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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 8  |  Issue : 4  |  Page : 180-183

Primary intradural liposarcoma of the cervical spine: Case report


Division of Neurosurgery, Department of Surgery, Ahmadu Bello University Teaching Hospital, Shika Zaria, Kaduna State, Nigeria

Date of Submission11-Feb-2019
Date of Acceptance14-Nov-2019
Date of Web Publication10-Feb-2020

Correspondence Address:
Dr. Abdullahi Onimisi Jimoh
Division of Neurosurgery, Department of Surgery, Ahmadu Bello University Teaching Hospital Zaria
Nigeria
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ais.ais_9_19

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  Abstract 


Liposarcoma is the commonest soft tissue sarcoma. It accounts for up to 20% of soft tissue sarcoma. Liposarcoma of the central nervous system is very rare. Pleomorphic liposarcoma (PLS) variant is a rare malignant soft tissue tumor comprising 5%-15% of liposarcomas. It is characterized by its high invasiveness. Few cases have been reported in the literature. We report a case of a pleomorphic variety of cervical spinal cord in a male that was operated with good outcome. The clinical presentation, management, and outcome in a case of primary PLS of the cervical spine, and a review of the literature, are presented. A 42-year-male presented with insidious upper back pain. Magnetic resonance imaging revealed an intradural, extramedullary lesion compressing the spinal cord adjacent to C3/C4. Urgent decompressive laminectomy with gross total resection was performed. Histopathology revealed high-grade PLS. The patient was followed-up to one year and has remained stable symptomatically. Surgical resection is a modality of treatment with adjuvant therapies. Of the few reported cases ours has a good outcome up to the one year follow-up. It should be considered in intradural cord lesion with the characteristic MRI finding.

Keywords: Cervical, intradural, liposarcoma, spinal cord


How to cite this article:
Jimoh AO, Okwuoma O. Primary intradural liposarcoma of the cervical spine: Case report. Arch Int Surg 2018;8:180-3

How to cite this URL:
Jimoh AO, Okwuoma O. Primary intradural liposarcoma of the cervical spine: Case report. Arch Int Surg [serial online] 2018 [cited 2020 Apr 7];8:180-3. Available from: http://www.archintsurg.org/text.asp?2018/8/4/180/278027




  Introduction Top


Liposarcoma is the most common soft tissue tumor and accounts for 20% of mesenchymal malignancies.[1],[2],[3] It usually originates from primitive mesenchymal cells rather than mature adipose tissue.[3],[4],[5] Five histological types are described: well-differentiated, dedifferentiated, myxoid, round cell, and pleomorphic.[1],[2],[5] The pleomorphic variant is very rare and accounts for 5%–10% of liposarcoma cases.[6]

Pleomorphic liposarcoma (PLS) is a high-grade tumor with the highest prevalence between the ages of 60 and 70 years, and it affects both sexes equally. It is the rarest form of liposarcoma.[4],[5],[7] Three main histological subtypes have been described. High-grade pleomorphic Malignant Fibrous Histiocytoma (MFH)-like sarcoma, cellular spindle cell neoplasm, and PLS characterized by epithelioid morphology.[3] The difficulty in diagnosing PLS lies in its exceedingly varied histological appearance.[8] Diagnosis, therefore, relies on the recognition of multivacuolated lipoblasts on histology in a high-grade PLS.[9] However, finding of a lipoblast, though the hallmark of any adipocytic lesion, is not sufficient for diagnosing LPS as there are a number of benign astrocytic lesions with similar feature. In addition, its absence does not exclude a liposarcoma.[5]


  Case Report Top


A 42-year-old man presented to our neurosurgical clinic on referral with a 14-month history of upper back pain, insidious in onset, dull, and aching radiating to the left upper limb and upper chest wall. It was ameliorated by analgesics. There was associated weakness and paraesthesia of the left upper limb. There were no bisphincteric dysfunction, antecedent history of trauma or history of chronic cough, or contact. There were no neurocutaneous symptoms or weight loss.

He is a known hypertensive with a history of ischemic stroke.

Physical examination revealed a healthy looking man with normal mental state, cranial nerves, and meninges. He had normal muscle bulk. The muscle power was 4+ on the left upper and lower limbs, and there was generalized hypertonia and hyperreflexia with bilateral extensor plantar response. Light touch and pin prick were impaired on the left upper limb, trunk, and lower limb.

Magnetic Resonance Imaging (MRI) of the cervical spine showed an intradural extramedullary hyperintense lesion on sagittal T1 and T2 [Figure 1]a and [Figure 1]b and T2 axial [Figure 1]c.
Figure 1: (a) T1WI ( Sagittal) of the cervical spine showing syringomyelia above C5. (b) T2WI (Sagittal) showing a heterogenous dorsal intradural, extramedullary space occupying lesion at the level of C6 with syringomyelia above and below the lesion.(c) shows an axial slice T2WI through the lesion at C6

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The patient had C5-C7 laminectomies, durotomy, and gross total tumor resection. The findings included a bulging dura with a yellowish nonencapsulated intradural extramedullary [Figure 2]a and [Figure 2]b. And also surgical specimen, [Figure 2]c.
Figure 2: (a and b) intraoperative photograph, (c) operative specimen

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The histology revealed a diffusely arranged infiltrative tumor composed of pleomorphic round, spindle, and polygonal cells, exhibiting moderate to marked nuclear atypia and numerous mitoses with multinucleated and abnormal forms. Lipoblasts, including giant forms, were scattered within the well-vascularized fibromyxoid stroma. Photomicrograph of the histological specimen is shown in [Figure 3]a and [Figure 3]b.
Figure 3: (a) photomicrographs of the histology of the specimen.(b) photomicrographs of the histology of the specimen

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Adjuvant radiotherapy was recommended as part of treatment.

One year postoperatively, the patient has had significant resolution of symptoms. However, he has occasional mild neuropathic pain on the left upper and lower limbs. This is comparism to other reported case [Table 1].
Table 1: Comparison of treatments applied in each case

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  Discussion Top


PLS is very rare and accounts for 5%–10% of lipomatous tumors. PLS could occur in various organs, the most common sites being the proximal extremities, especially the thigh.[3],[9],[10],[11],[12],[13] Other, less common, sites include the retroperitoneum, abdominal wall, chest wall, mesentery, pelvic cavity, spermatic cord, mediastinum, parietal pleura, pericardium, and the head and neck region.[6],[14],[15],[16],[17] However, primary involvement of the spine has not been reported, although a few cases have been described in the epidural space, the foramen of T7, and as metastasis.[1],[12],[18],[19],[20]

Primary spine tumors are rare compared with metastatic disease, multiple myeloma, and lymphoma, but[5] there are several case reports regarding primary spinal involvement of liposarcoma in the literature [Table 2]. This is perhaps the first case of primary intradural PLS reported in literature.
Table 2: Reported cases of Primary intradural spinal cord liposarcoma

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The patient has been symptoms free after surgery.

The clinical presentation is variable and can mostly be insidious before the signs of neural compression are evident. Therefore, magnetic resonance imaging, as done for our patient, is highly valuable in the imaging assessment of spinal tumors.[21] PLS mostly appears in the middle age, as our index case, and older patients, with equal sex distributions. However, some cases have been reported in younger patients, the youngest recorded being 8 years old.[22],[23],[24]

The diagnosis of PLS in our case was histological. Immunohistochemistry was not done. However, PLS has a nonspecific immunohistochemical profile and special stains maybe required to identify the presence of intracellular fat, exclude other substances such as mucin or glycogen, and eliminate other diagnoses.[9],[10],[11] Like other sarcomas, PLS expresses vimentin, while S100 protein, smooth muscle actin, cytokeratins AE1/AE3, and CAM5.2 have a variable expression.[9] Desmin is generally absent and either EMA or CD34 is negative.[9] Immunohistochemistry may be relevant in differential diagnosis: morphological features and positivity for vimentine eliminate the possibility of hemopathy such as signet ring lymphoma or clear cell myeloma. Clear cell sarcoma consistently exhibits immunoreactivity for S100 protein. According to Springfield, it is difficult to differentiate PLS from malignant fibrous histiocytoma.[13] Liposarcoma can be confounded with MFH and malignant bone mesenchymoma, mainly because of pleomorphic cell pattern.[25] MFH is rarely seen in the spine, and only nine cases have been described[26] and lipoblasts are not histological features.

The primary intradural location of PLS in this patient is quite unusual as there are no fat cells in the central nervous system (CNS). However, PLS is derived from mesenchymal tissues and the meninges, embryologically, are mesenchymal in origin.

Pleomorphic sarcomas are very aggressive: 30%–50% may metastasize and mortality may approach 40%–50%.[3],[27]


  Conclusion Top


Intradural pleomorphic sarcoma of the spine is rare. Intradural origin is a possible primary CNS site. Treatment options are still evolving; however, surgical resection remains the mainstay of treatment.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Cho SH, Rhim SC, Hyun SJ, Bae CW, Khang SK. Intradural involvement of multicentric myxoid liposarcoma. J Korean Neurosurg Soc 2010;48:276-80.  Back to cited text no. 1
    
2.
Lmejjati M, Loqa C, Haddi M, Hakkou M, BenAli SA. Primary liposarcoma of the lumbar spine. Joint Bone Spine 2008;75:482-5.  Back to cited text no. 2
    
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Dei Tos AP. Liposarcoma: New entities and evolving concepts. Ann Diagn Pathol 2000;4:252-66.  Back to cited text no. 3
    
4.
Hamlat A, Saikali S, Gueye EM, Le Strat A, Carsin-Nicol B, Brassier G. Primary liposarcoma of the thoracic spine: Case report. Eur Spine J 2005;14:613-8.  Back to cited text no. 4
    
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Dei Tos AP. Lipomatous tumor. Curr Diagn Pathol 2001;7:8-16.  Back to cited text no. 5
    
6.
Gebhard S, Coindre JM, Michels JJ, Terrier P, Bertrand G, Trassard M, et al. Pleomorphic liposarcoma: Clinicopathologic, immunohistochemical, and follow-up analysis of 63 cases: A study from the French Federation of Cancer Centers Sarcoma Group. Am J Surg Pathol 2002;26:601-16.  Back to cited text no. 6
    
7.
de Moraes FB, Cardoso AL, Tristão NA, Pimenta WE Jr, Daher S, de Souza Carneiro S, et al. Primary liposarcoma of the lumbar spine: Case report. Rev Bras Ortop 2012;47:124-9.  Back to cited text no. 7
    
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Schwartz A, Shuster M, Becker SM. Liposarcoma of bone. Report of a case and review of literature. J Bone Joint Surg Am. 1970;52:171-7.  Back to cited text no. 8
    
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Oliveira AM, Nascimento AG. Pleormorphic liposarcoma. Semi Diagn Pathol 2001;18:274-85.  Back to cited text no. 9
    
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Enzinger FM, Weiss SW. Soft Tissue Tumors. 3rd ed. St Louis: C.V. Mosby; 1988. p. 346-82.  Back to cited text no. 10
    
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Enzinger FM, Winslow DJ. Liposarcoma. A study of 103 cases. Virchows Arch 1962;103:367-88.  Back to cited text no. 11
    
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Reszel PA, Soule EH, Coventry MB. Liposarcoma of the extremities and limb girdles. J Bone Joint Surg Am 1996;48:229-44.  Back to cited text no. 12
    
13.
Springfield D. Liposarcoma. Clin Ortop 1993;289:50-7.  Back to cited text no. 13
    
14.
Mumert ML, Walsh MT, Jensen EM, Jensen RL. Pleomorphic liposarcoma originating from intracranial dura. J Neurooncol 2010;97:149-53.  Back to cited text no. 14
    
15.
Wang L, Ren W, Zhou X, Sheng W, Wang J. Pleomorphic liposarcoma: A clinicopathological, immunohistochemical and molecular cytogenetic study of 32 additional cases. Pathol Int 2013;63:523-31.  Back to cited text no. 15
    
16.
Wang JG, Wei ZM, Liu H, Li YJ. Primary pleomorphic liposarcoma of pericardium. Interact Cardiovasc Thorac Surg 2010;11:325-7.  Back to cited text no. 16
    
17.
Morales-Codina AM, Martin-Benlloch JA, Corbellas AM. Primary pleomorphic liposarcoma of the spine. Case report and review of literature. Int J Surg Case Rep 2016;25:114-9.  Back to cited text no. 17
    
18.
Ogose A, Hotta T, Inoue Y, Sakata S, Takano R, Yamamaura S. Myxoid liposarcoma metastatic to the epidural space without bone involvement. Report of two cases. Jpn J Clin Oncol 2001;21:447-9.  Back to cited text no. 18
    
19.
Turanli S, Ozer H, Ozyurekoglu T, Cakiroglu E. Liposarcoma in the epidural space. Spine 2000;25:1733-5.  Back to cited text no. 19
    
20.
Boriani S, Weinstein JN, Biagini R. Spine update. Primary bone tumors of the spine. Terminology and surgical staging. Spine 1997;22:1036-44.  Back to cited text no. 20
    
21.
Flemming DJ, Murphy MD, Carmichael BB, Bernard SA. Primary tumors of the spine. Semin Musculoskelet Radiol 2000;4:299-320.  Back to cited text no. 21
    
22.
Rudzinski E, Mawn L, Kuttesch J, Wushensky C, Wills M. Orbital pleomorphic liposarcoma in an eight-year-old boy. Pediatr Dev Pathol 2011;14:339-44.  Back to cited text no. 22
    
23.
Ahmed Z Shah HU, Yaqoob N, Muzaffar S. Pleormorphic liposarcoma in a ten-year-old child. J Pak Med Assoc 2004;54:533-4.  Back to cited text no. 23
    
24.
Saeed M, Plett S, Kim GE, Daldrup-Link H, Courtier J. Radiological-pathological correlation of pleomorphic liposarcoma of the anterior mediastinum in a 17 year old girl. Paediatric Radiol 2010;40:68-70.  Back to cited text no. 24
    
25.
Simmon M, Springfield D. Surgery for Bone and Soft Tissue Tumors. Philadelphia, PA: Lippincott-Raven publishers; 1997.  Back to cited text no. 25
    
26.
Sturm PF, Abramvitz J, Wagner C, Ferguson R, Walker S. Malignant fibrous histiocytoma of the spine. Spine 1992;17:975-7.  Back to cited text no. 26
    
27.
Downes KA, Goldblum JR, Montgomery EA, Fisher C. Pleomorphic liposarcoma: A clinicopathologic analysis of 19 cases. Mod Pathol 2001;14:179-84.  Back to cited text no. 27
    


    Figures

  [Figure 1], [Figure 2], [Figure 3]
 
 
    Tables

  [Table 1], [Table 2]



 

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