Treatment of Clival Chordomas: A 20-Year Experience and Systematic Literature Review
Abstract
:Simple Summary
Abstract
1. Introduction
2. Materials and Methods
2.1. Retrospective Case Series
2.2. Literature Review
2.2.1. Surgical Series
2.2.2. Molecular Studies
3. Results
3.1. Review of the Literature
3.1.1. Surgical Series
3.1.2. Molecular Studies
3.2. Institutional Series
4. Discussion
4.1. Not Controversial Issues
4.1.1. Age
4.1.2. Pathology (Proliferative Index)
4.1.3. Radiotherapy
4.2. Controversial Issues
4.2.1. Surgery
4.2.2. Recurrences and Definition of Cure
4.3. Limitations of the Study
5. Conclusions
Author Contributions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Acknowledgments
Conflicts of Interest
References
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Author, Year | N. of Patients | Surgical Approach | Complications | GTR | Follow-Up (Months) | Recurrence | Outcome |
---|---|---|---|---|---|---|---|
Samii, 2007 [16] | 49 | Open (49) | CSF leak 5.4%, meningitis 5.4% | 49% | 63 | NA | 5-year OS 65% |
Koutourousiou, 2012 [17] | 60 | Endoscopic (NA), open (NA) | CSF leak 20%, CN injury 6.7% | 67% | 17.8 | 20% | NA |
Ouyang, 2014 [18] | 77 | Endoscopic (NA), open (NA) | Overall 27.8%, CN injury 18.2% | 33% | 60 | NA | 3-year PFS 92.0% |
Jahangiri, 2015 [19] | 50 | Endoscopic (34), open (9), combined (7) | CSF leak 12%, CN injury 6%, meningitis 12% | 52% | 41 | 51% | NA |
Zhang, 2016 [20] | 32 | Endoscopic (32) | CSF leak 12% | 28% | 20 | NA | 5-year PFS 16.5% 5-year OS 69.5% |
Raza, 2018 [21] | 29 | NA | NA | 41% | 28 | NA | Disease-specific survival 44.4 months |
Zoli, 2018 [22] | 65 | Endoscopic (65) | CSF leak 2.5% | 59% | 52 | 40% | NA |
Wang, 2020 [23] | 49 | Endoscopic (49) | CSF leak 30.1%, CN injury 5.5% | 64–85% | 41.5 | 14% | Mortality 12% |
Bai, 2022 [8] | 284 | Endoscopic (349), open (31) | CSF leak 3.9% | 40% | 43.9 | 55% | 5-year disease-specific survival 71.0% |
Passeri, 2022 [24] | 210 | Endoscopic (142), open (123) | CSF leak 12.1%; CN injury 17.7% | 44% | 59.2 | 42% | 5-year PFS 52.1% 5-year OS 75.1% |
Author, Year | Material | Main Findings | |
---|---|---|---|
Bank Cells | Patients’ Tissues | ||
Tamborini et al., 2010 [25] | Sacrum, spine, and clivus chordomas | The existence of an autocrine/paracrine loop involving some imatinib-related RTKs | |
Horbinski et al., 2010 [26] | Skull base chordomas | Identification of biomarkers with a prognostic role | |
Le et al., 2011 [27] | Spine and skull base chordomas | High genomic instability (large copy number losses) | |
Shalaby et al., 2011 [28] | U-CH1 | Sacrum, spine, and skull base chordomas | The importance of molecular studies for targeted therapies (like EGFR antagonists) |
Aydemir et al., 2012 [3] | U-CH1 | Chordomas, nucleus pulposum | Characterization of chordoma stem cells |
Bayrak et al., 2013 [29] | U-CH1 | Chordomas, nucleus pulposum | Identification of down and upregulated miRNAs |
Kitamura et al., 2013 [30] | Skull base chordomas | Identification of specific genetic/molecular and clinical prognostic factors | |
Choy et al., 2014 [31] | Sacrum, spine, and skull base chordomas | Point mutations in tumor suppressor genes | |
Scheil-Bertram et al., 2014 [32] | U-CH1, 2 | Chordomas, nucleus pulposum | Identification and validation of genes involved in chordomas genesis |
Zhang et al., 2014 [33] | U-CH1, U-CH2 | Clival chordomas | Identification of down-regulated miRNAs as a potential therapeutic tool |
von Witzleben et al., 2015 [34] | U-CH1, 2, 3, 6, 7, 11, 12 | Sacrum | Development of new cell lines and evaluation of CDK4/6 inhibitors |
Wang et al., 2016 [35] | Sacrum and spine chordomas | Alterations of chromatin regulatory genes (SETD2) | |
Bai et al., 2020 [36] | Clival chordomas | Role of LncRNA in dural penetration |
Pt N# | Age | Sex | Symptom at Onset | N° of Surgeries | Surgical Approach | EOR | Complications | Adj. RxTp after First Surgery | Histology | Proliferative Index at First Surgery | Recurrence | Treatment of Recurrence | Pt Outcome (Yrs) |
---|---|---|---|---|---|---|---|---|---|---|---|---|---|
1 | 55 | F | diplopia | 1 | EEA | GTR | - | yes | chordoma pan-CK+/S100+/vimentin+ | 1–2% | no | AWD (4) | |
2 | 23 | M | diplopia | 1 | EEA | GTR | - | yes | chordoma s100+/EMA+/hTERT+/p53- | 1.5% | no | AFD (17) | |
3 | 57 | F | incidental | 1 | EEA | GTR | - | yes | chordoma pan-CK+/S100+/vimentin+/EMA+ | 5% | no | AWD (3) | |
4 | 29 | M | diplopia | 1 | EEA | GTR | - | yes | chordoma pan-CK+/S100+/vimentin+/EMA+ | 1% | no | AWD (3) | |
5 | 32 | M | incidental | 1 | EEA | STR | - | NA | chordoma pan-CK+/S100+/vimentin+ | 2% | NA | NA | |
6 | 41 | M | diplopia, loss of vision | 1 | EEA | GTR | - | yes | chordoma S100+/vimentin+/brachyury+ | NA | no | AFD (10) | |
7 | 80 | F | diplopia | 2 | EEA; STA | STR | - | no | chordoma pan-CK+/S100+/vimentin+/EMA+ | 5% | Yes @ 3 yrs | EEA + RT | AWD (6) |
8 | 59 | M | nystagmus, diplopia | 1 | EEA | GTR | CSF leak | no | chordoma pan-CK+/S100+/vimentin+ | 3% | no | DD (1) | |
9 | 40 | M | diplopia, headache | 1 | EEA | STR | - | yes | chordoma pan-CK+/S100+/vimentin+ | 3% | yes @ 5 yrs | RT | AWD (7) |
10 | 53 | M | diplopia | 1 | EEA | GTR | - | yes | chordoma pan-CK+/vimentin+ | NA | no | DOD (5) | |
11 | 76 | F | dysphonia | 1 | CR | GTR | - | yes | - | <2% | no | DOD (6) | |
12 | 54 | F | diplopia | 4 | EEA | PR | hearing loss; bilateral trigeminal pain | yes | chordoma pan-CK+/vimentin+ | NA | yes @ 4 yrs | STA + RT+ sirolimus (7 mL/die) and erlotinib (150 mg/die); @3 yrs STA; EEA | DD (7) |
13 | 43 | F | diplopia | 5 | EEA | STR | ophthalmoplegia, hypovisus | no, not indicated due to rapid residual growth | chordoma pan-CK+/S100+/vimentin+ | 2% | Yes @ <1 yrs | EEA + RT; EEA + RT; NA (other hospital), NA (other hospital) | AWD (6) |
14 | 85 | M | incidental | 2 | EEA | STR | - | NA | chordoma pan-CK+/S100+/vimentin+ | 5–10% | yes @ 2 yrs | EEA | DOD (3) |
15 | 23 | M | diplopia | 3 | EEA | PR | - | yes, proton + Imatinib | chordoma pan-CK+/S100+/vimentin+ | 2% | Yes @<1 yrs | EEA + C0 − C4 fixation, sirolimus (7 mL/die) and erlotinib (150 mg/die); CR | DOD (18) |
16 | 69 | M | diplopia | 2 | EEA | GTR | - | no | chordoma pan-CK+/vimentin+/S100+ | 2–3% | yes @5 yrs | EEA + RT | AWD (7) |
17 | 23 | M | incidental | 1 | CR | GTR | - | yes | chordoma CAM 5.2+/Vimentin+/S100+ | NA | NA | AFD (17) | |
18 | 82 | F | diplopia | 2 | STA | STR | visual impairment bilaterally, right VI CN palsy, ipsilateral hearing loss | no | chordoma | NA | yes @1 yr | STA | DD (2) |
19 | 41 | M | diplopia | 1 | CR | GTR | - | yes | chordoma | NA | no | AFD (24) | |
20 | 42 | M | V2 hypoaesthesia, dysphagia | 1 | CR | GTR | right VI CN palsy; obstructive hydrocephalus requiring CSF shunting | no | chordoma pan-CK+/S100+/vimentin+ | NA | no | DD (2) | |
21 | 40 | M | diplopia | 3 | CR | STR | left VII and VIII CN palsy and dysphagia; hemiplegia | yes + Cyber knife | chordoma pan-CK+/S100+/vimentin+/p53+ 30% | 8–10% | yes @ 2 yrs | CR; @<1 yr CR | DD (4) |
22 | 43 | M | left tinnitus | 1 | STA | GTR | CSF leak | NA | chordoma pan-CK+/S100+/ p53 +/brachyury+ | 3–4% | no | - | AFD (11) |
23 | 60 | F | rhinosinusitis | 1 | STA | GTR | CSF leak | NA | chordoma pan-CK+/S100+/vimentin+ | NA | no | AFD (16) | |
24 | 39 | F | diplopia | 4 | STA | GTR | - | no | chordoma | NA | yes @4 yrs | CR; @5 yrs STA+ RT; @3 yrs CR | DOD (30) |
25 | 72 | M | incidental | 1 | STA | GTR | seizure | no | chordoma pan-CK+/vimentin+ | 2% | no | DOD (7) | |
26 | 68 | F | diplopia | 1 | STA | GTR | - | NA | chordoma pan-CK+/S100+/vimentin+/p53 15% | 1% | no | DOD (2) | |
27 | 31 | M | diplopia | 4 | STA | GTR | obstructive hydrocephalus requiring CSF shunting; hemorrhagic infarct | no | chordoma p53+ | NA | yes @ 2 yrs | STA+RT; @4 yrs EEA+ Cyber Knife; @2 yrs CR | AFD (20) |
28 | 51 | M | V2 hypoaesthesia | 5 | STA | GTR | hypopituitarism, meningitis | no | chordoma pan-CK+/S100+/vimentin+ | 3% | yes @ 5 yrs | CR+ RT; @ 12 y STA + RT; @1 yrs EEA; @1 yr EEA + Sirolimus | DD (20) |
29 | 60 | M | diplopia | 3 | STA | STR | hypovisus | no | chordoma pan-CK+/S100+/vimentin+ | 2% | yes @ 2 yrs | EEA; @1 yr EEA | DOD (3) |
30 | 71 | F | diplopia | 2 | STA | STR | NA | no | chordoma | NA | yes @ 3 yrs | STA | DD (4) |
31 | 48 | F | diplopia | 2 | STA | STR | - | no | chordoma pan-CK+/vimentin+ | 4–5% | yes @ <1 yr | STA + RT | AFD (11) |
32 | 66 | F | diplopia | 2 | STA | GTR | - | no | chordoma pan-CK+/S100+/vimentin+/h-TERT+/p53+ 6–8% | 2% | yes @ 5 yrs | STA | AFD (17) |
33 | 55 | F | diplopia | 4 | STA | STR | bilateral VI CN palsy | yes | chordoma pan-CK+/S100+; p53+; hTERT+; | 1–12% | yes @ 2 yrs | STA; @ 2 yrs STA + RT; STA + chemo | DD (10) |
34 | 47 | F | headache | 3 | STA | GTR | CSF leakage; hypopituitarism | no | chordoma S100+/vimentin+ | 1% | yes @ 10 yrs | STA; @ 9 yrs EEA + RT | AWD (20) |
35 | 33 | M | headache | 1 | CR + c0-c2 fixation | NA | NA | chordoma pan-CK+/vimentin+/S100+ | NA | NA | DD (3) | ||
36 | 26 | F | tinnitus, left tongue fasciculation | 7 | CR + c0-c2 fixation | STR | VII to XII CN palsy | RT+oxygen-ozone therapy and Temodal and homeopathic anticancer therapies | chordoma pan-CK+/S100+/vimentin+; p53+ | 5–6% | yes @ 4 yrs | CR; (second surgical time) CR; CR + Sirolimus; EEA and transoral combined; @1 yr CR; EEA; STA + Radiotherapy | DD (8) |
37 | 44 | M | V3 hypoaesthesia, ptosis | 5 | STA | STR | hemiparesis, VI CN palsy; obstructive hydrocephalus requiring CSF shunting | no | chondroid chordoma; VEGF+, EGFRvIII+ | NA | yes @ <1 yr | STA; Cyber knife; STA; EEA; EEA + Bevacizumab + Erlotinib | DD (5) |
38 | 37 | M | diplopia | 2 | EEA | STR | - | no | chordoma; HTERT+; p53+ | NA | yes @ 1 yr | EEA | DD (13) |
39 | 79 | F | hypovisus | 6 | STA | NA | - | no | chordoma ck+/S100+/p53+; vimentin+/hTERT-; | 4–8% | NA | DOD (3) | |
40 | 66 | F | NA | 2 | STA | NA | - | NA | chordoma pan-CK+/S100+/vimentin+ | 3% | NA | NA (1) | |
41 | 65 | F | NA | 1 | EEA | NA | - | NA | chordoma pan-CK+/S100+/vimentin+ | NA | NA | NA (1) | |
42 | 36 | F | headache, diplopia | 1 | STA | NA | - | NA | chordoma pan-CK+/S100+/vimentin+/p53- | 3% | NA | AFD (20) |
Covariate | B | SE | Wald | p | Hazard Ratio | 95% CI |
---|---|---|---|---|---|---|
Age | −0.9215 | 0.4708 | 3.8307 | 0.0503 | 0.3979 | 0.1581 to 1.0013 |
Proliferative index | 0.9323 | 0.5355 | 3.0306 | 0.0817 | 2.5402 | 0.8893 to 7.2562 |
Radiotherapy | 1.1323 | 0.5045 | 5.0372 | 0.0248 | 3.1028 | 1.1543 to 8.3409 |
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Noya, C.; D’Alessandris, Q.G.; Doglietto, F.; Pallini, R.; Rigante, M.; Mattogno, P.P.; Gessi, M.; Montano, N.; Parrilla, C.; Galli, J.; et al. Treatment of Clival Chordomas: A 20-Year Experience and Systematic Literature Review. Cancers 2023, 15, 4493. https://doi.org/10.3390/cancers15184493
Noya C, D’Alessandris QG, Doglietto F, Pallini R, Rigante M, Mattogno PP, Gessi M, Montano N, Parrilla C, Galli J, et al. Treatment of Clival Chordomas: A 20-Year Experience and Systematic Literature Review. Cancers. 2023; 15(18):4493. https://doi.org/10.3390/cancers15184493
Chicago/Turabian StyleNoya, Carolina, Quintino Giorgio D’Alessandris, Francesco Doglietto, Roberto Pallini, Mario Rigante, Pier Paolo Mattogno, Marco Gessi, Nicola Montano, Claudio Parrilla, Jacopo Galli, and et al. 2023. "Treatment of Clival Chordomas: A 20-Year Experience and Systematic Literature Review" Cancers 15, no. 18: 4493. https://doi.org/10.3390/cancers15184493