Chordoma of the skull base: predictors of tumor recurrence

Pallini, Roberto; Maira, Giulio; Pierconti, Francesco; Falchetti, Maria Laura; Alvino, Ester; Cimino‐Reale, Graziella; Fernández, Eduardo B.; D’Ambrosio, Ettoré; Larocca, Luigi Maria

doi:10.3171/jns.2003.98.4.0812

Cited by 98 publications

(61 citation statements)

References 50 publications

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“…2,9,14,15) In recent studies, the extent of removal was microscopically total in 43.5% and 42.3%, subtotal in 47.8% and 27.0%, and partial in 8.7% and 30.0%. 2,27) In our study, total or near total removal was achieved in four patients (36%), subtotal resection in five (45%), and partial removal in two (18%) despite the less aggressive strategy. Recurrence was observed in five patients at 4 to 48 months (mean 24 months).…”

Section: Discussionmentioning

confidence: 99%

Skull Base Chordomas: Management and Results

Stüer

Schramm

Schaller

2006

Neurol. Med. Chir.(Tokyo)

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Growth patterns of skull base chordomas are related to important neurovascular structures. Local invasiveness results in``clinically malignant'' behavior. A high rate of transient neurological deficits occurs following radical surgery. At our institution, the principle of radical removal is not followed at any price. This study compared the results of our management with recent series. Eleven patients, five females and six males aged 24-65 years (mean 41 years), underwent removal of skull base chordoma with size one º3 cm, five 3-5 cm, and five À5 cm. Mostly, standard operative approaches were chosen. All patients underwent postoperative radiotherapy. Resection was subtotal/partial in seven patients and total in four with no mortality. Neurological deterioration occurred due to transient cranial nerve deficits in six patients. Temporary surgical morbidity (including cranial nerve deficits) was observed in seven patients. Median Karnofsky performance status score improved compared to preoperative (80), early postoperative (70), and latest assessment (90) (median 36 months). Five patients underwent reoperation due to tumor recurrence after 4-48 months (mean 24 months). Most patients undergoing removal of skull base chordomas suffer from transient neurological deficits which are mainly nonsignificant as the patients return to preoperative functional status. The apparently high rate of incomplete tumor resection (64%) reflects the infiltrative behavior and relationship with neurovascular structures. The operative strategy should not be excessively aggressive at any price, but rather take into account the options of radiotherapy and observation of residual tumor.

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Section: Discussionmentioning

confidence: 99%

Skull Base Chordomas: Management and Results

Stüer

Schramm

Schaller

2006

Neurol. Med. Chir.(Tokyo)

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“…5 Aggressive resection followed by adjuvant LINAC or protonbeam radiotherapy provides the best results, as exemplified in our series. 40,50 Fibrous dysplasia in a spinal location with monostotic disease is extremely rare. 14,22,27,45 Until 2006, only 25 cases had been reported among patients of all ages.…”

Section: Primary Bone Tumors Affecting the Atlantoaxial Skeleton Of Cmentioning

confidence: 99%

Primary atlantoaxial bone tumors in children: management strategies and long-term follow-up

Menezes¹,

Ahmed²

2014

PED

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Object Atlantoaxial tumors account for a substantial proportion of primary bone tumors in children. Before resection, surgeons must consider the complex regional anatomy, the potential for neurological compromise, craniocervical instability, and the question of tumor resectability in a growing spine. Using current technology, the authors analyzed surgical cases in this light and present outcomes and treatment recommendations after long-term patient follow-up. Methods: The authors reviewed clinical records for 23 children whose primary atlantoaxial bone tumors were treated from 1996 through 2010. Results Pathological lesions among the 23 patients were 4 aneurysmal bone cysts, 2 osteochondromas, 5 chordomas, 4 osteoblastomas, 3 fibrous dysplasias, 4 eosinophilic granulomas, and 1 Ewing's sarcoma. Clinical presentation consisted of neck pain (n = 23), headaches and occipital pain (n = 16), myelopathy (n = 8), and torticollis (n = 4). Selective angiography and coil embolization were undertaken for all patients with aneurysmal bone cysts and osteoblastomas, 2 patients with chordomas, 1 patient with fibrous dysplasia, and 1 patient with Ewing's sarcoma. Primary embolization treatment of radiation-induced aneurysmal bone cyst of the atlas showed complete reossification. Results of CT-guided needle biopsy were diagnostic for 1 patient with eosinophilic granuloma and 1 with Ewing's sarcoma. Needle biopsies performed before referral were associated with extreme blood loss for 1 patient and misdiagnosis for 2 patients. Surgery involved lateral extrapharyngeal, transoral, posterior, and posterolateral approaches with vertebral artery rerouting. Complete resection was possible for 9 patients (2 with osteochondroma, 3 with fibrous dysplasia, 2 with chordoma, and 2 with osteoblastoma). Decompression and internal fusion were performed for 3 patients with aneurysmal bone cysts. Of the 23 patients, 7 underwent dorsal fusion and 4 underwent ventral fusion of the axis body. Chemotherapy was necessary for the patients with eosinophilic granuloma with multifocal disease and for the patient with Ewing's sarcoma. There was no morbidity, and there were no deaths. All patients with benign lesions were free of disease at the time of the follow-up visit (mean ± SD follow-up 8.8 ± 1.1 years; range 2–18 years). Chordomas received proton or LINAC irradiation, and as of 4–15 years of follow-up, no recurrence has been noted. Conclusions Because most atlantoaxial tumors in children are benign, an intralesional procedure could suffice. Vascular control and staged resection are critical. Ventral transoral fusion or lateral extrapharyngeal fusion has been successful. Resection with ventral fusion and reconstruction are essential for vertebral body collapse. Management of eosinophilic granulomas must be individualized and might require diagnosis through needle biopsy.

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“…[4,20] These tumors are some of the most common tumors of axial skeleton, although in the skull they are still rare, comprise approximately 0.1 to 0.2% of all primary intracranial neoplasms. [3,20] No typical symptoms are associated with skull base chordomas because they depend on tumor location and size. [3] The molecular basis for the clinical behavior of these neoplasm is unknown.…”

Section: Introductionmentioning

confidence: 99%

Reversion-inducing cysteine-rich protein with kazal motifs and matrix metalloproteinase-9 are prognostic markers in skull base chordomas

et al. 2009

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Prognosis of chordomas is difficult to predict based solely on histological findings. The purpose of this study was to assess expressions of reversion-inducing cysteine-rich protein with kazal motifs (RECK) and matrix metalloproteinase (MMP)-2, MMP-9 in skull base chordomas and to find out their correlations to outcome.Immunohistochemical study was performed in 19 samples (initial, n=11; recurrent, n=8) from 11 patients. The correlations among expression of RECK, MMP-2, MMP-9 and their prognostic values were analyzed. Significant correlation between RECK and MMP-9 was found, but there was no correlation found between MMP-2 and MMP-9.Higher MMP-9 expression significantly influenced outcome. Furthermore, MMP-9/RECK ratio showed significant correlation to outcome, showing their inverse relationship in the disease progress of skull base chordoma. RECK and MMP-9 can be valuable markers to predict prognosis in skull base chordomas.

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Chordoma of the skull base: predictors of tumor recurrence

Abstract: Reactivation of telomerase in chordomas is a reliable predictor of outcome. The ability to predict the biological behavior of chordomas might have immediate implications in the management of this disease in patients who undergo surgery.

Cited by 98 publications

References 50 publications

Skull Base Chordomas: Management and Results

Skull Base Chordomas: Management and Results

Primary atlantoaxial bone tumors in children: management strategies and long-term follow-up

Reversion-inducing cysteine-rich protein with kazal motifs and matrix metalloproteinase-9 are prognostic markers in skull base chordomas

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