High Expression of TGF-ß1 Predicting Tumor Progression in Skull Base Chordomas.

OBJECTIVE: To investigate the expression characteristics and prognostic value of transforming growth factor ß1 (TGF-ß1) in primary skull base chordomas (SBCs). METHODS: The mRNA expression levels of TGF-ß1 were measured in 57 frozen samples from patients with primary SBCs. Clinical data collection, follow-up, correlations, and survival analyses were performed. RESULTS: In the series of 57 patients (29 men and 28 women) with primary SBCs, the mean value of TGF-ß1 mRNA was 1.713 with a median of 0.904. Twenty-four SBCs were soft type and 33 were hard type. The Mann-Whitney U test revealed that the expression level of TGF-ß1 mRNA in hard type SBCs was significantly higher than the expression level found in the soft type (P = 0.03). The independent-samples median test suggested that the expression level of TGF-ß1 mRNA in female patients' SBCs was significantly higher than that in male patients' SBCs (P = 0.01). Expression differences of TGF-ß1 were not seen among different pathological subtypes, tumor blood supply, or degree of resection. The Spearman rank correlation coefficient clarified that TGF-ß1 mRNA levels were not correlated with tumor diameter, preoperative Karnofsky Performance Status (KPS), postoperative KPS, follow-up KPS, age, or intraoperative blood loss. The multivariate Cox analysis revealed that pathological subtype (P = 0.008), expression level of TGF-ß1 mRNA (P = 0.01), and tumor texture (P = 0.03) were all independent prognostic factors for tumor progression. CONCLUSIONS: SBCs in female patients and SBCs with hard texture were prone to have high TGF-ß1 mRNA expression. High expression of TGF-ß1, hard tumor texture, and conventional subtype were all independent risk factors for tumor progression.

MR Imaging Grading System for Skull Base Chordoma.

BACKGROUND AND PURPOSE: Skull base chordoma has been widely studied in recent years, however, imaging characteristics of this tumor have not been well elaborated. The purpose of this study was to establish an MR imaging grading system for skull base chordoma. MATERIALS AND METHODS: In this study, 156 patients with skull base chordomas were retrospectively assessed. Tumor-to-pons signal intensity ratios were calculated from pretreatment MR images RT1 (ratio of tumor to pons signal intensity in T1 FLAIR sequence), RT2 (ratio of tumor to pons signal intensity in T2 sequence) and REN (ratio of tumor to pons signal intensity in enhanced T1 FLAIR sequence), and significant ratios for overall survival and progression-free survival were selected to establish a grading system. Clinical variables among different MR imaging grades were then analyzed to evaluate the usefulness of the grading system. RESULTS: RT2 (P < .001) and REN (P = .04) were identified as significant variables affecting progression-free survival. After analysis, the classification criteria were set as follows: MR grade I, RT2 > 2.49 and REN ≤ 0.77; MR grade II, RT2 > 2.49 and REN > 0.77, or RT2 ≤ 2.49 and REN ≤ 0.77; and MR grade III, RT2 ≤ 2.49 and REN > 0.77. MR grade III tumors had a more abundant tumor blood supply than MR grade I tumors (P < .001), and the intraoperative blood loss of MR grade III tumors was higher than that of MR grade I tumors (P = .002). Additionally, skull base chordoma progression risk increased by 2.071 times for every single MR grade increase (P < .001). CONCLUSIONS: A higher RT2 value was a negative indicator of tumor progression, whereas a higher REN value was a positive risk factor of tumor progression. MR grade III tumors showed a more abundant blood supply than MR grade I tumors, and the risk of skull base chordoma progression increased with every single MR grade increase.

Transarterial Onyx embolization of sacral chordoma: case report and review of the literature.

AIM: To report a successful transarterial Onyx embolization of a highly vascularized sacral chordoma. CASE PRESENTATION: A 74-year-old patient presented with progressive lower back pain and anorectal and urogenital dysfunction complicated by excessive intraoperative blood loss requiring emergent endovascular intervention. INTERVENTION: Emergent pelvic angiography demonstrated a highly vascularized sacral chordoma. Transarterial embolization with Onyx was performed successfully via a single arterial pedicle, the median sacral artery. Surgical extirpation was achieved with the expected amount of intraoperative blood loss without further complications. CONCLUSION: Preoperative transarterial Onyx embolization can be performed safely and effectively to assist in the surgical resection of sacral chordomas.

Pulsed laser-induced liquid jet for skull base tumor removal with vascular preservation through the transsphenoidal approach: a clinical investigation.

BACKGROUND: The transsphenoidal approach has recently been used to treat complex lesions beyond the sella turcica, but the difficulties of dealing with small vessels, deep and narrow space, and working angle may limit the procedures. To overcome these problems, we have developed a pulsed laser-induced liquid jet (LILJ) system to dissect tumor tissue with preservation of fine blood vessels within deep and narrow working spaces and evaluated its utility and safety. METHODS: The LILJ system was applied to 14 consecutive patients with uncharacteristically complex skull base tumor treated through the extended transsphenoidal approach. This system consists of a bayonet-shaped catheter incorporating a jet generator formed of stainless tube (external diameter 1.10 mm, internal diameter 0.78 mm), which was surrounded by a coaxial polytetrafluoroethylene 14-G equivalent suction tube to be able to incorporate into the confined working spaces. Minor modifications could be fitted for the catheter (15 to 18 cm length, straight or side flexion tip), and total weight was around 7 g. FINDINGS: Precise dissection and mass reduction of the tumor were obtained in all cases except one recurrent case of chordoma with significant fibrosis due to radiation. Both small arteries and veins were preserved, allowing subsequent microsurgical devascularization. Intraoperative blood loss was minimal, and tumor removal rate was satisfactory after the introduction of the system. No complication was related to use of the LILJ system. CONCLUSION: Although comparison between conventional surgical instruments is mandatory in the future, the present study suggests that the LILJ system can achieve safe and optimum removal of complex skull base tumor. Potential application for minimally invasive endoscopic system, as well as potentials for changing the design of the catheter in according to preference of surgeon with low cost, may give advantages over conventional surgical instruments.

Expression of vascular endothelial growth factor and matrix metalloproteinase-9 in sacral chordoma.

Sacral chordoma is a vessel-rich and infiltrative tumor, but the fundamental knowledge of its biological behavior remains unknown. This study was designed to investigate the expression levels and contributions of vascular endothelial growth factor (VEGF) and matrix metalloproteinase-9 (MMP-9) in the angiogenesis and recurrence of sacral chordoma and their correlations. An immunohistochemical method was used to investigate the expression of VEGF, MMP-9, and microvascular density (MVD) in 36 patients with sacral chordoma. Their differences in expressions were statistically analyzed and their correlations with angiogenesis and recurrence were evaluated. The mean MVD of sacral chordomas was significantly higher than that of the adjacent normal tissues (P = 0.033). Immunoreactivity for VEGF and MMP-9 was significantly higher in sacral chordoma tissues than in adjacent normal tissues (P = 0.008, P = 0.005). The mean MVD of VEGF and MMP-9 were statistically higher in positive group than in negative group (P = 0.015, P = 0.004), respectively . Moreover, a significant correlation was found between the VEGF and MMP-9 (P = 0.002). The log-rank test revealed that continuous disease-free survival time (CDFS) was significantly shorter in the MMP-9-positive group than in the MMP-9-negative group (P = 0.019), but the difference in the VEGF-positive group and the VEGF-negative group was not statistically significant (P = 0.938). Our data suggest that VEGF and MMP-9 might act with a synergistic effect and can positively regulate the angiogenesis in sacral chordoma. Positive expression of MMP-9 might indicate the local recurrence of sacral chordoma. The result suggests that some specific drugs which inhibit VEGF, MMP-9, or their receptors may have a good therapeutic effect for sacral chordoma.

Surgical treatment of sacral chordomas combined with transcatheter arterial embolization.

STUDY DESIGN: A retrospective study, analyzing midterm results of transcatheter arterial embolization (TAE) for removal of a sacral chordoma. OBJECTIVE: To develop a suitable method to maximally remove the tumor. SUMMARY OF BACKGROUND DATA: Although previous reports have reported the use of TAE for removal of tumors, there is currently no data available on using this method to treat sacral chordomas. METHODS: Thirty patients with sacral chordomas, who underwent surgical treatment from January 1994 to September 2005, were selected. All 30 patients underwent the posterior approach after TAE of the main arteries that supplied the sacral chordoma. The average follow-up was 44.6 months after the operation. The blood lost during the operation, blood transfusion, and the blood drained from the patient after the operation was reviewed. In addition, the sphincter muscle function of the bladder and bowel was observed. RESULTS: The intraoperative blood loss averaged 1200 mL during the removal of the tumor. The average blood drained from the patient after the operation was 650 mL. The average blood transfusion postoperatively was 1080 mL. Of the 30 patients, 12 had recurrence and 2 died of tumor metastasis. In the 17 patients whose sacral nerve roots had been bilaterally reserved at and above the S3 level, the sphincter muscle function of the bladder and bowel was good, whereas the function of the sphincter muscles was impaired in 2 patients with nerve roots reserved only at and above the S1 level. In 1 case, colostomy and ureterocutaneosomy were used. CONCLUSIONS: In comparison with historical literature, preoperative TAE for excising the sacral tumor can significantly decrease intraoperative blood loss, make the surgical field clear, possibly eliminate the need for using an anterior approach, and facilitate the maximal removal of the sacral chordoma. It is an encouraging technique for excising the sacral chordomas.

Occlusion of the abdominal aorta by balloon dilation catheter assisting surgical excision of a sacrum chordoma: case report.

Chordoma is known to be the most common primary tumor of the sacrum. Its surgery is challenging from many aspects. A large amount of bleeding is one of the biggest issues. A 52-year-old woman was admitted to our clinic with a huge mass at sacrum. The mass was diagnosed as chordoma after a needle biopsy. Prior to the surgery, a balloon dilation catheter (BDC) was placed in the distal abdominal aorta via the femoral artery. Just after the skin incision, the BDC was inflated with contrast medium and total occlusion of the aorta was achieved. At the end of the operation we observed that total hemorrhage had decreased dramatically. No complications were recorded except hypertension during the occlusion of the aorta which was expected. The patient was discharged after two weeks of hospitalization post-operatively without any neurological deficit. Our report is the second in the neurosurgical literature to our knowledge. Although this result is preliminary and needs to be replicated and expanded upon, our first experience with this method is effective for decreasing the amount of bleeding. We believe that our method prevents hemo-dynamical problems caused by massive bleeding and complications secondary to massive transfusion.

Clival chordoma manifesting as nasal bleeding. A case report.

Chordoma is a rare cartilaginous tumor, for which bleeding presentation is unusual. We report a case of rare hemorrhaged clival chordoma, which was diagnosed correctly by magnetic resonance imaging. A 32-year-old man presented with nasal bleeding. The tumor was totally removed via a trans-sphenoidal approach, from which the surgical specimen confirmed chordoma. Epistaxis seemed to be caused by the spreading of the intratumoral hemorrhage into the sphenoid sinus. This case demonstrates the importance of an exact differential diagnostic evaluation, including chordoma, by use of modern imaging techniques for nasal bleeding.

[Hypervascular chordoma: report of a case].

The authors report a rare case of sellar chordoma with a marked vascularity documented on cerebral angiogram. Its possible pathogenesis and some therapeutic problems were briefly discussed. Only a few cases of chordoma with such a positive tumor stain have been reported previously.

MR of vascular encasement in parasellar masses: comparison with angiography and CT.

The relationship between tumor mass and vascular involvement as seen on MR imaging was examined in 11 patients with masses in the parasellar region, and the findings were correlated with CT and angiography. In six cases, MR was superior to CT and angiography in depicting the relationship of the tumor to adjacent blood vessels. In these cases, MR demonstrated tumor surrounding the blood vessel without changing the diameter of its lumen. Angiography did not reveal encasement in these cases. In four cases, both MR and angiography showed signs of vascular encasement with narrowing of the vessel's lumen. In two cases, MR was equivocal while angiography revealed vascular encasement in one case and was negative for encasement in the other. CT was less sensitive than MR in defining vascular encasement since there is usually little contrast between an enhancing tumor and the major blood vessels. Coronal scanning appeared to be the best plane of imaging and correlated well with the anteroposterior angiogram. We propose that MR is the method of choice for evaluating arterial encasement by tumors and may obviate the need for angiography in those cases in which MR is positive for a basal lesion.

Spinal chordomas.

A series of 54 patients with spinal chordomas were treated at Memorial Sloan-Kettering Cancer Center between 1949 and 1976. Thirty-six lesions were located in the sacrococcygeal region and 18 involved the vertebral column at a higher level. The male to female ratio was 35:19. Vertebral chordomas generally occurred in a younger age group. Our radiological findings suggest that there is marked soft-tissue extension anterior to the vertebral column at the time of initial diagnosis. Eleven of 18 vertebral chordomas and 10 of 36 sacral chordomas were found to have disseminated metastases during their course. Analysis of the various modes of therapy reveals that the median survival for both groups is approximately 6 years. However, the 5-year survival for the sacrococcygeal group was 66% as opposed to 50% for the vertebral group. Radiation therapy produced significant palliation but objective evidence of tumor regression was difficult to assess. Chemotherapy in a small number of patients did not have any effect on the tumor. With the advent of computerized tomography scanning, further studies should be done to document the response of this tumor to radiation therapy.