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A giant interhemispheric lipoma and corpus callosum agenesis.

Byline: Erhan Karaaslan, Mustafa Ozcetin and Ramazan Tetikcok

The lipomas of the central nervous system are rare lesions and are often located in the midline and in the corpus callosum. They are rarely symptomatic and account for 0.06-0.46 % of intracranial lesions. We report a 5 year old girl who 'was admitted to the pediatrics clinic due to an intractable complex partial seizure. Biochemical tests for seizure etiology did not reveal any specific cause. Electroencephalography (EEG) showed minimal epileptic activity. Magnetic resonance imaging (MRI) showed that there was a 50x36 mm in diameter mass, and corpus callosum agenesis. Her seizures were controlled with carbamazepine (10mg/kg/day). Although intrahemispheric lipomas are rare, they should be considered in the differential diagnosis of non-febrile seizures in children.

KEY WORDS: Intrahemispheric lipoma, Corpus callosum agenesis, Children.

How to cite this article:

Karaaslan E, Ozcetin M, Tetikcok R. A giant interhemispheric lipoma and corpus callosum agenesis. Pak J Med Sci 2011;27(4):948-949

INTRODUCTION

Lipomas are the most common benign tumors of the adipose tissue. Although these tumors are frequently observed elsewhere on the body, lipomas are rare benign tumors of the central nervous system that accounts for 0.06-0.46% of intracranial tumors.1 The lesions are often asymptomatic; they are frequently diagnosed incidentally during radiological investigations or at autopsy.2 In this report management of a child presenting with the diagnosis of a giant interhemispheric lipoma and corpus callosum agenesis is discussed with the relevant literature.

CASE REPORT

A five years old female child was admitted to pediatric emergency clinic with the complaint of intractable partial seizures. On physical examination there was no other motor and sensory deficit, except anisocoria (i.e. left pupil minimally midratic). Systemic examination was unremarkable. Routine hematologic and biochemical analysis were within normal limits. The sleep electroencephalogram (EEG) demonstrated that there were isolated sharp spiked slow waves and minimal abnormal epileptic activity. On cranial MRI, posterior and splenium of corpus callosum were not present; in addition there was 50x36 mm hyperdense lipomatous mass at the anterior of corpus callosum on T1-weighted images. Bilateral anterior cerebral arteries were coursing through the mass and were giving their branches within the lipoma (Figure-A, B). The patient was examined in the neurosurgery and ophthalmology department. On ophthalmic examination there were neither papilledema nor visual field defects but left pupil was anisocoric.

The patient was treated with carbamezepime (10mg/kg/day), her seizures were controlled.

DISCUSSION

Intracranial lipoma was first described in 1856 by Rokitansy after a necropsy but diagnosis of the lesion in living patient was not made until 1939.3 There is no sex predilection but cerebellopontine angle lipomas appear twice as common in males.4

Although the etiology and pathogenesis of intracranial lipomas are nor clearly understood, derivation from the embryological "meninx primitiva" theory is the most plausible theory that it suggests an abnormal, persistent focus of meninx primitiva differention into adipose tissue and maturation into a lipoma.1 Histopathological examination of lipomas reveals that they are composed of mature adipocytes, fibromuscular tissue, bone and calcifications, vessels and nerves passing through the lipoma.1,5,6

About 50% of the intracranial lipomas are asymptomatic.4,7 The most common presenting symptom is epilepsy.3,8 Epileptic seizures may be either grand mal or petit mall, but the seizures are usually severe partial type and they often appear before 15 years age.8

Headache, vomiting, hemiplegia vertigo, mental retardation or emotional lability are the other findings.7,9 Due to their localizations intracranial lipomas may lead to unilateral sensorioneural hearing loss10, precocious puberty11 or hydrocephaly.12,13

The rare occurrence of intracranial lipomas makes the diagnosis difficult. Since lipomas are usually asymptomatic they are diagnosed incidentally on CT or MRI evaluations or at autopsy as in our case. On CT, lipomas are sharply demarcated areas of marked hypodensity that do not demonstrate enhancement after contrast injection. Calcification is often present within the fibrous capsule of the lipoma. On MRI there is a hypointense mass on T1 and T2 -weighted sequences and it appears hypointense on FS (fat saturated) sequences.14 Most intracranial lipomas occur in the midline in the region of the corpus callosum (45%), the ambient cistern and suprasellar region (23%).1,6 These lesions are frequently associated with hypogenesis or agenesis of corpus callosum.4 Intracerebral vessels and cranial nerves may cross through the mass.1 Similarly our case showed that anterior cerebral arteries were crossing through the mass and giving its branches within the lipoma, visualized by contrast enhanced MRI images.

Because of the rarity of these neoplasms therapeutic experience with primary intracranial lipomas is also extremely limited. Surgical resection should only be considered in patient with intractable seizures, or hydrocephaly or intractable headache.2,9,10 As intracranial lipomas cannot be treated successfully by surgical resection the ideal treatment consists of conservative approaches.8 For this reason anticonvulsants can be used in patients with seizures as in our case.

In conclusion, although intracranial lipomas are very rarely seen, these tumors should be considered in the differential diagnosis of the intracranial tumors in children with partial seizures and non-febrile seizure.

REFERENCES

1. Truwit CL, Barkovich AJ. Pathogenesis of intracranial lipoma: An MR study in 42 patients. AJR Am J Roentgenol 1990;155:855-865.

2. Yilmazlar S, Kocaeli H, Aksoy K. Quadrigeminal cistern lipoma. J Clin Neurosci 2005;12:596-599.

3. Wallace D. Lipoma of the corpus callosum. J Neurol Neurosurg Psychiatry 1976;39:1179-1185.

4. Suri V, Sharma MC, Suri A, Karak AK, Garg A, Sarkar C, et al. Myelolipomatous change in an interhemispheric lipoma associated with corpus callosum agenesis: Case report. Neurosurgery 2008;62:E745.

5. Kiymaz N, Cirak B. Central nervous system lipomas. Tohoku J Exp Med 2002;198:203-206.

6. Pekarski KL, Prayson RA. Suprasellar spindle cell lipoma. Ann Diagn Pathol 2009;13:173-175.

7. Baeesa SS, Higgins MJ, Ventureyra EC. Dorsal brain stem lipomas: case report. Neurosurgery 1996;38:1031-1035.

8. Kieslich M, Ehlers S, Bollinger M, Jacobi G. Midline developmental anomalies with lipomas in the corpus callosum region. J Child Neurol 2000;15:85-89.

9. Davutoglu M, Yesil Y, Asan A, Dolu H, Karabiber H. Non-febrile seizure associated with pericallosal lipoma. Pediatr Neonatol 2008;49:245-247.

10. Brodsky JR, Smith TW, Litofsky S, Lee DJ. Lipoma of the cerebellopontine angle. Am J Otolaryngol 2006;27:271-274.

11. Park YS, Kwon JT, Park US. Interhemispheric osteolipoma with agenesis of the corpus callosum. J Korean Neurosurg Soc 2010;47:148-150.

12. Kazner E, Stochdorph O, Wende S, Grumme T. Intracranial lipoma. Diagnostic and therapeutic considerations. J Neurosurg 1980;52:234-245.

13. Maiuri F, Corriero G, Gallicchio B, Simonetti L. Lipoma of the ambient cistern causing obstructive hydrocephalus. J Neurosurg Sci 1987;31:53-58.

14. Yildiz H, Hakyemez B, Koroglu M, Yesildag A, Baykal B. Intracranial lipomas: Importance of localization. Neuroradiology 2006;48:1-7.
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Author:Karaaslan, Erhan; Ozcetin, Mustafa; Tetikcok, Ramazan
Publication:Pakistan Journal of Medical Sciences
Article Type:Clinical report
Geographic Code:9PAKI
Date:Sep 30, 2011
Words:1141
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