Print version ISSN 0004-282X
Arq. Neuro-Psiquiatr. vol.66 no.2b São Paulo June 2008
Sacral chordoma en-bloc resection and lumbar-iliac stabilization
Ressecção en bloc de cordoma sacral e estabilização lomboilíaca
Ruy Yoshiaki OkajiI; José Marcondes da Silveira JrII; Milton Kanenori NakanoIII; Leandro Tomokazu KiutiIV; Maria Cecília Nieves Teixeira MaioranoIV
IAssistant Professor of Neurosurgery, FAMEMA - Neurosurgery Service - Clinical Hospital of the Marilia Medical School - Marilia SP, Brazil (FAMEMA)
IIOrthopaedic Surgeon, FAMEMA - Neurosurgery Service - Clinical Hospital of the Marilia Medical School - Marilia SP, Brazil (FAMEMA)
IIIOncologist Surgeon, FAMEMA - Neurosurgery Service - Clinical Hospital of the Marilia Medical School - Marilia SP, Brazil (FAMEMA)
IVMedical Student, FAMEMA - Neurosurgery Service - Clinical Hospital of the Marilia Medical School - Marilia SP, Brazil (FAMEMA)
Chordoma is a rare malignant bone tumor originated from embryonic remains of notochord. Restricted to the axial skeleton, it is more frequently found in the sacrococcygeal (60%) and cervical spine (30%) areas. It occurs with a frequency twice larger in man than in women and it is uncommon in people with age under 40 years, occurring predominantly between the 5th and 7th decades of life1,3. The diagnosis is performed through the clinical features, physical examination, including rectal touch and image, being the computed tomography (CT) and the magnetic resonance imaging (MRI), the main methods to delimitate the local extension of the tumor2. Because of its unfavorable evolution, that is, slow, with unspecific symptoms and locally aggressive, its precocious diagnosis is difficult to be performed. According to the literature, the most frequently reported symptomatology is pain3,4,11. Most of the sacral chordomas are initially presented with a considerable extra axial tumoral growth3,4. Metastases are not frequent and most of the pathologists consider chordomas as on a low malignant degree. The differential diagnosis involves giant cell tumors, chondrosarcoma, lymphoma and metastatic adenocarcinoma. The surgical treatment is the only healing form, but, due to the possibility of impairment on neural and adjacent structures by the tumor, its total resection results in important functional deficits5.
We report the case of a female patient with swelling and pain on the sacral area and partial loss of strength of inferior limbs with evolution in one year.
A 72 years old, white, woman referring pain in left inferior limb with an evolution of one year, burn-like, continuous, progressive, with slight relief when using painkillers and nonsteroidal antiinflammatory drugs (NSAIDs). She refers progressive loss of strength in this same limb, evolving, in the last 2 months, to urinary and fecal retention, and tingling in the genital area. On clinical examination, a swelling in the sacral area was observed, painless on palpation and without inflammatory signs, with hardened consistency. Pain in left inferior limb following the same way as S1. Decrease of the motor activity (plantar flexion grade 2) with abolished Achilles reflex. Hypoesthesia in sellar area. Pelvic MRI has shown an expansive process below the S1 level (Fig 1). Needle biopsy was compatible with chondrosarcoma.
She was submitted to the en-block resection of the lesion, with previous and posterior approach at the same surgical moment. Patient in dorsal decubitus with support under the lumbar-sacral area, under general anesthesia; performed transperitoneal access to the lumbar/sacral transition area, being visualized the lesion (Fig 2). We performed clotting of the nourishing vases of the lesion (branches of the iliac arteries), as well as venous ligation. Next we performed sacral osteotomy between S1 and S2.
Posterior via: patient in ventral decubitus, elliptical incision around the sacral swelling extending to the L4 spinous process, exhibiting lamina and transversal processes from L4 to S1; dissection of the tumoral mass, with resection of a large part of the gluteus musculature bilaterally; laminectomy L5 and S1 up to identification of S1 roots, being accomplished ligation of the dural sac below that level and amputation. Concluded S1-S2 sacral osteotomy followed by sacral amputation with in-block retreat of the lesion with exiguous surgical margins (Fig 2). Performed fixation with pedicular and iliac screws on L4, L5 and iliac bone, with good stability (Fig 3) and precocious ambulation (15 days). Posterior anatomopathological revealed histology compatible with chordoma.
The patient, now with 1 year and 8 months of folow-up, ambulates with help of a walker, has plegia of the left S1 root, urinary incontinence and fecal retention.
We received the approval from Ethics Committees of Marilia Medical School and the informed consent from the patient permitting this publication.
The image methods and histopathology have important roles on the diagnosis and on determining the treatment of those tumors. In spite of the radical resection is the best treatment for malignant sacral tumors, the total sacrectomy for such tumors has been used in few situations, mainly in those resistant to non-interventionist therapies4-7, it has been reported a larger recurrence period for patients treated with total resection than for those with subtotal resection8.
The extension of the sacral resection determines the pelvic stability and the need or not of having a reconstruction. However, the total sacrectomy with or without retreat of ileum adjacent parts results in complete dissociation of the spine and pelvis causing both vertical and rotational instability. Thus, there is need of a lumbar-iliac reconstruction for reestablish the pelvic stability7,9.
The functional consequences for the patient should be clearly discussed in the preoperative evaluation7.
Because of the high risk of recurrence of this disease, all of the patients should be seriously followed-up with MRI or CT8.
We emphasize the need of a precocious diagnosis, resection with appropriated margins and improvement of surgical techniques, image studies and adjuvant therapies so that we can obtain a better prognostic for those patients. Multiprofessional approach for the patient is fundamental for the physical rehabilitation and psychological support, for improving the patient's quality of life and postoperative clinical follow-up10-12.
1. Anson KM, Byrne PO, Robertson ID, Gullan RW, Montgomery AC. Radical excision of sacrococcygeal tumours. Br J Surg 1994;81:460-446. [ Links ]
2. Yonemoto T, Tatezaki S, Takenouchi T, Ishii T, Satoh T, Moriya H. The surgical management of sacrococcygeal chordoma. Cancer 1999;85:878-883. [ Links ]
3. Fourney DR, Gokaslan ZL. Current management of sacral chordoma. Neurosurg Focus 2003;15:1-8. [ Links ]
4. Zileli M, Hoscoskun C, Brastianos P, Sabah D. Surgical treatment of primary sacral tumors: complications associated with sacrectomy. Neurosurg Focus 2003;15:1-8. [ Links ]
5. Tomita K, Tsuchiya H. Total sacrectomy and reconstruction for huge sacral tumors. Spine 1990;15:1223-1227. [ Links ]
6. Wuisman P, Lieshout O, Sugihara S, van Dijk M. Total sacrectomy and reconstruction: oncologic and functional outcome. Clin Orthop Relat Res 2000;381:192-203. [ Links ]
7. Gallia GL, Haque R, Garonzik I, et al. Spinal pelvic reconstruction after total sacrectomy for en bloc resection of a giant sacral chordoma: technical note. J Neurosurg Spine 2005;3:501-506. [ Links ]
8. York JE, Kaczaraj A, Abi-Said D, et al. Sacral chordoma: 40-year experience at a major cancer center. Neurosurgery 1999;44:74-79. [ Links ]
9. Zhang HY, Thongtrangan I, Balabhadra RS, Murovic JA, Kim DH. Surgical techniques for total sacrectomy and spinopelvic reconstruction. Neurosurg Focus 2003;15:1-10. [ Links ]
10. Doita M, Harada T, Iguchi T, et al. Total sacrectomy and reconstruction for sacral tumors. Spine 2003;28:29e6-301. [ Links ]
11. Bethke KP, Neifeld JP, Lawrence W Jr. Diagnosis and management of sacrococcygeal chordoma. J Surg Oncol 1991;48:232-238. [ Links ]
12. Cheng EY, Ozerdemoglu RA, Transfeldt EE, Thompson RC Jr. Lumbosacral chordoma: prognostic factors and treatment. Spine 1999;24:1639-1645. [ Links ]
Received 1 August 2007, received in final form 29 February 2008. Accepted 17 March 2008.
Dr. Ruy Yoshiaki okaji - Avenida Monte carmelo 800 - 17519-030 Marília SP - brasil. e-mail: email@example.com