PATHOLOGY
A 12-Year-Old
Boy
with Two
Lipomas
in the
Third Ventricle A Case
Report
Samruay Shuangshoti, M.D.,* Jajaval Dharmmaponpilas,
OUR
PREVIOUS
revealed that 0.1 and 1.5 per cent
study
lipomas comprise only of intracranial and intraspinal tumors, respectively.’ Because of the rarity, we describe here a child in whom such the third ventricle. Clinical
tumors arose
in
History
A week before
hospitalization, this 12-yearold boy suddenly developed diplegia of the lower limbs. One morning, when he got out of bed, being unable to stand, he fell on the floor. A few days later, paralysis of the upper limbs and dysphagia began. On admission, his vital signs were normal. He had weakness of the muscles of all extremities and absence of the tendon reflexes.
Consciousness, sensation
were
cranial nerve functions, and intact. The eyegrounds were
normal. The lungs were crepitant throughBlood count and blood chemistry readings were normal. A lumbar puncture showed clear colorless cerebrospinal fluid (CSF), pressure 130 mm. The CSF contained 15 lymphocytes/cu mm, 37.5 mg/100 ml of protein, 86 mg/100 ml of sugar, and was sterile. out.
Assistant Professor of Pathology, Faculty of Medicine, Chulalungkorn University, Bangkok 5, Thailand. ** Instructor of Pathology. *
M.D.**
The clinical impression was acute poliomyelitis. The patient died of respiratory failure two weeks after the onset of illness.
Autopsy Findings The thorax and abdomen showed bilateral bronchopneumonia. Sections through various levels of the spinal cord disclosed intact anterior horn cells, with no inflammation. The brain weighed 1,300 gm and showed no herniation or hydrocephalus. Two rounded yellow and encapsulated masses, 8.0 and 5.0 mm in diameter, were found in the third ventricle. The larger mass, in the anterosuperior part of the ventricle (Fig. 1 A), did not occlude the interventricular foramens. The smaller mass, in the posterior part of the ventricle on the left side, was near the pineal body; it filled the groove formed by the medial wall of the thalamus and the ventricular roof. Histologically, both masses were made up of sheets of mature adipose tissue cells, separated by thin bands of connective tissue. The larger mass, having a few psammomatbodies, was covered in part by the epithelium of the choroid plexus, and attached by sparse villi of the plexus (Figs. 1 B and 1C). Sudanophilic droplets, presumably neutral lipid, were present in the cytoplasm of the adipose tissue cells. The diagnosis was two lipomas of the third ventricle, one arising in the choroid plexus.
459
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__.--.......
FIG. 1. A. Coronal section of the brain through the region of the third ventricle and mammillary bodies showing to the anterior part of the ventricular roof. Disruption of the corpus callosum is an artifact. B. The tumor consists of mature adipose tissue cells, and is covered by the epithelium of the choroid plexus. (H and E stain, x400). C. Multiple villi of the choroid plexus are on the surface, and a few psammoma bodies lie in the peripheral portion of the lipoma. (H and E stain, x400). a
lipoma (arrow) attaching
Although any mass in the third as a neuroepithelial (colloid) cyst, may produce symptoms even when it is as small as 7 mm across,’ we thought that these two lipomas should be asymptomatic, since there was no papilledema, hydrocephComments.
ventricle such
alus, and cerebral herniation. The immediate cause of the quadriplegia was not evident at autopsy. Discussion In
addition
to
lipoma3-8
and
liposar-
coma,9,lO other primary tumors of the central nervous system (CNS) may contain some adipose tissue, as in meningioma with lipomatous or liposarcomatous components.11,12 The lipomas in our patient, and particularly the larger one, must be distinguished from the intraventricular meningiomas which may contain
bodies. 11,13 We that since suggest, however, psammoma bodies or calcospherites are disseminated normally in the stroma of the choroid plexus, and may be found from the age of two months, 14 these were presumably entrapped within the lipoma. Nor do we regard the tumors in our case to be teratomas,15 even though lying in the midline of the brain. No elements other than adipose tissue were found in many sections. That a neuraxial lipoma may be a tumorlike malformation derived from the mesenchyme or its derivatives is supported by a number of reports of lipomas of the CNS associated with similar tumors in other parts of the neuraxis, or with other congenital defects of the brain or elsewhere in the body.3.5,7.g,16 Their usual occurrence in
adipose tissue and psammoma
460
Downloaded from cpj.sagepub.com at UNIVERSITY OF SASKATCHEWAN LIBRARY on June 19, 2015
points also to a congenital origin, although they may be encountered at any age. 5,8 The frequent occurrence of these neuraxial lipomas in the midline-as in the corpus callosum, 5,8 the third ventricle, the spinal children
or near the midline as in the lateral ventricular choroid plexus4-6-suggests further their pathogenesis is related to a dysrhaphic disorder of the CNS with maldifferentiation of the mesenchyme or its derivatives.11,18 Mesenchymal cells and their derivatives are pluripotential; they may differentiate into other mesenchymal elements, such as adipose tissue.’9 Lipomas, therefore, may arise from the CNS mesenchyme or meninges as do meningiomas 17 and primary neuraxial rhabdomyosarcoma.2°
cord,3,7
Acknowledgments
5.
corpus callosum: a clinicopathologic study. Am. J. Roentgenol. Rad. Ther. Nucl. Med. 55: 125, 1946. 6. Machacek, F.: Lipom des Balkens und symmetrische plexuslipome. Wien. Klin. Wochenschr. 78: 390, 1966. 7. Wolbach, S., and Millet, J. A.: Diffuse subdural lipomatosis of the spinal cord in an infant. Boston
Med, Surg. J. 681, 1913. 168: 8. Zettner, A., and Netsky, M. G.: Lipoma of the corpus callosum. J. Neuropathol. Exp. Neurol. 19: 305, 1960. 9. Caldwell, J. A., and Zinninger, M. M.: Extradural liposarcoma of the spinal canal: a clinical and pathological report. Surg. Gynecol. Obstet.
40: 476, 1925. 10. Kothandaram, P.: Dural liposarcoma associated with subdural hematoma. J. Neurosurg. 33: 85, 1970. 11. Lapresle, J., Netsky, M. G., and Zimmerman, H. M.: The pathology of meningiomas. Am. J. Pathol.
28: 757, 1952. 12.
13.
14.
15.
References 1.
2.
Shuangshoti, S., and Panyathanya, R.: Neural neoplasms in Thailand: a study of 2,897 case. Neurology (Minneap.) 24: 1127, 1974. —, Robert, M. P., and Netsky, M. G.: Neuroepithelial (colloid) cysts: pathogenesis and relation to choroid plexus and ependyma. Arch.
Pathol. 80: 214, 1965. 3. Baker, A. B., and Adams, J. M.: Lipomatosis of the central nervous system. Am. J. Cancer 34: 214, 1938. 4. Liber, A. R., and Lisa, J. R.: Stromal tumors of the choroid plexus. Am. J. Clin. Pathol. 10: 710, 1946.
S.: Neoplasm of mixed mesenchymal neuroepithelial origin: liposarcomatous meningioma combined with gliomas. J. Neurol. Neurosurg. Psychiatr. 36: 377, 1973. Kandel, E. I., and Filatov, Y. M.: Clinical picture and surgical treatment of meningiomas of the lateral ventricle. Proc. 3rd Int. Cong. Neurol. 110: 719, 1966. Surg. Shuangshoti, S., and Netsky, M. G.: Human choroid plexus: morphologic and histochemical alterations with age. Am. J. Anat. 128: 73, 1970. Ingraham, F. D., and Bailey, O. T.: Cystic teratomas and teratoid tumors of the nervous system in infancy and childhood. J. Neurosurg. 3: 511, 1946. Haberland, C., and Perou, M.: Encephalocraniocutaneous lipomatosis: a new example of ectomesodermal dysgenesis. Arch. Neurol. 22: 144, 1970. Shuangshoti, S.: Meningioma of the optic nerve. Br. J. Ophthamol. 57: 265, 1973. —, and Netsky, M. G.: Histogenesis of choroid plexus in man. Am. J. Anat. 118: 283, 1966. Willis, R. A.: Pathology of Tumours, 3rd ed.
Shuangshoti, and
°
This study was supported by Rachadapiseksompoj Research Grant of Chulalongkorn University on the Royal Silver Jubilee Anniversary. Permission to report this case was given by Professor Somsak Dhechakaisaya, Head of the Department of Pathology, Chulalongkorn University.
List, C. F., Holt, J. F., and Everett, M.: Lipoma of the
16.
17. 18. 19.
London, Butterworths, 1960, pp 722-31. 20.
Shuangshoti, S., Piyaratn, P., and Viriyapanich, P. L.: Primary rhabdomyosarcoma of cerebellum: necropsy report. Cancer 22: 367, 1968.
461
Downloaded from cpj.sagepub.com at UNIVERSITY OF SASKATCHEWAN LIBRARY on June 19, 2015
A 12-Year-Old
Boy
with Two
Lipomas
in the
Third Ventricle A Case
Report
Samruay Shuangshoti, M.D.,* Jajaval Dharmmaponpilas,
OUR
PREVIOUS
revealed that 0.1 and 1.5 per cent
study
lipomas comprise only of intracranial and intraspinal tumors, respectively.’ Because of the rarity, we describe here a child in whom such the third ventricle. Clinical
tumors arose
in
History
A week before
hospitalization, this 12-yearold boy suddenly developed diplegia of the lower limbs. One morning, when he got out of bed, being unable to stand, he fell on the floor. A few days later, paralysis of the upper limbs and dysphagia began. On admission, his vital signs were normal. He had weakness of the muscles of all extremities and absence of the tendon reflexes.
Consciousness, sensation
were
cranial nerve functions, and intact. The eyegrounds were
normal. The lungs were crepitant throughBlood count and blood chemistry readings were normal. A lumbar puncture showed clear colorless cerebrospinal fluid (CSF), pressure 130 mm. The CSF contained 15 lymphocytes/cu mm, 37.5 mg/100 ml of protein, 86 mg/100 ml of sugar, and was sterile. out.
Assistant Professor of Pathology, Faculty of Medicine, Chulalungkorn University, Bangkok 5, Thailand. ** Instructor of Pathology. *
M.D.**
The clinical impression was acute poliomyelitis. The patient died of respiratory failure two weeks after the onset of illness.
Autopsy Findings The thorax and abdomen showed bilateral bronchopneumonia. Sections through various levels of the spinal cord disclosed intact anterior horn cells, with no inflammation. The brain weighed 1,300 gm and showed no herniation or hydrocephalus. Two rounded yellow and encapsulated masses, 8.0 and 5.0 mm in diameter, were found in the third ventricle. The larger mass, in the anterosuperior part of the ventricle (Fig. 1 A), did not occlude the interventricular foramens. The smaller mass, in the posterior part of the ventricle on the left side, was near the pineal body; it filled the groove formed by the medial wall of the thalamus and the ventricular roof. Histologically, both masses were made up of sheets of mature adipose tissue cells, separated by thin bands of connective tissue. The larger mass, having a few psammomatbodies, was covered in part by the epithelium of the choroid plexus, and attached by sparse villi of the plexus (Figs. 1 B and 1C). Sudanophilic droplets, presumably neutral lipid, were present in the cytoplasm of the adipose tissue cells. The diagnosis was two lipomas of the third ventricle, one arising in the choroid plexus.
459
Downloaded from cpj.sagepub.com at UNIVERSITY OF SASKATCHEWAN LIBRARY on June 19, 2015
__.--.......
FIG. 1. A. Coronal section of the brain through the region of the third ventricle and mammillary bodies showing to the anterior part of the ventricular roof. Disruption of the corpus callosum is an artifact. B. The tumor consists of mature adipose tissue cells, and is covered by the epithelium of the choroid plexus. (H and E stain, x400). C. Multiple villi of the choroid plexus are on the surface, and a few psammoma bodies lie in the peripheral portion of the lipoma. (H and E stain, x400). a
lipoma (arrow) attaching
Although any mass in the third as a neuroepithelial (colloid) cyst, may produce symptoms even when it is as small as 7 mm across,’ we thought that these two lipomas should be asymptomatic, since there was no papilledema, hydrocephComments.
ventricle such
alus, and cerebral herniation. The immediate cause of the quadriplegia was not evident at autopsy. Discussion In
addition
to
lipoma3-8
and
liposar-
coma,9,lO other primary tumors of the central nervous system (CNS) may contain some adipose tissue, as in meningioma with lipomatous or liposarcomatous components.11,12 The lipomas in our patient, and particularly the larger one, must be distinguished from the intraventricular meningiomas which may contain
bodies. 11,13 We that since suggest, however, psammoma bodies or calcospherites are disseminated normally in the stroma of the choroid plexus, and may be found from the age of two months, 14 these were presumably entrapped within the lipoma. Nor do we regard the tumors in our case to be teratomas,15 even though lying in the midline of the brain. No elements other than adipose tissue were found in many sections. That a neuraxial lipoma may be a tumorlike malformation derived from the mesenchyme or its derivatives is supported by a number of reports of lipomas of the CNS associated with similar tumors in other parts of the neuraxis, or with other congenital defects of the brain or elsewhere in the body.3.5,7.g,16 Their usual occurrence in
adipose tissue and psammoma
460
Downloaded from cpj.sagepub.com at UNIVERSITY OF SASKATCHEWAN LIBRARY on June 19, 2015
points also to a congenital origin, although they may be encountered at any age. 5,8 The frequent occurrence of these neuraxial lipomas in the midline-as in the corpus callosum, 5,8 the third ventricle, the spinal children
or near the midline as in the lateral ventricular choroid plexus4-6-suggests further their pathogenesis is related to a dysrhaphic disorder of the CNS with maldifferentiation of the mesenchyme or its derivatives.11,18 Mesenchymal cells and their derivatives are pluripotential; they may differentiate into other mesenchymal elements, such as adipose tissue.’9 Lipomas, therefore, may arise from the CNS mesenchyme or meninges as do meningiomas 17 and primary neuraxial rhabdomyosarcoma.2°
cord,3,7
Acknowledgments
5.
corpus callosum: a clinicopathologic study. Am. J. Roentgenol. Rad. Ther. Nucl. Med. 55: 125, 1946. 6. Machacek, F.: Lipom des Balkens und symmetrische plexuslipome. Wien. Klin. Wochenschr. 78: 390, 1966. 7. Wolbach, S., and Millet, J. A.: Diffuse subdural lipomatosis of the spinal cord in an infant. Boston
Med, Surg. J. 681, 1913. 168: 8. Zettner, A., and Netsky, M. G.: Lipoma of the corpus callosum. J. Neuropathol. Exp. Neurol. 19: 305, 1960. 9. Caldwell, J. A., and Zinninger, M. M.: Extradural liposarcoma of the spinal canal: a clinical and pathological report. Surg. Gynecol. Obstet.
40: 476, 1925. 10. Kothandaram, P.: Dural liposarcoma associated with subdural hematoma. J. Neurosurg. 33: 85, 1970. 11. Lapresle, J., Netsky, M. G., and Zimmerman, H. M.: The pathology of meningiomas. Am. J. Pathol.
28: 757, 1952. 12.
13.
14.
15.
References 1.
2.
Shuangshoti, S., and Panyathanya, R.: Neural neoplasms in Thailand: a study of 2,897 case. Neurology (Minneap.) 24: 1127, 1974. —, Robert, M. P., and Netsky, M. G.: Neuroepithelial (colloid) cysts: pathogenesis and relation to choroid plexus and ependyma. Arch.
Pathol. 80: 214, 1965. 3. Baker, A. B., and Adams, J. M.: Lipomatosis of the central nervous system. Am. J. Cancer 34: 214, 1938. 4. Liber, A. R., and Lisa, J. R.: Stromal tumors of the choroid plexus. Am. J. Clin. Pathol. 10: 710, 1946.
S.: Neoplasm of mixed mesenchymal neuroepithelial origin: liposarcomatous meningioma combined with gliomas. J. Neurol. Neurosurg. Psychiatr. 36: 377, 1973. Kandel, E. I., and Filatov, Y. M.: Clinical picture and surgical treatment of meningiomas of the lateral ventricle. Proc. 3rd Int. Cong. Neurol. 110: 719, 1966. Surg. Shuangshoti, S., and Netsky, M. G.: Human choroid plexus: morphologic and histochemical alterations with age. Am. J. Anat. 128: 73, 1970. Ingraham, F. D., and Bailey, O. T.: Cystic teratomas and teratoid tumors of the nervous system in infancy and childhood. J. Neurosurg. 3: 511, 1946. Haberland, C., and Perou, M.: Encephalocraniocutaneous lipomatosis: a new example of ectomesodermal dysgenesis. Arch. Neurol. 22: 144, 1970. Shuangshoti, S.: Meningioma of the optic nerve. Br. J. Ophthamol. 57: 265, 1973. —, and Netsky, M. G.: Histogenesis of choroid plexus in man. Am. J. Anat. 118: 283, 1966. Willis, R. A.: Pathology of Tumours, 3rd ed.
Shuangshoti, and
°
This study was supported by Rachadapiseksompoj Research Grant of Chulalongkorn University on the Royal Silver Jubilee Anniversary. Permission to report this case was given by Professor Somsak Dhechakaisaya, Head of the Department of Pathology, Chulalongkorn University.
List, C. F., Holt, J. F., and Everett, M.: Lipoma of the
16.
17. 18. 19.
London, Butterworths, 1960, pp 722-31. 20.
Shuangshoti, S., Piyaratn, P., and Viriyapanich, P. L.: Primary rhabdomyosarcoma of cerebellum: necropsy report. Cancer 22: 367, 1968.
461
Downloaded from cpj.sagepub.com at UNIVERSITY OF SASKATCHEWAN LIBRARY on June 19, 2015
