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Objective: Chordomas represent one of the most challenging subsets of skull base and craniovertebral junction (CVJ) tumors to treat. Despite extensive resection followed by proton-beam radiation therapy, the recurrence rate remains high, highlighting the importance of developing efficient treatment strategies. In this study, the authors present their experience in treating clival and CVJ chordomas over a 29-year period., Methods: The authors conducted a retrospective study of clival and CVJ chordomas that were surgically treated at their institution from 1991 to 2020. This study focuses on three aspects of the management of these tumors: the factors influencing the extent of resection (EOR), the predictors of survival, and the outcomes of the endoscopic endonasal approaches (EEAs) compared with open approaches (OAs)., Results: A total of 265 surgical procedures were performed in 210 patients, including 123 OAs (46.4%) and 142 EEAs (53.6%). Tumors that had an intradural extension (p = 0.03), brainstem contact (p = 0.005), cavernous sinus extension (p = 0.004), major artery encasement (p = 0.01), petrous apex extension (p = 0.003), or high volume (p = 0.0003) were significantly associated with a lower EOR. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 52.1% and 75.1%, respectively. Gross-total resection and Ki-67 labeling index < 6% were considered to be independent prognostic factors of longer PFS (p = 0.0005 and p = 0.003, respectively) and OS (p = 0.02 and p = 0.03, respectively). Postoperative radiation therapy correlated independently with a longer PFS (p = 0.006). Previous surgical treatment was associated with a lower EOR (p = 0.01) and a higher rate of CSF leakage after EEAs (p = 0.02) but did not have significantly lower PFS and OS compared with primary surgery. Previously radiation therapy correlated with a worse outcome, with lower PFS and OS (p = 0.001 and p = 0.007, respectively). EEAs were more frequently used in patients with upper and middle clival tumors (p = 0.002 and p < 0.0001, respectively), had a better rate of EOR (p = 0.003), and had a lower risk of de novo neurological deficit (p < 0.0001) compared with OAs. The overall rate of postoperative CSF leakage after EEAs was 14.8%., Conclusions: This large study showed that gross-total resection should be attempted in a multidisciplinary skull base center before providing radiation therapy. EEAs should be considered as the gold-standard approach for upper/middle clival lesions based on the satisfactory surgical outcome, but OAs remain important tools for large complex chordomas.

Objective: The endoscopic endonasal approach (EEA) to the lower clivus and craniovertebral junction (CVJ) has been traditionally performed via resection of the nasopharyngeal soft tissues. Alternatively, an inferiorly based rhinopharyngeal (RP) flap (RPF) can be dissected to help reconstruct the postoperative defect and separate it from the oropharynx. To date, there is no evidence regarding the viability and potential clinical impact of the RPF. The aim of this study was to assess RPF viability and its impact on clinical outcome., Methods: A retrospective cohort of 60 patients who underwent EEA to the lower clivus and CVJ was studied. The RPF was used in 30 patients (RPF group), and the nasopharyngeal soft tissues were resected in 30 patients (control group)., Results: Chordoma was the most common surgical indication in both groups (47% in the RPF group vs 63% in the control group, p = 0.313), followed by odontoid pannus (20% in the RPF group vs 10%, p = 0.313). The two groups did not significantly differ in terms of extent of tumor (p = 0.271), intraoperative CSF leak (p = 0.438), and skull base reconstruction techniques other than the RPF (nasoseptal flap, p = 0.301; fascia lata, p = 0.791; inlay graft, p = 0.793; and prophylactic lumbar drain, p = 0.781). Postoperative soft-tissue enhancement covering the lower clivus and CVJ observed on MRI was significantly higher in the RPF group (100% vs 26%, p < 0.001). The RPF group had a significantly lower rate of nasoseptal flap necrosis (3% vs 20%, p = 0.044) and surgical site infection (3% vs 27%, p = 0.026) while having similar rates of postoperative CSF leakage (17% in the RPF group vs 20%, p = 0.739) and meningitis (7% in the RPF group vs 17%, p = 0.424). Oropharyngeal bacterial flora dominated the infections in the control group but not those in the RPF group, suggesting that the RPF acted as a barrier between the nasopharynx and oropharynx., Conclusions: The RPF provides viable vascularized tissue coverage to the lower clivus and CVJ. Its use was associated with decreased rates of nasoseptal flap necrosis and local infection, likely due to separation from the oropharynx.

Objective: The anteromedial triangle (AMT) is the triangle formed by the ophthalmic (V1) and maxillary (V2) nerves. Opening of this bony space offers a limited access to the sphenoid sinus (SphS). This study aims to demonstrate the utility of the orbitopterygopalatine corridor (OPC), obtained by enlarging the AMT and transposing the contents of the pterygopalatine fossa (PPF) and V2, as an entrance to the SphS, maxillary sinus (MaxS), and nasal cavity., Methods: Five formalin-injected cadaveric specimens were used for this study (10 approaches). A classic pterional approach was performed. An OPC was created through the inferior orbital fissure, between the orbit and the PPF, by transposing the PPF inferiorly. The extent of the OPC was measured using neuronavigation and manual measurements. Two illustrative cases using the OPC to access skull base tumors are presented in the body of the article., Results: Via the OPC, the SphS, MaxS, ethmoid sinus (EthS), and nasal cavity could be accessed. The use of endoscopic assistance through the OPC achieved better visualization of the EthS, SphS, MaxS, clivus, and nasal cavity. A significant gain in the area of exposure could be achieved using the OPC compared to the AMT (22.4 mm2 vs 504.1 mm2)., Conclusions: Opening of the AMT and transposition of V2 and the contents of the PPF creates the OPC, a potentially useful deep keyhole to access the paranasal sinuses and clival region through a middle fossa approach. It is a valuable alternative approach to reach deep-seated skull base lesions infiltrating the cavernous sinus and middle cranial fossa and extending into the paranasal sinus.

Intraventricular rupture of a colloid cyst is a rare phenomenon and has been proposed as a mechanism for sudden death in patients with colloid cysts. Imaging of a colloid cyst during rupture has been described in only one other instance. The authors report a highly unusual case of a 53-year-old man who presented with acute onset headaches and imaging findings of hydrocephalus caused by a colloid cyst originating from the septum pellucidum and superior surface of the roof of the third ventricle. Interestingly, the colloid cyst revealed imaging signs of intraventricular rupture characterized by a tail-like drainage of cystic contents into the occipital horn of the lateral ventricle. The patient was surgically treated with a craniotomy and transcallosal approach to the colloid cyst, where it was noted that the cyst wall was spontaneously open. This rare case highlights unique imaging findings of a rare event in an infrequent pathology confirmed with intraoperative microscopy. The authors further document the process of cyst rupture and speculate on its pathomechanisms.