma of the corpus callosum.
Journal of Neuropathology & Experimental Neurology 19:305–319.
ABSTRACT
Intracranial lipomas are uncommon benign mesenchymal tumors,
found usually near or at the midline. The existence of such intracra-
nial tumors has been documented in the literature in only over
200 cases. Although usually asymptomatic, they can sometimes
trigger neurological symptoms, specifically epileptic seizures. We
describe the incidental finding of a lipoma of the corpus callosum
at autopsy in a 58 year-old woman with a history of seizures, and
provide a concise review of the pertinent literature with respect to
this entity.
Brain Pathology 19 (2009) 743–744