Child's Brain 5: 476-483 (1979)

Lipoma of the Corpus callosum: Report of a Case and Review of the Literature Tatsunori Suemit.su, Shim-Ichi Nakajima, Katsuko Kuwajima, Kenji Nihei and Shigehiko Kamoshita Department ef Pediatrics, Jichi Medical School. Tochigiken

Key Words. Lipoma • Corpus callosum • Circular calcification • Computerized tomography Abstract. A case of lipoma of the corpus callosum associated with subcutaneous lipoma, whose symptoms were headache and trembling of the extremities, was reported. Roentgenograms showed characteristic findings and computerized tomography revealed a mass of very low density. The literature was reviewed and 15 cases not included in previous compilations were found. Clinical and radiological findings in 89 cases including the present case were analyzed.

Lipoma of the corpus callosum is a fairly rare disease: it is thus inter­ esting to report the occasional cases. The purpose of this paper is to present a case and to review the literature, chiefly from a clinical viewpoint.

Case Report

Downloaded by: King's College London 137.73.144.138 - 9/25/2018 1:56:45 PM

A 13-year-old Japanese boy has had a small lump on the front of the head since the age of 2 years. His family history was not contributory. He was well until May 1977, when he was attacked with cramps in the gastrocnemius several times while sleeping. After the episode of cramps, he had occasional headaches, one of which was followed by vomiting. 2 months later, he had a sensation of numbness in his hands and feet, followed by trembling of the upper extremities at a frequency of 3/sec. lasting half an hour, pro­ nounced on the right. During those episodes, the level of consciousness was clear and neither convulsion nor paralysis was noted. I month later, he was admitted to the Jichi Medical School Hospital for further examination. Physical examination revealed a rather obese boy (height 162 cm, weight 66 kg). A soft subcutaneous mass measuring 3 * 3 * 1 cm was located on the midline of the

Lipoma of the Corpus callosum

477

parietal region. Neurological examination revealed no abnormal findings. The routine hematological, serological, biochemical, and urine examinations wre all normal. Analysis of serum lipids was normal. Plain skull roentgenogram showed a central mass of radiolucent material measuring approximately 3 x 3 cm outlined with a curvilinear calcification (fig. I). There was no evidence of increased intracranial pressure nor bone defect. Pneumoencephalogram showed laterally dislocated lateral ventricles and depressed septum pellucidum. In addition, the third ventricle was dilated in width (10 cm) but not elevated. The carotid angiogram showed that the left anterior cerebral artery with the preserved normal con­ figuration in the third portion (genu), was suddenly displaced in the fourth portion by the radiolucent mass and formed a loop, from which a wide branch of irregular diameter (fig. 2) entered the mass. In the venous-phase angiogram, a large vein was draining from the mass into the internal cerebral vein, which was pulled upward at the concourse. The great vein of Galen ran the normal sigmoid curve.

Downloaded by: King's College London 137.73.144.138 - 9/25/2018 1:56:45 PM

Fig. 1. Plain skull roentgenogram. Midline low-density mass surrounded by a cir­ cular calcification.

Sucniitsu Nakajima/Kuwajima/Nihei Kamoshita

478

Fig. 3. Computerized tomography: a low-density central mass is revealed.

Downloaded by: King's College London 137.73.144.138 - 9/25/2018 1:56:45 PM

Fig. 2. Left carotid angiogram: the pericallosal artery, whose diameter is irregularly wide, is elevated in the fourth portion by the radiolucent mass.

Lipoma of the Corpus callosum

479

Computerized tomography revealed a central mass with a lower density than water, Haunsfield's number being 120. No ventricular dilation was noted (fig. 3). These clinical and laboratory findings were compatible with a lipoma of the corpus callosum associated with a subcutaneous lipoma. The subcutaneous mass was excised from between the scalp dermis and the galea. Histologically it was a mature fatty tissue with rich vasculature. In the 10 months following diagnosis he has remained free of clinical symptoms. In particular, he has not had any convulsions and his EEG was normal.

Discussion

Clinical Findings The details are shown in table II. The most common symptom are convulsions (45 cases; 56%). Mental changes are also common (32 cases; 40%). Paralysis (14 cases; 17%) and headaches (13 cases; 16%) were oc­ casionally noted. 3 patients [14, 21] were described to have had trembling of extremities as in our patient. The symptoms due to a tumor of the corpus callosum have been described as Bristowe’s syndrome which consists of peculiar mental disturb­ ances including apathy, lethargy and amnesia: hemiplegia, and convulsive attacks. In regard to a lipoma of the corpus callosum, even complete absence of clinical symptoms is not so rare, and neurological signs are often negative. Therefore, the clinical diagnosis of lipoma of the corpus callosum can be difficult. It is noteworthy that the association of lipoma of the corpus callosum with subcutaneous lipoma on the midline is described in 9 cases (11%) and with obesity in 6 cases (7%). Both are interesting in connection with ab­ normal lipid metabolism.

Downloaded by: King's College London 137.73.144.138 - 9/25/2018 1:56:45 PM

30 [II] to 50% [22] of intracranial lipomas, which account for about 0.1% of all brain tumors [16] are sited in the corpus callosum. A recent report [19] indicates that 92 cases of lipoma of the corpus callosum have been reported since the first description by Rokitansky in 1856. In Japan only 5 cases [7, 8, 10, 12] have been reported so far. U p to the present, List el at. [11 ], Nordin et al. [ 14], Zettner et al. [22], Cooper et al. [6] and Nonaka et al. [12] reviewed the literature and charted the pertinent clinical features of the cases. We have been able to find 15 cases not previously compiled in these reviews. These cases are listed in table I together with the present case. The following considerations are based on the analysis of 89 cases including those listed in table 1.

480

Suemitsu/Nakajima Kuwajima/Nihci/Kamoshita Tabic I. Recent reports of the lipoma of the corpus callosum Case Year Reference Age Years No.

Sex

Anatomical findings

Clinical findings

genu, splénium rostrum agenesis of the corpus callosum agenesis of the corpus callosum frontal meningocele rostrum agenesis of the corpus callosum and septum pellucidum

grand mal type epilepsy spastic hemiplegia, headache, generalized epilepsy, MD, athetosis grand mal type convulsion, automatism. MD psychomotor deficiency

1 2

I960 1962

2 9

38 14

F F

3

1962

3

15

M

4

1962

1

1

M

5

1965

18

19

F

6

1966

12

42

F

27

M

1967

15

1974

4

31 73

M F

1974

5

8 8

M M

21

M

7*

M

8 9 10 II

12 13

1975

19

II

M

13 this study 13

M M

14 15 16

1978

bulging into the lateral ventricle synostosis in C4-5 spina bifida in L5 in whole length of the corpus callosum rostrum

Tonic clonic convulsion. MD. vertigo, nystagmus. athetosis, microcephalus, syndactyly headache, nervousness. papilledema grand mal type epilepsy. left hemiconvulsion grand mal type epilepsy dead with apoplexy

headache, vomiting generalized convulsion, midline subcutaneous lipoma agenesis of the splénium craniostosis anterior fontanelle bone asymptomatic agenesis of the corpus hypnotic, trouble in the character, retardation in callosum language MD, hypnotic. agenesis of the corpus hemiconvulsion callosum agitation, convulsion headache, trembling

1 Months.

Downloaded by: King's College London 137.73.144.138 - 9/25/2018 1:56:45 PM

Radiological Findings After Sosman described the simple skull roentgenogram of this disease, it has been said to be so characteristic that no other diagnostic tests are necessary. In the midline, there is a radiolucent shadow made by fatty tissue,

Lipoma of the Corpus callosum

481

Table II. Clinical features in the 81 cases of lipoma of the corpus callosum Clinical features

Convulsion Mental disturbance Paralysis Headache Subcutaneous lipoma Obesity Trembling of extremities Vertigo Urinary disturbance Asymptomatic

Cases n

%

45 32 14 13 9 6 4 4 2 8

56 40 17 16 11 7 5 5 2 10

Therapy and Prognosis Because of the extensive vascularity of the tumor and close association with the anterior cerebral artery, surgical removal of the tumor is very difficult. 23 cases underwent craniotomy, but in many cases only to confirm the diagnosis. 11 of 16 cases who underwent resection died within 3 months. As this tumor has neither rapid growth nor malignant transformation, it seems sufficient to avoid surgery and control seizures if present.

Downloaded by: King's College London 137.73.144.138 - 9/25/2018 1:56:45 PM

whose roentgen ray coefficient is low, surrounded by a circular calcification. Such a pattern is expressed as crescent-shaped [17], sickle-shaped [14] and curvilinear [5]. When disraphic states are present as well, a midline skeletal deficit may be seen [10, 11, 22]. The pneumoencephalogram reveals laterally displaced anterior horns of the lateral ventricles, and widened and depressed septum pellucidum and third ventricles. Among 65 cases which underwent craniotomy or autopsy, agenesis or hypogenesis of the corpus callosum was found in 24 cases. Under such circumstances, it is said that the third ventricles are elevated. However, it seems difficult to determine whether the lipomas are accompanied by agenesis or not if only a pneumoencephalogram is performed [20], In the cerebral angiogram, the characteristic findings are tortuosity and dilation of the anterior cerebral artery, from which many branches enter the tumor. Drainage veins flow into the great cerebral vein and the internal cerebral veins.

Suemitsu/Nakajima/Kuwajima/Nihei/Kamoshita

482

Pathogenesis According to Bucika [4], intracranial lipoma is not a true neoplasm but a kind of hamartoma. Although its pathogenesis is still unknown, in respect to its origin, there are several speculations including displacement of embryonal mesenchymal tissue, hyperplasia of fat cells of normal pia, and fatty metaplasia of connective tissue or pial cells [16]. The fact that cases are often accompanied with agenesis of the corpus callosum and dysraphic disorders makes the displacement theory more likely. Association of sub­ cutaneous lipomas and obesity as in the present case perhaps suggests a generalized abnormality of lipid metabolism, although at present definite evidence is lacking.

1 2

3 4 5 6 7 8 9 10 11 12 13 14

Bianchedi, S. e Perizza, A.: II lipoma del corpo calloso. Minerva paediat. 14: 1015-1020(1962). Bonnal, Darcourt, G .; Bérard-Badier, M., et al.: Lipome du corps calleux. Calcification unilatérale. Etude des séquelles postopératives. Revue neurol. 103: 501-506 (1960). Bossi, L. e Caffaratti, E. : Considerazioni cliniche e radiologiche su di un caso di lipoma del corpo calloso. Minerva med. Roma 53: 690-697 (1962). Budka, H.: Intracranial lipomatous hamartomas (intracranial ‘lipomas’) Acta neuropath. 28: 205-222 (1974). Cascino, J.P.; Lake, F .; Jackson, C., et al. : Lipoma of the corpus callosum. J. int. Coll. Surg. 29: 171-174(1958). Cooper, W.C. and Hagen, K.O. von: Lipoma of the corpus callosum. Report of a case. Bull. Los Ang. neurol. Soc. 273: 39-44 (1962). Harada, M. ; Kinoshita, K., and Matsukado, Y. : Lipoma in the region of the corpus callosum (in Japanese). Brain Dev. 6: 59-63 (1974). Honda, I. and Shirai, S. : On the lipoma of the corpus callosum (in Japanese). Trans. Jap. path. Soc. 23: 603-605 (1933). Ketz, E. von: Zur Klinik und Therapie der sog. Balkenlipome. Zentbl. Neurochir. 16: 80-84 (1956). Kuwabara, T.; Yoshioka, M., and Akashi, K..: Intracranial lipoma, a case report (in Japanese). Clin. Neurol. 37; 485-486 (1963). List, C.F.; Holt, J.F., and Everett, M.: Lipoma of the corpus callosum. Am. J. Roentg. 55: 125-134 (1946). Manganiello, L.O.J. ; Daniel, E.F., and Hair, L.Q. : Lipoma of the corpus callosum. J. Neurosurg. 24: 892-894 (1966). Nonaka, F.; Otsuka, A.; Ezuka, L, et al.: Lipoma of the corpus callosum, a case report (in Japanese). Brain Nerve 26: 709-716 (1974). Nordin, W.A.; Tesluk, H., and Jones, R.K. : Lipoma of the corpus callosum. Archs. Neurol. Psychiat., Chicago 74: 300-307 (1955).

Downloaded by: King's College London 137.73.144.138 - 9/25/2018 1:56:45 PM

References

Lipoma of the Corpus callosum

16 17 18 19 20 21 22

Sabouraud, O.; Pecker, J.; Simon, P., et al.: Lipomes du corps calleux. Données angiographiques et discussion pathogénique de leur séméiologie clinique. Revue neurol. 117: 557-570 (1967). Schmid, A.H. : Lipoma of the cerebellum. Acta neuropath. 26: 75-80 (1973). Smith, C.D. and Weaver, E.N.: Lipoma of the corpus callosum. Am. J. Roentg. 69: 605-607 (1953). Trillet, M.; Michel, D. et Pellet, J.: Lipome du corps calleux associé à une agénésie du septum pellucidum. J. Radiol. Electrol. Méd. nucl. 46: 184-187 (1965). Turpin, J.C. et Raux, E. : Pronostic du lipome du corps calleux de l'enfant et de l’adolescent. Pédiatrie 30: 513-524 (1975). Worpert, S.M.; Carter, B.L., and Ferris, F.J.: Lipomas of the corpus callosum, an angiographic analysis. Am. J. Roentg. 115: 92-99 (1972). Weidner, W.; Jannetta, P.J.; Saul, R. et al.: The neuroradiology of tumors of the corpus callosum. Neurology, Minneap. 15: 1071-1077 (1965). Zettner, A. and Netsky, M.G.: Lipoma of the corpus callosum. J. Neuropath, exp. Neurol. 19: 305-319 (1960).

Tatsunori Suemitsu, MD, Department of Pediatrics Jichi Medical School, 3311-1 Minamikawachi, Tochigiken, 329-04 (Japan)

Downloaded by: King's College London 137.73.144.138 - 9/25/2018 1:56:45 PM

15

483