Clivus chordoma case reports
A case of a late-stage clival chordoma in a 29-year-old African American male and the unfortunate linear progression course since his initial diagnosis. Upon his initial encounter in 2020, radiation therapy did not offer any promising curative outcome. He was initially treated with a combination treatment of partial resection, radiation, and proposed oral imatinib, none of which modified the natural history and progression of his illness. Instead, these methods were performed as palliative measures to reduce the current size of the tumors and decrease growth rates to minimize his pain. Social issues acted as a contributory risk factor in his prognosis and due to the patient's socioeconomic barriers, he was not able to continue seeking available radiotherapy, leading to disease exacerbation. Poor adherence was noted during his follow-up. The risks of being affected by this disease are likely multifactorial and more reports of such cases need to be added to bridge this gap in the current literature. In addition, there is a gap in the current study of reports of such tumors found in diverse racial groups and in patients who are in their first few decades of life. Novel treatment strategies were reviewed, and it is expected they could generate assertive treatment guidelines 1).
describe the endoscopic endonasal transclival approach (EETCA) and its inferolateral transtubercular extension to assess the intradural surgical field gained through JT removal. We also complement the dissections with an illustrative case.
Methods: EETCA was surgically simulated, and the anatomical landmarks were assessed in eight cadaveric heads. Microsurgical dissections were additionally performed along the endoscopic surgical path. Lastly, we present an intraoperative video of the trans-JT approach in a patient with lower clival chordoma.
Results: The EETCA allowed adequate extracranial visualization and removal of the JT. The surgical bony window-obtained along the clivus and centered at the JT via the EETCA-measured 11 × 9 × 7 mm. Removal of the JT provided an improved intradural field within the lower third of the cerebellopontine cistern to expose an area bordered by the cranial nerves VII/VIII and flocculus superior and anterior margin of the lateral recess of the fourth ventricle and cranial nerves IX-XI inferiorly, centered on the foramen of Luschka.
Conclusions: Removal of the JT via EETCA improves exposure along the lower third of the cerebellopontine and upper cerebellomedullary cisterns. The inferolateral transtubercular extension of the EETCA provides access to the lateral recess of the fourth ventricle, in combination with the ventral midline pontomedullary region 2).
A technical report on the application of the microsuture technique for clivus region cerebrospinal fluid fistula closure in a patient with previous chordoma treatment and nonspecific coronavirus disease 2019 complications.
The application of the microsuture technique resulted in stable remission of the fistula and complete regression of the clinical manifestations.
The microsuture technique for clivus region fistula closure is a potentially valuable alternative to vascularized flap closure 3).
A 56-year-old female initially presented with new onset left eye visual deficits. She previously underwent sublabial transsphenoidal subtotal resection of the tumor, confirmed as clival chordoma. She subsequently presented to our institution. She was treated with an expanded endonasal resection of the remaining chordoma followed by CyberKnife radiosurgery. Two years later, surveillance imaging identified tumor recurrence within the right clivus posterior to the carotid artery. Intraoperatively, in the previously operated irradiated skull base, the normal bony architecture of the sella was absent, resulting in the inability to distinguish the anterior genu of the internal carotid artery (ICA) from the adjacent tumor. Using Doppler ultrasonography, the course of the ICA was imaged in real time, allowing for safe, gross total tumor resection. In the setting of prior operation, radiation, or extensive disease, the normal bony architecture of the sella may be disrupted, placing the cavernous ICA at risk. We report what we believe is the first use of intraoperative ultrasound during the endoscopic endonasal approach in the setting of a previously operated, radiated sella 4).
2017
A 77-year-old female was referred because of diplopia and progressively worsening headaches. Head imaging revealed a large expansile and erosive mass in the skull base. The patient underwent a successful endoscopic endonasal trans-sphenoidal resection of the mass, with biopsy confirming the diagnosis of chordoma. Postoperatively, the patient experienced an improvement in neurological symptoms. Chordomas can present a diagnostic challenge due to the rare occurrence and a tendency to involve any region within the craniospinal axis 5).
Cha et al. report two cases of pediatric PD chordoma with loss of SMARCB1/INI1 expression, which is very rare among the pediatric chordoma types. Both patients presented clival masses on preoperative MRI. Histologically, both tumors had nonclassic histologic features for conventional chordoma: sheets of large epithelioid to spindle cells with vesicular nuclei and prominent nucleoli. Both cases revealed nuclear expression of brachyury, loss of SMARCB1/INI1 expression and lack of embryonal, neuroectodermal, or epithelial component. One case showed heterozygous loss of EWSR1 gene by break-apart fluorescence in situ hybridization that reflected loss of SMARCB1/INI1 gene. Based on the clival location and histologic findings along with the loss of SMARCB1/INI1 expression and positivity for nuclear brachyury staining, the final pathologic diagnosis for both cases was PD chordoma 6).
In the Department of Neurosurgery, Austin Hospital, Heidelberg, Victoria, Australia, a case of a 25 year-old male patient with chordoma in the inferior clivus which was initially debulked via a transnasal endoscopic approach. He unfortunately had a large recurrence of tumor requiring re-do resection. With the aim to achieve maximal surgical resection, we then chose the technique of a transoral approach with Le Fort 1 maxillotomy and midline palatal split. Post-operative course for the patient was uneventful and post-operative MRI confirmed significant debulking of the clival lesion. The technique employed for the surgical procedure is presented here in detail as is our experience over two decades using this technique for tumors, inflammatory lesions and congenital abnormalities at the cranio-cervical junction 7).