The preoperative levels of fibrinogen and albumin were assessed in 183 primary skull base chordoma patients. The cutoff values were determined by X-tile software, and their correlations with patient prognosis were further explored using the Kaplan-Meier curve and Cox proportional hazards regression analysis. In addition, the predictive performances of these markers in survival were evaluated by receiver operating characteristic curves.
The values of fibrinogen and albumin in skull base chordoma patients ranged from 1.73 to 7.40 and 37.6 to 53.0 g/L, respectively. The optimal cutoff values for fibrinogen and albumin were 3.29 and 44.60 g/L, respectively. Fibrinogen and albumin were correlated with the patient age and tumor pathology types. Albumin, but not fibrinogen, was associated with the patients' progression-free survival and overall survival. Importantly, the FA score, which combines fibrinogen and albumin, could independently predict both progression-free survival and overall survival and enhanced the performance of fibrinogen or albumin in survival prediction in skull base chordoma.
This data reveals the clinical prognostic role of albumin and suggest that the FA score may be a valuable prognostic grading system in skull base chordoma 1).
All consecutive cases of skull base dedifferentiated chordomas treated surgically in a referral Center from January 1990 to June 2019 were retrospectively evaluated to assess peculiar pathological, radiological and clinical features. Patient data were retrieved from paper and electronic records.
RESULTS: Six cases (4 F; mean age at surgery 46 years, range 35-64), treated surgically at our Institution were identified. Transformation to dedifferentiated chordomas occurred after radiation therapy in 3 cases (mean 13.6 years after treatment, range 5-25), 2 during tumor progression, while one was de-novo. Magnetic resonance imaging and surgical examination revealed the presence of two different tumor components, corresponding to the conventional and dedifferentiated portion at histological examination. The de novo case presented a PIK3CA mutation. DNA methylation analysis revealed consistent epigenetic changes in TERT, MAGEA11 and UXT. Prognosis was poor as 5 out of 6 patients died after surgery and radiation therapy, with a mean overall survival of 29 months (range 11-52).
Skull base dedifferentiated chordomas are extremely rare and aggressive neoplasms with characteristic magnetic resonance imaging, surgical and histological features. Therefore, an early and accurate histological diagnosis is of paramount relevance. Molecular analysis appears promising to define mechanisms involved in tumor dedifferentiation 2).
Thirty-one patients with a mean age of 10.7 yr (range 0.8-22) harboring skull base chordomas were evaluated. Rassi et al., retrospectively analyzed the outcomes and prognostic factors for 18 patients treated by the senior author, with uniform management of surgery with the aim of gross total resection and adjuvant Proton beam radiotherapy. Mean follow-up was 119.2 mo (range 8-263).
Abducens nerve palsy was the most common presenting symptom. Imaging disclosed large tumors that often involve multiple anatomical compartments. Patients undergoing gross total resection had significantly increased progression free survival (P = .02) and overall survival (P = .05) compared with those having subtotal resection. Those who lived through the period of risk for recurrence without disease progression had a higher probability of living entirely free of progression (P = .03; odds ratio = 16.0). Age, sex, and histopathological variant did not yield statistical significance in survival.
Long-term overall and progression-free survival in children harboring skull base chordomas can be achieved with gross surgical resection and proton-beam radiotherapy, despite an advanced stage at presentation. Collins' law does apply to pediatric skull base chordomas, and children with this disease have a high hope for cure 3).
Shimony et al retrospectively collected all patients with skull-base chordomas operated on between 2004 and 2014. Detailed radiological description of the compartments being occupied by the tumor and the degree of surgical resection is discussed.
Eighteen patients were operated. Seventeen endoscopic surgeries were done in 15 patients, and 7 craniotomies were done in 5 patients. The mean age was 48.9 years (±19.8 years). When reviewing the anatomical compartments, they found that the most common were the upper clivus chordoma (95.6%) and lower clivus (58.3%), left cavernous sinus (66.7%) and petrous apex (∼60%). Most of the patients had intradural tumor involvement (70.8%). In all craniotomy cases, there was residual tumor in multiple compartments. In the endoscopic cases, the most difficult compartments for total resection were the lower clivus, and lateral extensions to the petrous apex or cavernous sinus.
The experience shows that the endoscopic approach is a good option for midline tumors without significant lateral extension. In cases with very lateral or lower extensions, additional approaches should be added trying to achieve complete resection 4).
A total of 47 patients (26 men; mean age 48.5 years) treated in 5 centers were included. Histology was centrally reviewed; additionally, semiquantitative N- and E-cadherin expression analysis was performed. Prognostic factors were obtained from multivariate regression models. For survival analysis the Kaplan-Meier method was used. Results The median follow-up period was 5.2 years. Complete resection, incomplete resection, and extended biopsy were performed in 14.9%, 80.9%, and 4.3% of patients, respectively. Surgical morbidity was not associated with extent of resection. Adjuvant radiation therapy was performed in 30 (63.8%) of 47 patients. The median progression-free survival (PFS) was 7.3 years. Complete resection prolonged median overall survival (OS) (p = 0.04). Male patients presented with worse PFS (4.8 years vs 9.8 years; p = 0.04) and OS (8.3 years vs not reached; p = 0.03) even though complete resection was exclusively achieved in the male subpopulation. Multivariate analysis confirmed male sex as the most important risk factor for tumor progression (p = 0.04) and death (p = 0.02). Age, duration of symptoms, initial Karnofsky Performance Scale score, brainstem compression, involvement of the petrous bone, infiltration of the dura mater, modality and dose of radiation therapy, and the E- and N-cadherin expression patterns did not gain prognostic relevance.
In skull base chordomas, male patients bear a higher risk of progressive disease and death. Male patients might benefit from more aggressive adjuvant therapy and/or from a closer follow-up schedule 5).