|Year : 2022 | Volume
| Issue : 2 | Page : 120-124
Dumbbell chondrosarcoma of lumbar spine with intradural extension: A unique case
Biren Khimji Patel, Ganesh Divakar, Tobin George, Krishnakumar Kesavapisharady, Sreenath Prabha Rajeev, HV Easwer, Rajalakshmi Poyuran
Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Science and Technology, Thiruvananthapuram, Kerala, India
|Date of Submission||11-Dec-2021|
|Date of Acceptance||15-Jan-2022|
|Date of Web Publication||31-May-2022|
Department of Neurosurgery, Sree Chitra Tirunal Institute for Medical Science and Technology, Thiruvananthapuram - 695 011, Kerala
Source of Support: None, Conflict of Interest: None
Chondrosarcomas are very rare tumors of the spine constituting for only 10% of all bony tumors and 12% of primary malignant tumors of the spine. It is the third most common primary malignant bone tumor after osteosarcoma and Ewing's sarcoma, and the third most common primary malignant tumor of the spine after chordoma and osteosarcoma. The thoracic vertebrae are the most commonly affected, followed by the cervical and lumbar. These are slow-growing tumors and are usually asymptomatic until late in their course when they cause neurological deficits by compression on the thecal sac, spinal cord, or nerve roots. As is the case for all malignant spinal tumors, en bloc resection without contamination or violation of the reactive zone (pseudocapsule) is the most appropriate surgical strategy, but in some cases, this is impossible due to unacceptable morbidity because of close proximity to critical neurovascular structures or multicompartmental location of the tumor. Here, we describe a case of L5 chondrosarcoma arising from the left pedicle with significant intradural and extraspinal components. Intradural extension of chondrosarcomas, or for that matter any malignant spinal tumor, has never been reported in literature.
Keywords: Intradural, rare case, spinal chondrosarcoma
|How to cite this article:|
Patel BK, Divakar G, George T, Kesavapisharady K, Rajeev SP, Easwer H V, Poyuran R. Dumbbell chondrosarcoma of lumbar spine with intradural extension: A unique case. J Spinal Surg 2022;9:120-4
|How to cite this URL:|
Patel BK, Divakar G, George T, Kesavapisharady K, Rajeev SP, Easwer H V, Poyuran R. Dumbbell chondrosarcoma of lumbar spine with intradural extension: A unique case. J Spinal Surg [serial online] 2022 [cited 2022 Jul 7];9:120-4. Available from: http://www.jossworld.org/text.asp?2022/9/2/120/346362
| Introduction|| |
Chondrosarcoma is a rare primary malignant bone tumor after osteosarcoma and Ewing's sarcoma with an incidence of 2%–12% in various series. The thoracic spine is most frequently involved, followed by the cervical and lumbar region. Tumor can originate from any of the three growth centers of the vertebra with the incidence of about 5% from the vertebral body, 40% from the posterior element, and 45% from both, with intradural extension being extremely rare. We report a rare case of spinal chondrosarcoma which was located in both extradural as well as intradural compartments at L5 level and extending into left L5–S1 facet joint.
| Case Report|| |
72-year-old gentleman, a known diabetic with good glycemic control, presented to us with insidious onset episodic dull aching pain and numbness of the right gluteal region for the past 1 year, aggravated on prolonged standing and walking for over half a kilometer. General physical and neurological examination was unremarkable except for mild tenderness in the right lower back on deep palpation.
Computed tomography (CT) of the spine [Figure 1] was suggestive of an irregular lytic expansile lesion with soft-tissue component and multiple irregular foci of “ring and arc” type of calcification predominantly arising from the posterior elements of the left side of L5 vertebra.
|Figure 1: Computed tomography and magnetic resonance imaging images: axial (upper left) and right parasagittal computed tomography images (upper right) show a multilobulated expansile lytic lesion arising from the left pedicle of L5 and extending into the intraspinal as well as paraspinal compartments. The lesion shows punctate and curvilinear calcifications (ring and arc pattern) characteristic of an aggressive chondroid lesion. Sagittal (lower left) T2-weighted images show a heterogeneous lesion which is a predominantly hyperintense (hyperintense as compared with the signal intensity of adjacent paraspinal muscles) with heterogeneous low- and high-signal intensities, suggesting calcified as well as hyalinized parts, respectively. Gd-enhanced axial image (lower right) shows diffuse heterogeneous but prominent peripheral enhancement|
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Magnetic resonance imaging (MRI) of the spine [Figure 1] was suggestive of a well-defined heterogeneous bony matrix mass lesion which was hypointense on T1 and predominantly hyperintense on T2 sequence of size 34 mm × 35 mm × 34 mm arising from the left pedicle and lamina of L5 vertebra with extension into the vertebral body and superior aspect of the sacral end plate, central spinal canal and paraspinal area. The lesion was compressing both traversing and exiting nerve roots, and there was heterogeneous contrast enhancement.
Based on the typical imaging features, a diagnosis of chondrosarcoma arising from the left L5 pedicle was made. Differentials, including other malignant bony lesions such as osteosarcoma, giant cell tumor, and metastasis, were not considered in view of the typical imaging appearance and absence of other lesions in the neuraxis or elsewhere.
The patient was staged as Weinstein–Boriani–Biagini (WBB) Type 1–4 E and Tomita Type 6 [Figure 2]. After a detailed workup, we planned for a radical excision of the lesion with wide margins; however, the patient did not consent for a morbid procedure such as total en bloc excision or nerve root sacrifice. It was therefore decided to proceed with a gross-total resection of the lesion with neural preservation and instrumented fusion.
|Figure 2: Right parasagittal CT image (a) and Axial post contrast CT image (b) show a multilobulated expansile lytic lesion arising from the left pedicle of L5 and extending into the intraspinal as well as paraspinal compartments. The lesion shows punctate and curvilinear calcifications (ring and arc pattern) characteristic of an aggressive chondroid lesion. Artist's representation of the tumor showing the multicompartmental extension, helpful in staging and planning for surgery (c and d)|
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The patient was positioned prone, and L4–S1 with the transverse processes were exposed on both sides. The tumor was seen to be arising from the left lamina and pedicle of the L5 vertebra and was extending into the left L5–S1 facet joint. The tumor was pale gray in color, very firm in consistency (cartilaginous feel), and highly vascular with interspersed bony tissue. There was significant extension inside the dura which was smooth and thinned out at that level [Figure 3]. Intradural extension of the tumor was most probably due to the chronic compression of the dura by the tumor that led to the defect in the dura. The tumor was excised in a piecemeal manner. Total excision of both extradural and intradural components was achieved. Wide excision of the adjacent dura was done and the defect was repaired using an artificial dural substitute and further reinforced with oxidized regenerated cellulose and fibrin glue. Instrumented stabilization was done using transpedicular screws and rods.
|Figure 3: Intra-operative microphotograph of the tumor shown in figure 3(a) with extradural part of the tumor designated by arrow. Figure 3(b) shows intradural extension of the tumor displacing the cauda equina roots with intradural part of the tumor designated by arrow|
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Microscopic examination report of both the intradural and extradural components showed a cartilaginous tumor exhibiting lobules of atypical chondrocytes in a chondroid and myxoid matrix. The tumor showed increased cellularity, pleomorphism, and focal calcification. The neoplastic cartilage was infiltrating the adjacent adipose and fibrocollagenous tissue. These features were suggestive of conventional chondrosarcoma, WHO Grade II [Figure 4]. He was advised adjuvant radiotherapy but did not consent for the same. Postoperatively, the patient had a significant decrease in pain sensation and was asymptomatic at 2½ years follow-up, with no residual or recurrence of tumor on imaging [Figure 5].
|Figure 4: Histopathology – Grade II conventional chondrosarcoma composed of lobules of atypical chondrocytes in a chondroid and myxoid matrix (a and b) with focal calcification (A-arrows). The tumor cells are positive for S-100 protein (c) and there is adipose tissue infiltration (D, arrow). [a-d: Hematoxylin and eosin stain, c: Immunoperoxidase; Magnification = scale bar (A: 200 μm; B and C: 50 μm; D: 100 μm)|
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|Figure 5: Postoperative spinal radiographs and magnetic resonance imaging images at two and half years follow-up: lateral (a) radiograph of lumbar spine show instrumented L4–S1 vertebral levels. Postoperative sagittal (b), axial magnetic resonance imaging (c), and Gd-enhanced axial magnetic resonance imaging (d) showing a loculated cerebrospinal fluid collection at the site of dural defect, no evidence of residual tumor.|
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| Discussion|| |
Chondrosarcomas are mesenchymal, nonmeningothelial tumors characterized by the formation of cartilage matrix, which can occur along the mobile spine and are composed of chondroid matrix mineralization of “ring and arcs” and a nonmineralized portion of hyaline cartilage., Chondrosarcomas are mainly seen in the thoracic region (approximately 60%), followed by the lumbar spine (20%–39%) and cervical spine (19%–20%). They are considered extremely rare tumors with an incidence of approximately 1 per 200,000 per year in the general population. Most chondrosarcomas are diagnosed between 30 and 70 years of age and seen slightly more in males than females.
The most common presenting symptom in chondrosarcoma is pain. Palpable mass and neurologic deficit are found in about 50%of the cases.
Chondrosarcomas are known to be resistant to radiotherapy and chemotherapy making surgical excision the primary modality of therapy. Furthermore, the surgical outcome depends on the margin of excision achieved.,
Chondrosarcoma has traditionally been classified into conventional and variant types. The variant types of chondrosarcoma include clear cell type which is less aggressive and the high-grade mesenchymal and dedifferentiated tumors associated with poor prognosis. Conventional chondrosarcoma forms approximately 85% of all chondrosarcomas and it is further classified into primary (85%) and secondary (15%). The primary chondrosarcoma arises within the bone and then invades outside through the cortex with a large soft-tissue mass. A secondary chondrosarcoma usually develops on the surface of the bone because of malignant transformation within the cartilage cap of a preexisting benign tumor such as osteochondroma., Secondary chondrosarcomas tend to be of a lower grade and exhibit a better prognosis than primary tumors.
Histologic grading indicates the biological behavior of these tumors and is directly connected with prognosis and the risk of metastases. Grade I tumors have low cellularity, lack of pleomorphism, contain a rich hyaline cartilage matrix, and rarely metastasize. In contrast, Grade III chondrosarcomas are extremely cellular with pleomorphism, mitotic figures, and mucomyxoid matrix areas with occurrence of metastases in 70% of patients. Grade II chondrosarcoma has some of the characteristics of both Grade I and Grade III.
The radiological features of chondrosarcomas differ according to the histologic grade. Moth-eaten destruction and interrupted periosteal reaction are characteristics of high-grade tumors. The presence of “ring and arc” calcification in the tumor matrix correlates with a high grade of differentiation. The differential diagnosis depends on the presence of calcifications. Radiological criteria for the diagnosis of chondrosarcoma have been described as deep endosteal scalloping (>2/3 of cortical thickness), cortical disruption, periosteal reaction, soft-tissue mass, and intense radionuclide uptake. CT and MRI are needed to find out the extraosseous extension. Chondrosarcoma shows a hypointense to isointense signal on T1-weighted MRI. On T2-weighted MRI, hyperintense signals correspond to areas of high water content of hyaline cartilage, and hypointense signals represent mineralization areas.,, In contrast-enhanced MRI, non-enhancing intralesional regions represent hyaline cartilage, cystic mucoid tissue, or necrosis. It may reveal a peripheral ring of enhancement or heterogeneous enhancement of the entire tumor.
Enneking staging system has proved to be beneficial for surgical planning of many spinal neoplasms, particularly chondrosarcoma. Tumors are classified based on histologic grade and presence of metastasis, where Stage I is low grade, Stage II is high grade, and Stage III indicates the presence of distant metastasis, and by anatomic site, where intracompartmental is Subtype A and extracompartmental is Subtype B.
The WBB staging has also proved to be beneficial in defining the extent of the lesion. According to this classification, the vertebral body is topographically divided into twelve zones similar to the clock hours and five layers beginning from the paravertebral extraosseous region to the dural involvement as A to E, where A is extraosseous (soft tissues), B is intraosseous (superficial), C is intraosseous (deep), D is extraosseous (extradural), and E is extraosseous (intradural) and M is metastasis.,
Tomita staging is considered to be a new modified version of Enneking surgical staging, and it is described as Stage I for lesion confined within the vertebral body, Stage II for the lesion extending to the pedicle, Stage III for the lesion extending to the whole vertebra, Stage IV for the lesion extending to the epidural space, Stage V for lesion extending to the paravertebral space, Stage VI for the lesion with extension to paravertebral space and neighboring vertebral levels, and Stage VII for lesion extending to multiple non-contiguous levels.,,
En bloc resection surgery of tumor forms a central tenet in the management of chondrosarcoma of the spine owing to the inherent resistance of chondrosarcoma to conventional radiation and chemotherapy. Surgical staging of tumor is absolutely essential before surgery so as to select the best surgical strategy to tackle tumor as well as to assess the need for adjuvant therapy. Achievement of a wide tumor-free surgical margin should always be the primary goal while operating such a tumor. Furthermore, the surgeon should aim at preserving or even improving functionality, relieving pain, and controlling local tumor recurrence, thus facilitating prolonged survival.
In primary spinal tumors, the most important predictor of local recurrence is residual tumor tissue. Hence, en bloc resection is considered to be the standard of care. However, the feasibility of en bloc resection or its prognosis in spinal chondrosarcoma with an intradural extension like in our case has not been described in literature. In such rare cases, gross-total resection in a piecemeal manner with a tumor-free margin is the best possible treatment that can be provided to the patient. The use of spinal instrumentation helps the surgeon to be more aggressive in his resection which will ultimately lower the chances of recurrence of the tumor and improve the functional outcome as well.
Radiotherapy is frequently used in patients with inadequate margins; however, survival for these patients still remains low. Boriani et al. did a systematic multicenter review of patients with low-grade malignant tumors of the spine and concluded that radiation as an adjunct to incomplete excision of the mass has little beneficial effect on the outcome and locoregional tumor control. Hence, radiation as a primary treatment for chondrosarcoma of the spine is not indicated.
Chemotherapy has not proved to affect the outcome at all in spinal chondrosarcoma except in cases of mesenchymal chondrosarcoma which were found to be responsive to doxorubicin-based regimen.,,
| Conclusions|| |
Primary chondrosarcomas of the lumbar vertebrae are extremely rare in the elderly and are associated with a high risk of morbidity and mortality. Moreover, a contiguous intradural extension of such tumors has not been reported in literature. The risks of potential immediate postoperative morbidity and long-term neurological deficits associated with neurovascular compromise should be carefully weighed against the improved recurrence-free survival associated with an aggressive en bloc resection which is the standard of care for such lesions. We feel that spinal chondrosarcomas in the elderly, particularly those with rare intradural invasion like in our case, where en bloc resection is not feasible without significant morbidity, should undergo gross-total piecemeal resection with the aid of spinal instrumentation to lessen the chances of recurrence and postsurgical instability, and improve survival and quality of life.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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