|Year : 2018 | Volume
| Issue : 4 | Page : 180-183
Primary intradural liposarcoma of the cervical spine: Case report
Abdullahi Onimisi Jimoh, Okwunodulu Okwuoma
Division of Neurosurgery, Department of Surgery, Ahmadu Bello University Teaching Hospital, Shika Zaria, Kaduna State, Nigeria
|Date of Submission||11-Feb-2019|
|Date of Acceptance||14-Nov-2019|
|Date of Web Publication||10-Feb-2020|
Dr. Abdullahi Onimisi Jimoh
Division of Neurosurgery, Department of Surgery, Ahmadu Bello University Teaching Hospital Zaria
Source of Support: None, Conflict of Interest: None
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
| Introduction|| |
Liposarcoma is the most common soft tissue tumor and accounts for 20% of mesenchymal malignancies.,, It usually originates from primitive mesenchymal cells rather than mature adipose tissue.,, Five histological types are described: well-differentiated, dedifferentiated, myxoid, round cell, and pleomorphic.,, The pleomorphic variant is very rare and accounts for 5%–10% of liposarcoma cases.
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.,, 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. The difficulty in diagnosing PLS lies in its exceedingly varied histological appearance. Diagnosis, therefore, relies on the recognition of multivacuolated lipoblasts on histology in a high-grade PLS. 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.
| Case Report|| |
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.
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].
| Discussion|| |
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.,,,,, 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.,,,, 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.,,,,
Primary spine tumors are rare compared with metastatic disease, multiple myeloma, and lymphoma, but 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.
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. 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.,,
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.,, Like other sarcomas, PLS expresses vimentin, while S100 protein, smooth muscle actin, cytokeratins AE1/AE3, and CAM5.2 have a variable expression. Desmin is generally absent and either EMA or CD34 is negative. 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. Liposarcoma can be confounded with MFH and malignant bone mesenchymoma, mainly because of pleomorphic cell pattern. MFH is rarely seen in the spine, and only nine cases have been described 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%.,
| Conclusion|| |
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.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]
[Table 1], [Table 2]