Original Article
Volume 5, No.1
April 2001
 Miloudi Gazzaz
 Said Derraz
 A Elouahabi
 Ali Akhaddar
 Abdelslam El Khamlichi
 Department of Neurosurgery
 Hopital des Specialites
 CHU Ibn Sina
 Rabat
 Morocco
 Correspondence:
 Dr. Abdelslam El Khamlichi
 Department of Neurosurgery
 Hopital des Specialites ONO
 CHU Ibn Sina, BP 6444
 Rabat Instituts
 Rabat
 Morocco
 Fax: (212) 7 770 212
 
 
Intramedullary lipoma without spinal dysraphism - Report of four cases

ABSTRACT


Background
: Intramedullary lipomas without spinal dysraphism are uncommon. Their treatment is still controversial.

Materials and Method: Four cases of intramedullary lipoma were treated in our department. The clinical presentation and neuroradiological findings ie. computed tomography (CT) scan in three cases and magnetic resonance imaging (MRI) in the fourth have led to a diagnosis of compressive myelopathy, secondary to an intradural lesion in the cervicothoracic region. Surgery was limited to decompression with biopsy in two cases and partial resection in the remaining two cases.

Results: The histologic examination showed mature lipoma in all cases. Outcome was good and patients completely recovered.

Conclusion: Literature review showed much controversy regarding surgical management of these tumours. The operation which is recommended must be conservative and limited to partial resection of the tumour.

Keywords: Lipoma, spinal cord and spinal dysraphism.

INTRODUCTION

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.

CASE REPORT

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.  

DISCUSSION

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.

CONCLUSION

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.

REFERENCES

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

 


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