• Users Online: 206
  • Print this page
  • Email this page


 
 Table of Contents  
CASE REPORT
Year : 2019  |  Volume : 2  |  Issue : 1  |  Page : 102-107

Sacral chordoma with degenerative spondylolisthesis and upper lumbar disc herniation


1 Department of Orthopaedic and Spine Surgery, Zydus Hospitals and Healthcare Research Pvt. Ltd, Ahmedabad, Gujarat, India
2 Department of Surgery, Zydus Hospitals and Healthcare Research Pvt. Ltd, Ahmedabad, Gujarat, India

Date of Web Publication11-Jan-2019

Correspondence Address:
Dr. Hitesh N Modi
Department of Spine Surgery, Zydus Hospitals and Healthcare Research Pvt. Ltd, Thaltej, Ahmedabad, Gujarat
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/isj.isj_24_17

Get Permissions

  Abstract 


Sacral chordoma is a rare condition requiring multidisciplinary approach for management. Here, we report a 72-year-old male patient who was diagnosed with sacral chordoma with L2–L3 disc herniation and L5–S1 degenerative spondylolisthesis and L1 body fracture. The patient was first managed by discectomy L2–L3 with D12–L3 decompression and fixation. Sacral chordoma excision was done 10 months later. The chordoma was excised by anterior laparoscopic resection and mobilization of tissues from the tumor followed by posterior sacrectomy with L5–S1 decompression and extension of fixation in a single stage. Proline mesh was used to support the colon posteriorly. This was further complicated by proximal junction fracture due to fall which was further managed by proximal extension of the rod-screw construct. The patient became symptom free without any radiotherapy or chemotherapy and is able to walk independently, two years following the primary surgery without recurrence of tumor.

Keywords: Degenerative spondylolisthesis, laparoscopic adhesiolysis, posterior sacrectomy, sacral chordoma, upper lumbar disc herniation


How to cite this article:
Goel SA, Modi HN, Desai YJ, Patel B. Sacral chordoma with degenerative spondylolisthesis and upper lumbar disc herniation. Indian Spine J 2019;2:102-7

How to cite this URL:
Goel SA, Modi HN, Desai YJ, Patel B. Sacral chordoma with degenerative spondylolisthesis and upper lumbar disc herniation. Indian Spine J [serial online] 2019 [cited 2019 Jul 21];2:102-7. Available from: http://www.isjonline.com/text.asp?2019/2/1/102/249895




  Introduction Top


Sacral tumors are rare conditions, but whenever encountered, their management is complex and requires a team effort.[1] Primary benign and malignant tumors of the sacrum are 2%–4% of all primary bone neoplasms and 1%–7% of all primary spinal tumors.[2] The most common sacral tumors are chordoma, representing 40% of all primary sacral neoplasms.[3] Surgical treatment of sacral chordoma is one of the most challenging fields in spine because of the complicated anatomy of the sacral site. In majority of the cases, only radical treatment such as sacrectomy may give optimal control.[1] Whenever attempted, it is usually done in multiple phases in order to release the chordoma from all sites. However, several problems such as bowel, bladder, and sexual dysfunction; infection; blood loss; and pelvic instability are frequently encountered after sacrectomy. It has also been reported that patients with such tumors have a poor prognosis.[4],[5] Here, we report a unique case of sacral chordoma with degenerative spondylolisthesis and upper lumbar disc herniation which was managed with a view of patient's requirement. Our case represents a treatment dilemma when such case is presented. It also focuses on the importance of neuromonitoring and bone scalpel while resecting such tumors.


  Case Report Top


A 72-year-old male patient presented to the outpatient department with complaints of low back pain for 2 weeks with severe left lower limb radicular pain. The pain increased on changing position and coughing. He had imbalance while walking along with weakness and numbness in the left knee and anterior thigh region. There were no bowel and bladder complaints. The patient was on antiplatelet medicines for 5 years due to previous history of coronary artery bypass graft. He was a known case of diabetes mellitus and asthma. On physical examination, the patient had bilateral straight leg raising of >70°. Femoral nerve stress test was positive on the left side. Power in left knee and hip was 2/5 along with hypoesthesia in the left anterior thigh and knee. Knee reflex was absent on the left side, but Babinski was flexor bilaterally.

The radiographs and magnetic resonance imaging (MRI) of the lumbar spine were advised. MRI showed old fracture with kyphosis at L1 region along with spondylolisthesis at L5–S1 on X-ray. MRI also showed severe cord compression at L2–L3 level due to large herniation of the disc on the left side along with a growth in the anterior part of sacrum. It also showed a large, well-encapsulated mass in front of the sacrum [Figure 1]a,[Figure 1]b,[Figure 1]c,[Figure 1]d,[Figure 1]e. Computed tomography (CT)-guided biopsy of the growth was performed and the diagnosis of chordoma was confirmed.
Figure 1: Preoperative magnetic resonance imaging and X-ray of the patient. (a) Sagittal magnetic resonance imaging showing L1 fracture and L2–L3 disc herniation with canal stenosis. (b) X-ray LS spine lateral view showing L5–S1 spondylolisthesis (c) Myelogram indicating complete block at L2–L3 level. (d) T1WI axial images at L2–L3 showing huge disc herniation causing compression of cord. (e) Growth seen in anterior part of the sacrum which was confirmed as chordoma with computed tomography-guided biopsy

Click here to view


The patient was advised surgical resection of the tumor along with decompression and posterior fixation of the spine simultaneously with detailed discussion on both pathologies. However, since he predominantly had symptoms of L2–L3 disc, his main requirement was to relieve pain and weakness in the left lower limb. The patient opted for a surgical procedure of less magnitude and hence was operated with D12–L3 decompression and fixation with discectomy at L2–L3 level [Figure 2]a,[Figure 2]b,[Figure 2]c. A large fragment of sequestrated disc was removed which was compressing the cord. Immediately after surgery, his leg pain disappeared and weakness improved in two weeks time. The patient was given anti-osteoporotic regimen in the postoperative period. Although he was able to perform all his duties normally including driving vehicle, he was informed about warning signs such as worsening of radicular pain as well as bowel and bladder symptoms. He was kept on regular follow-up.
Figure 2: (a and b) Postoperative X-ray after D12–L3 decompression and fixation of spine. The patient was pain free and had improved neurology after the procedure. (c) Postoperative follow-up sagittal magnetic resonance imaging films showing decompression at L2–L3 level with fixation of spine from D12 to L3

Click here to view


The patient was apparently well for nearly 10 months following the primary surgery. He then presented with severe radicular pain of the right lower limb along with tingling and numbness and his claudication distance at that stage was <50 m. He had developed urinary retention for which he underwent catheterization at a local hospital. On physical examination, the power in bilateral lower limbs was 4/5, the reflexes were of 2+ grade, and sensations in both lower limbs were normal. There was a palpable mass encountered on per-rectal examination with slightly decreased rectal tone. MRI of the lumbosacral spine was advised and it showed a large, well-defined, altered intensity lesion involving lower sacral and coccygeal segment distal to S3–S4 level with exophytic presacral component [Figure 3]a and [Figure 3]b. On comparison with the previous MRI, there was a marginal increase in the size of the lesion. Spondylolisthesis at L5–S1 also showed foraminal stenosis with compression of L5 nerve root. CT scan also displayed large sacral tumor which had similar findings as in MRI [Figure 3]c.
Figure 3: (a-c) Magnetic resonance imaging of lumbosacral region, 6 months after the primary procedure. The mass in the anterior part of the sacrum increased in size and was even palpable by per-rectal examination. (c) Computed tomography scan images of sacrum showing tumor mass anterior to sacrum behind the colon

Click here to view


Considering the patient's present situation, we decided to resect the tumor along with decompression and fixation of the L5–S1 region. The surgery was performed under neuromonitoring to reduce the risk of nerve injury during the procedure. We also planned to monitor sacral fibers to reduce the chances of bowel and bladder dysfunction postoperatively. Adhesions anteriorly with abdominal structures are major challenge and therefore, anterior laparoscopic adhesiolysis and isolation of tumor from other structures was planned. Laparoscopic surgeon did adhesiolysis and isolation of tumor with identification of intact tumor wall under laparoscopic vision, and the entire tumor was removed from the back by performing posterior sacrectomy [Figure 4]a,[Figure 4]b,[Figure 4]c,[Figure 4]d followed by L5–S1 decompression and extension of fusion performed in a single stage [Figure 4]e. Proline mesh was used to support the colon after tumor excision. Bone scalpel was used to perform sacrectomy below the S1 level without damaging the tumor wall or other soft-tissue structures. During the entire posterior surgery, intraoperative neuromonitoring along with monitoring of sacral fibers was performed which was maintained throughout and improved during decompression. The patient became symptom free and was able to walk independently without support. His catheter was removed after 2 weeks of surgery and he was able to pass urine without any difficulty. His condition and neurology improved completely after 6 weeks of surgery and he became independent. Three months postoperatively, his CT scan and MRI showed complete excision of tumor without any signs of recurrence of tumor.
Figure 4: Intraoperative images of sacrectomy and chordoma excision. (a) Sacrectomy using a bone scalpel. (b) Visualization of chordoma. (c) After chordoma excision. (d) Excised sacral chordoma. (e) Postoperative X-ray showing D12–S1 fixation and excision of sacral tumor below S2

Click here to view


However, 4 months later, he presented with mechanical back pain and imbalance while walking. He had recent history of sudden fall while walking. The neurology in both lower limbs was normal and bowel and bladder functions showed no impairment. On further investigation, it was found that he had compression fracture with junctional compression at proximal level of surgery [Figure 5]a and [Figure 5]b. Two weeks of conservative treatment neither relieved his pain nor improved his imbalance. Therefore, he was re-operated by decompression at proximal level and extension of the spinal fixation [Figure 5]c till T5 level. The patient started walking 1 month after the second procedure. The independence in walking was attained gradually with physiotherapy and exercises. Considering his proximal-level compression fracture, putting him on teriparatide injection was given a thought. However, after consulting few hematologists and oncophysicians and with a history of sacral chordoma, it was not started due to a remote possibility of its potential to cause osteosarcoma.[6],[7] He was continued with bisphosphonate and calcium treatment.
Figure 5: (a) Lateral X-ray film of lumbar spine showing compression fracture at D11 (white arrow) and (b) Sagittal image of magnetic resonance imaging showing compression fracture of D11 just after the injury. (c) Anteroposterior and lateral views of spine after the final surgery of proximal extension of the rod-screw construct to manage the complication of proximal junction kyphosis

Click here to view



  Discussion Top


Sacral chordoma is a rare condition and its management often requires multidisciplinary approach.[1] Here, we present a rare case of sacral chordoma which coexisted with disc herniation, spondylolisthesis, and lumbar canal stenosis (LCS). This may further guide various surgeons in the management of such a case where, besides sacral tumors, other conditions of the spine may be present simultaneously. Chordoma excision with distal extension of fusion was carried out only after 6 months of primary surgery when he started having bowel and bladder involvement. Considering his age, past history of heart surgery, diabetes, and current anticoagulation medications, it was a high-risk surgery which was discussed with the patient and he consented for it. Anterior laparoscopic adhesiolysis was performed to limit the blood loss, since the patient was on blood thinners. Posterior resection of the tumor was also done in the same stage.

Majority of the existing literature deals with cases of isolated sacral chordoma and their management.[8] Our patient had sacral chordoma along with high lumbar disc prolapse, degenerative spondylolisthesis, and proximal junctional kyphosis. Moreover, the patient was a case of coronary artery disease and had undergone coronary artery bypass graft. He was a known diabetic and on blood thinners. This further resulted in dilemma about what to operate first. The patient was managed according to the symptoms and requirements to limit the blood loss. Since he predominantly had symptoms of L1–L3 along with L2–L3 disc herniation and L1 fracture, it was decided to go for decompression and fixation (D12–L3) of spine without the tumor excision. The patient returned back to normal daily activity after the primary surgery. Though the biopsy of the tumor was taken preoperatively which had confirmed sacral chordoma, the patient was frequently followed up for any deterioration of symptoms.

A number of modalities have been described for the management of sacral tumors with little benefit. Radiotherapy has been found to be of some use in benign primary sacral tumors.[9],[10] In our case neither radiotherapy nor chemotherapy was given to the patient. However, it is suggested that radiotherapy should be restricted to in-operable cases because of the risk of malignant transformation.[1] A study by Nakajo et al. reported on percutaneous ethanol injection therapy combined with irradiation for successful management of sacral chordoma.[11] Chemotherapy has been shown to be of little importance in such cases as they are benign aggressive lesions.[1] However, a few studies have reported the use of chemotherapy in cases of metastatic lesions. Some case reports have even shown sacral chordoma response to chemotherapy.[12] Imatinib mesylate and cisplatin have been used in a few cases with favorable outcomes.[13],[14] A recent study by Shinji et al. reported treatment of sacral chordoma with a drug called pazopanib.[15] However, the patient survived for only 14 months with the medical management. Embolization of the sacral tumor with various agents such as Gelfoam® has also been shown to be successful.[16] Surgical resection has been considered as the mainstay of the treatment; however, recurrence after en bloc resection has also been reported.[17] We preferred surgical resection in our case.

Although surgical resection has been widely reported for the management of sacral chordoma, patients suffer from serious complications such as bowel, bladder, and sexual dysfunctions; excessive blood loss; and infections.[1] Various surgical techniques have been described to remove sacral chordoma.[18] Anterior, posterior, and combined approaches have been used successfully, with majority of the surgeons preferring posterior approach. The role of anterior approach is to ligate the main tumor vessel and/or expose the anterior aspect of the tumor by isolating the proper plane of resection. Isolated anterior approach has been used for small sacral tumors. Ours is a unique case report where anterior laparoscopic technique was used to resect and mobilize the tissue along with posterior sacrectomy and chordoma removal. Osteotomes have been traditionally used in the process to remove sacral tumors.[19] They have been found to damage soft-tissue structures and hence considered a little unsafe.[20] Bone scalpel has been used successfully in cervical laminectomy.[21] Similarly, bone scalpel was used in this case for sacral tumor excision. S2 nerve root's evoked potentials were closely monitored and the patient showed no bowel and bladder involvement after surgery. The use of intraoperative neuromonitoring further prevented the events which could result in alteration of neurology in the patient.

The complex management of sacral tumor is based on the complete removal of the tumor tissue in conjunction with the rehabilitation process and long-term follow-up. In our case, complete resection of tumor and simultaneous attention to stabilize spondylolisthesis gave patient better mobility postoperatively. Anterior laparoscopic resection and mobilization of the tissue along with posterior sacrectomy with bone scalpel and chordoma excision resulted in less postoperative morbidity to the patient.

Proximal junction kyphosis (PJK) is a known complication of long-level spine fixation.[22] It was also noticed in this case 4 months after the second surgery. The patient had a history of fall while walking in the house which resulted in a proximal-level fracture with kyphosis. Usually, PJK needs revision surgery in the form of extension of fusion which was similar in this case also. We had tried to give him conservative treatment trial in the form of analgesics, calcitonin nasal spray along with on-going anti-osteoporotic medicines and bed rest. However, it did not work and the rod-screw construct was then extended proximally and stabilization of the spine was done. The patient was given bisphosphonates and calcium supplements in the postoperative period. There was discussion on whether to put him on teriparatide injections. However, after taking opinion from the hematologist and oncologist, with a view of published literature,[6],[7] it was decided not to give teriparatide. The patient suffered with PJK after 1 year of sacral chordoma removal. Walking was resumed by him after 1 month of physiotherapy following the proximal extension of the rod-screw construct. At the end of 2 years of the primary procedure, the patient is pain free now and walks independently.


  Conclusion Top


Management of sacral chordoma requires a multidisciplinary approach. En bloc surgical resection of the tumor is the mainstay of the treatment and can be attempted by anterior, posterior, or combined means. We reported a unique case of sacral chordoma with coexisting disc herniation, LCS, and spondylolisthesis, which was operated by discectomy and fusion first as it was the only symptomatic condition, followed by combined anterior laparoscopic isolation of tumor and posterior sacral chordoma excision using bone scalpel under neurological monitoring along with fixation of spondylolisthesis. Fusion was extended later because of the development of PJK. This case report highlights the treatment approach while facing multiple issues in the same patient.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understand that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

This study has been partially funded by AO Spine Asia Pacific Grant (Modi HN, Goel SA, Desai YJ, 2017).

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Varga PP, Bors I, Lazary A. Sacral tumors and management. Orthop Clin North Am 2009;40:105-23, vii.  Back to cited text no. 1
    
2.
Feldenzer JA, McGauley JL, McGillicuddy JE. Sacral and presacral tumors: Problems in diagnosis and management. Neurosurgery 1989;25:884-91.  Back to cited text no. 2
    
3.
Disler DG, Miklic D. Imaging findings in tumors of the sacrum. AJR Am J Roentgenol 1999;173:1699-706.  Back to cited text no. 3
    
4.
Bergh P, Gunterberg B, Meis-Kindblom JM, Kindblom LG. Prognostic factors and outcome of pelvic, sacral, and spinal chondrosarcomas: A center-based study of 69 cases. Cancer 2001;91:1201-12.  Back to cited text no. 4
    
5.
York JE, Berk RH, Fuller GN, Rao JS, Abi-Said D, Wildrick DM, et al. Chondrosarcoma of the spine: 1954 to 1997. J Neurosurg 1999;90:73-8.  Back to cited text no. 5
    
6.
Subbiah V, Madsen VS, Raymond AK, Benjamin RS, Ludwig JA. Of mice and men: Divergent risks of teriparatide-induced osteosarcoma. Osteoporos Int 2010;21:1041-5.  Back to cited text no. 6
    
7.
Watanabe A, Yoneyama S, Nakajima M, Sato N, Takao-Kawabata R, Isogai Y, et al. Osteosarcoma in Sprague-Dawley rats after long-term treatment with teriparatide (human parathyroid hormone (1-34)). J Toxicol Sci 2012;37:617-29.  Back to cited text no. 7
    
8.
Ailon T, Torabi R, Fisher CG, Rhines LD, Clarke MJ, Bettegowda C, et al. Management of locally recurrent chordoma of the mobile spine and sacrum: A Systematic review. Spine (Phila Pa 1976) 2016;41 Suppl 20:S193-8.  Back to cited text no. 8
    
9.
Raque GH Jr., Vitaz TW, Shields CB. Treatment of neoplastic diseases of the sacrum. J Surg Oncol 2001;76:301-7.  Back to cited text no. 9
    
10.
York JE, Kaczaraj A, Abi-Said D, Fuller GN, Skibber JM, Janjan NA, et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery 1999;44:74-9.  Back to cited text no. 10
    
11.
Nakajo M, Ohkubo K, Fukukura Y, Nandate T, Nakajo M. Treatment of recurrent chordomas by percutaneous ethanol injection therapy and radiation therapy. Acta Radiol 2006;47:297-300.  Back to cited text no. 11
    
12.
Dominkus M, Ruggieri P, Bertoni F, Briccoli A, Picci P, Rocca M, et al. Histologically verified lung metastases in benign giant cell tumours–14 cases from a single institution. Int Orthop 2006;30:499-504.  Back to cited text no. 12
    
13.
Casali PG, Messina A, Stacchiotti S, Tamborini E, Crippa F, Gronchi A, et al. Imatinib mesylate in chordoma. Cancer 2004;101:2086-97.  Back to cited text no. 13
    
14.
Hindi N, Casali PG, Morosi C, Messina A, Palassini E, Pilotti S, et al. Imatinib in advanced chordoma: A retrospective case series analysis. Eur J Cancer 2015;51:2609-14.  Back to cited text no. 14
    
15.
Shinji A, Okada S, Nakamura T. A case of chordoma of the sacrum treated with pazopanib. Gan To Kagaku Ryoho 2016;43:267-9.  Back to cited text no. 15
    
16.
Hosalkar HS, Jones KJ, King JJ, Lackman RD. Serial arterial embolization for large sacral giant-cell tumors: Mid- to long-term results. Spine (Phila Pa 1976) 2007;32:1107-15.  Back to cited text no. 16
    
17.
Dubory A, Missenard G, Court C. Late local recurrence, at 19 and 17 years, of sacral chordoma treated by en bloc resection. Orthop Traumatol Surg Res 2016;102:121-5.  Back to cited text no. 17
    
18.
Zhang HY, Thongtrangan I, Balabhadra RS, Murovic JA, Kim DH. Surgical techniques for total sacrectomy and spinopelvic reconstruction. Neurosurg Focus 2003;15:E5.  Back to cited text no. 18
    
19.
Sahakitrungruang C, Chantra K. One-staged subtotal sacrectomy for primary sacral tumor. Ann Surg Oncol 2009;16:2594.  Back to cited text no. 19
    
20.
Gerbault O, Daniel RK, Kosins AM. The role of piezoelectric instrumentation in rhinoplasty surgery. Aesthet Surg J 2016;36:21-34.  Back to cited text no. 20
    
21.
Onen MR, Yuvruk E, Akay S, Naderi S. The reliability of the ultrasonic bone scalpel in cervical spondylotic myelopathy: A comparative study of 46 patients. World Neurosurg 2015;84:1962-7.  Back to cited text no. 21
    
22.
Scheer JK, Osorio JA, Smith JS, Schwab F, Lafage V, Hart RA, et al. Development of validated computer-based preoperative predictive model for proximal junction failure (PJF) or clinically significant PJK with 86% accuracy based on 510 ASD patients with 2-year follow-up. Spine (Phila Pa 1976) 2016;41:E1328-35.  Back to cited text no. 22
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed467    
    Printed61    
    Emailed0    
    PDF Downloaded71    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]