Spinal cord lipomas without associated spinal dysraphism are uncommon,
accounting for 1% of spinal cord tumours.5,7,8 These mostly involve the
cervicothoracic region and their growth causes ventral flattening of the
cord and separation of the posterior columns.4,7 Magnetic resonance imaging
(MRI) provides information regarding the location so that adequate decompressive
surgery can be performed.4,9,12 We report our experience with four cases
of intramedullary lipoma that were treated surgically in our institution
and discuss the possible pathogenesis, clinical, radiological and therapeutic
aspects of these lesions.
Patient 1: A 15-year-old female, with unremarkable past medical
history presented with painful dysesthesia in the interscapular and thoraco-lumbar
areas, followed by gait disturbances. Symptoms started 3 months prior
to presentation. Clinical examination revealed spastic paraparesis with
difficult gait, hypoesthesia with T6 sensory level and sphincter disturbances.
Plain x-ray showed spinal cord enlargement from C7 to T5, associated with
thoracic scoliosis. Myelography by suboccipital approach demonstrated
a complete block of the spinal canal. Computed tomography (CT) scan showed
a large low density mass (-30 to –130 HU) displacing the cord and extending
from C6 to T6. Nature of this mass was suggestive of a lipoma. Laminectomy
from T6 to C6 was performed. There was a large intradural intramedullary
mass without clear cleavage plane between the spinal cord and the tumour.
Partial resection with duraplasty was performed. Pathological examination
confirmed the diagnosis of lipoma. There were no postoperative complications.
Follow-up during 14 years has shown no evidence of progression.
Patient 2: A 15-year-old patient was admitted to our institution
in January 1988 with low back pain of several months’ duration. The pain
was in the lower interscapular area and radiated to the right side of
the chest. The patient denied any weakness in the legs or bladder and
bowel difficulties. General and neurological examinations were within
normal limits. Plain x-ray film of the spine showed spinal canal enlargement
and pedicular erosion from T1 to T7. Myelography revealed a complete block
at T7. CT scan showed intramedullary fatty lesion extending from T2 to
T7 (Fig. 1). Surgical exploration consisted of laminiectomy. At dural
opening, the intramedullary location of the tumour was confirmed. Biopsy
and duraplasty was made. Histological examination revealed that the tumour
was composed of mature fatty tissue. The post-operative course was uneventful
with slight clinical improvement. Six years later neurological examination
was normal.
Patient 3: This otherwise healthy 43-year-old man was admitted
to our institution with 2-month history of neck pain and bilateral brachalgia
associated with progressive weakness of the lower and upper limbs and
difficulty in walking. There were no bladder or bowel disturbances. Examination
revealed a flaccid type of distal weakness with hyporeflexia of both upper
limbs. There was a spastic paraparesis with brisk tendon jerks, up going
plantars and sustained ankle clonus bilaterally. Pain and temperature
sensations were reduced from T4. Vibration and joint position senses were
normal. X-rays of the cervicothoracic spine showed evidence of pedicular
erosions. Lumbar myelography demonstrated complete block at T4. CT scan
showed an intramedullary fatty lesion (-95 HU). At operation decompressive
laminectomy C3 to T4 was performed. There was a lipomatous intramedullary
tumour without clear cleavage plane between the tumour, spinal cord and
the roots. Surgery was limited to a biopsy. Histological examination confirmed
it to be a mature lipoma. Post-operative course was uneventful and follow-up
at 3 years revealed no abnormality.
Patient 4: A 16-year-old female was admitted to our institution
in February 1998 with a history of slowly progressive weakness of lower
and upper limbs and sphincter dysfunction that developed over a 6-month
period. Physical examination revealed spastic tetraplegia with 0-1/5 strength
in all muscle groups of the upper and lower limbs. There was hyporeflexia
with sensory level at C6 and impaired position sense. MRI showed a solid
intramedullary tumour from C6 to T1 vertebrae with expanded spinal cord.
Its signal intensities on both T1– and T2– weighted images was suggestive
of a fatty mass (Fig. 2). At operation, C5 to T5 cervicothoracic laminectomy
was done showing dural tube to be tense and non-pulsatile without epidural
fat. Intradural inspection demon-strated a lipomatous lesion arising dorsal
to the spinal cord (Fig. 3). The lesion was partially resected using microsurgical
technique and ultrasonic aspirator. Sufficient resection was possible
to allow dural closure without tension. Histopathological examination
showed mature fatty cells (Fig. 4). Post-operative course was uneventful
with progressive resolution of neurologic symptoms. Post-operative rehabilitation
showed good results. Twenty days after surgery, the patient started walking
with support. Control MRI showed that over 50% of the lesion was removed
(Fig. 5). Eight months later, she had normal strength in all muscle groups
of upper and lower extremities and she returned to school.
|
|
Figure 1
|
|
Figure 2
|
|
| |
Figure
1 - CT scan shows a large low density
mass (-80 HU) occupying almost all of the spinal canal area shifting
the cord. |
|
Figure
2 - Sagittal T1-weighted image
of a solid cervicothoracic intramedullary lipoma with signal characteristics
of fat. |
|
|
|
Figure 3
|
|
| |
Figure
3 - Pre-operative view showing
the intramedullary lipoma. |
|
|
|
Figure 4
|
|
Figure 5
|
|
| |
Figure
4 - Photomicrograph of excised
tissue showing some strands of peripheral nervous tissue amidst
mature fat cells (H & E x 100). |
|
Figure
5 - Control MRI showing residual
lipoma and spinal cord decompression. |
|
|
Intramedullary lipomas are very rare.4,5,7-9 They are estimated to be 1%
of all primary spinal cord tumours.7-9 Kodama, et al reviewed 186 cases
since 1876 and 99 cases were reported by Mrabet in 1992.3,4 Although there
is no consensus about the nomenclature of lipomas among pathologists, most
consider them as well-differentiated teratomas.10 Other authors call them
angiolipomas or fibrolipomas if vascularity or collagen is predominant.1,2
Strands of tissues derived from other germ cell layers are therefore common
in lipomas.11 They are not considered neoplastic, but since they are capable
of slow autonomous growth they can cause neurological symptoms due to the
confinement of the cord in the spinal canal.13 Maldevelopment with the resultant
inclusions of embryonic remains of fatty cells during the formation of the
neural tube is considered to be the most probable explanation of the origin
of these lipomas.1,9
Lipomas without spinal dysraphism affect equally males and females in the
third decade.8 Clinical manifestations depend on their location. The cervicothoracic
spine is, as in our four cases, the area most commonly involved.9 Therefore,
tetraparesis or tetraplegia is the usual presentation of this disease. Intracranial
extension into the posterior fossa is extremely rare with only 8 cases reported
in the literature.4,7 It may be said that CT and MRI provide information
that is almost pathognomonic of this condition. The diagnosis of a lipoma
can be established by CT scanning on the basis of a typical low density
as observed in our first 3 cases. MRI is the investigation of choice.4 Due
to the high rate of fat, lipoma appears to be in high signal on both T1–
and T2-weighted images.12 MRI gives further information regarding the location
and its relationship to the cord.9,12 Control MRI is necessary to evaluate
residual lipoma.12 Guidelines for their management are controversial.6 Most
lipomas cannot be re-moved from the parenchyma. Adhesion of lipoma to the
adjacent neural parenchyma is well-known and excessive removal of the mass
may impair neurological function immediately after surgery due to intraoperative
parenchymal injury. As there is no clear cleavage plane between neural structures
and lipomas, spinal cord decompression with subtotal resection should be
the main purpose of surgery.6,9 Generally, 30-40% of the tumour can be removed.
It may be associated with dural grafting.7 This procedure must be done using
operating microscope and ultrasonic aspiration.9,12 The nerve stimulator
is useful. It should be emphasised, however, that these are congenital lesions
and if they remain asymptomatic they should be left alone.8 Those that produce
signs and symptoms due to spinal cord compression mandate decompressive
surgery.
Intramedullary lipomas are rare. Signs of spinal cord compression are the
usual manifestations. MRI provides more information concerning their location,
extent and relationship to the cord. Their treatment remains controversial.
The operation must be conservative, limited to a partial resection of the
tumour and even to a decompression biopsy when the conditions deem it necessary.
| 1. |
Ammerman
BJ, Henry JM, De Girolami U, Earle KM: Intradural lipomas of the
spinal cord. A clinico-pathological correlation. J Pathol 1971,
104: 141-144 |
| 2. |
Guiffre
R, Gambacorta D: Lipoma of the spinal cord: Case report. J Neurosurg
1971, 35: 335-337 |
| 3. |
Kodama
T, Numagushi Y, Gellard FE, Sadato N: Magnetic resonance imaging
of a high cervical intradural lipoma: Comput Med Imaging Graph 1991,
15: 93-95 |
| 4. |
Kogler
A, Orsolic A, Kogler V: Intramedullary lipoma of dorsocervico-thoracic
spinal cord with intracranial extension and hydrocephalus: Paed
Neurosurg 1998, 28: 257-260 |
| 5. |
Johnson
RE, Robinson GH: Subpial lipoma of the spinal cord, Radiol 111:
121-125 |
| 6. |
Mori
K, Kamimura Y, Uchia Y, Kurisada M, Eguchi S: Large intramedullary
lipoma of the cervical cord and posterior fossa. J Neurosurg 1986,
64: 974-976 |
| 7. |
Mrabet
A, Zouari A, Mouelhi T, Khouaja F, Ghariani MT, Hila A, Hadda A:
Leslipomes intramedullaires cervicobulbaires avec revue de la litterature.
Neurochirurg 1992, 38: 309-314 |
| 8. |
Peter
D: Intramedullary lipoma. Spine 1991, 17(4): 979-981 |
| 9. |
Rahman
NU, Salih MAM, Jamjoom AH, Jamjoom ZA: Congenital intramedullary
lipoma of the dorso-cervical spinal cord with intracranial extension:
Case report. Neurosurg 1994, 34: 1081-1084 |
| 10. |
Timmer
EA, Van Rooj WJJ, Beute GN, Teepen JL: Intramedullary lipoma. Neuro
Radiol 1996, 38: 159-160 |
| 11. |
Tresser
N, Parveen T, Roessmann U: Intracranial lipomas with teromatous
elements. Arch Pathol Lab Med 1993, 117: 918-920 |
| 12. |
Wilson
JT, Shapiro RH, Wald SL: Multiple intraspinal lipomata with intracranial
extension. Paed Neurosurg 1996, 24: 5-7 |
| 13. |
Wood
BP, Harwood-Nash DC, Berger P, Goske M: Intradural spinal lipoma
of the cervical cord. AJR 1985, 145: 174-176 |
|