Diagnosis and management of a rare case of clival chordoma in a young male patient.

Chordomas are uncommon bone slow-growing tumors developing from remnants of the notochord. They are typically seen in adults, and rarely in children. We present the case of a 16-year-old male patient with a clival chordoma, presenting with progressive headache and diplopia. In this case report we aim to provide an educational explanation of the radiological findings, diagnostic challenges, and therapeutic and management strategies.

Intradural anatomy and mobilization techniques of oculomotor, trochlear and abducens nerve after microsurgical dissection: a cadaveric study.

BACKGROUND: This study investigates the mobilization of cranial nerves in the upper clival region to improve surgical approaches. Cadaveric specimens (n = 20) were dissected to examine the oculomotor, trochlear, and abducens nerves. Dissection techniques focused on the nerves' intradural course and their relationship to surrounding structures. METHODS: Pre-dissection revealed the nerves' entry points into the clival dura and their proximity to each other. Measurements were taken to quantify these distances. Following intradural dissection, measurements were again obtained to assess the degree of nerve mobilization. RESULTS: Dissection showed that the abducens nerve takes three folds during its course: at the dural foramen, towards the posterior cavernous sinus, and lastly within the cavernous sinus. The trochlear nerve enters the dura and makes two bends before entering the cavernous sinus. The oculomotor nerve enters the cavernous sinus directly and runs parallel to the trochlear nerve. Importantly, intradural dissection increased the space between the abducens nerves (by 4.21 mm) and between the oculomotor and trochlear nerves (by 3.09 mm on average). This indicates that nerve mobilization can create wider surgical corridors for approaching lesions in the upper clivus region. CONCLUSIONS: This study provides a detailed anatomical analysis of the oculomotor, trochlear, and abducens nerves in the upper clivus. The cadaveric dissections and measurements demonstrate the feasibility of mobilizing these nerves to achieve wider surgical corridors. This information can be valuable for surgeons planning endoscopic or microscopic approaches to lesions in the upper clivus region.

Anatomical Study of the Supratentorial Extension for the Retrolabyrinthine Presigmoid Approaches.

BACKGROUND: Supratentorial craniotomy represents the upper part of the combined trans-tentorial or the supra-infratentorial presigmoid approach. In this study, we provide qualitative and quantitative analyses for the supratentorial extension of the presigmoid retrolabyrinthine suprameatal approach (PRSA). METHODS: The infratentorial PRSA followed by the supratentorial extension craniotomy with dividing and removal of the tentorial strip were performed on both sides of 5 injected human cadaver heads (n = 10 sides). Quantitative analysis was performed for the surface area gained (surgical accessibility) by adding the supratentorial craniotomy. Qualitative analysis was performed for the parts of the brainstem, cranial nerves, and vascular structures that became accessible by adding the supratentorial craniotomy. The anatomical obstacles encountered in the added operative corridor were analyzed. RESULTS: The supratentorial extension of PRSA provides an increase in surgical accessibility of 102.65% as compared to the PRSA standalone. The mean surface area of the exposed brainstem is 197.98 (standard deviation: 76.222) and 401.209 (standard deviation: 123.96) for the infratentorial and the combined supra-infratentorial presigmoid approach, respectively. Exposure for parts of III, IV, and V cranial nerves is added after the extension, and the surface area of the outer craniotomy defect has increased by 60.32%. Parts of the basilar, anterior inferior cerebellar, and superior cerebellar arteries are accessible after the supratentorial extension. CONCLUSIONS: The supratentorial extension of PRSA allows access to the supra-trigeminal area of the pons and the lower part of the midbrain. Considering this surgical accessibility and exposure significantly assists in planning such complex approaches while targeting central skull base lesions.

Navigating the complexity of skull base osteomyelitis: a case study and comprehensive review.

Skull base osteomyelitis is a rare and life-threatening infection of the skull base, commonly seen in elderly diabetic patients as a result of otogenic or paranasal infection. The diagnosis is based on a series of arguments, including a high clinical suspicion, imaging findings, negative biopsies for malignancy, and microbiological isolation. Complications, including abscess formation and vascular involvement, mandate a multidisciplinary treatment approach, primarily involving broad-spectrum antibiotics and surgical debridement, but the prognosis is usually poor. Herein, we describe the case of a 55-year-old male, who 15 years prior, underwent radiation therapy for nasopharyngeal carcinoma. He presented with an infection of the skull base with extensive bone erosion accompanied by an uncommon complication; an intra clival abscess. Despite aggressive antibiotic therapy, the patient ultimately succumbed to septic shock.

Gamma knife radiosurgery for clival metastasis: case series and systematic review.

PURPOSE: Clival metastatic cancer is rare and has limited literature to guide management. We describe management of clival metastasis with Gamma Knife radiosurgery (GKRS). We augment our findings with a systematic review of all forms of radiation therapy for clival metastasis. METHODS: Records of 14 patients with clival metastasis who underwent GKRS at the University of Pittsburgh Medical Center from 2002 to 2023 were reviewed. Treatment parameters and clinical outcomes were assessed. A systematic review was conducted using evidence-based guidelines. RESULTS: The average age was 61 years with male predominance (n = 10) and average follow-up of 12.4 months. The most common primary cancers were prostate (n = 3) and lung (n = 3). The average time from cancer diagnosis to clival metastasis was 34 months. The most common presenting symptoms were headache (n = 9) and diplopia (n = 7). Five patients presented with abducens nerve palsies, and two presented with oculomotor nerve palsies. The median tumor volume was 9.3 cc, and the median margin dose was 15 Gy. Eleven patients achieved tumor control after one procedure, and three with progression obtained tumor control after repeat GKRS. One patient recovered abducens nerve function. The median survival from cancer diagnosis and GKRS were 49.7 and 15.3 months, respectively. The cause of death was progression of systemic cancer in six patients, clival metastasis in one, and unknown in four. The systematic review included 31 studies with heterogeneous descriptions of treatment and outcomes. CONCLUSION: Clival metastasis is rare and associated with poor prognosis. GKRS is a safe, effective treatment for clival metastasis.

Endoscopic Transnasal Navigation Guided Treatment of Clivus Abscess in Adult with Mucormycosis: A Rare Report.

Osteomyelitis and abscess of the clivus are rare conditions thought to arise from contiguous spread of infection from the paranasal sinuses. Clival osteomyelitis is a rare potentially life-threatening skull base infection which is generally challenging to diagnose and treat. It is typically seen in the pediatric population and is very rare in the adult population. The exact pathophysiology of osteomyelitis of the clivus is relatively uncertain. Here, we describe a case of a 32-year-old immunocompetent female with a primary complaint of headache, with no significant medical history of diabetes, hypertension or rhinosinusitis or SARS COV-2 for the past 18 months. This case report demonstrates an image-guided and endoscopic approach to surgical localization and treatment of abscesses in the clival area.

CSF-Venous Fistula of the Clival Skull Base: A Unique Case Study and Literature Review.

An adolescent male presented with orthostatic headaches following head trauma. MRI showed cerebellar tonsil displacement and a bony defect in the clival skull base. Digital subtraction myelography (DSM) confirmed a cerebrospinal fluid-venous fistula (CVF). This was repaired endoscopically. CVFs cause uncontrolled flow of CSF into the venous system resulting in symptoms of intracranial hypotension. They're often difficult to identify on initial imaging. This is the first reported CVF originating in the central skull base, and the first treated via endoscopic trans-nasal approach. CVFs may elude initial imaging, making DSM crucial for unexplained spontaneous intracranial hypotension. Laryngoscope, 134:645-647, 2024.

Pediatric retroclival hematomas.

BACKGROUND: Traumatic retroclival hematomas (RCHs) are infrequent occurrences among the pediatric population. The existing body of research pertaining to these hematomas primarily consists of case reports or small case series, which do not provide adequate guidance for managing this condition. OBJECTIVE: This study aims to present a report on four cases of RCHs. Additionally, we aim to conduct a systematic review to consolidate the existing literature on pediatric RCHs. METHODS: The authors conducted a systematic review in accordance with the PRISMA and CARE guidelines. A multivariate logistic regression model was developed to evaluate the potential impact of various clinical variables on clinical outcomes. The study also documented four of our cases, one of which was a rare occurrence of spontaneous subdural RCH. RESULTS: A total of 62 traumatic RCHs have been documented in the literature. We documented three cases of traumatic RCHs and one case of spontaneous RCH. A systematic analysis of 65 traumatic RCHs was performed. Of trauma cases, 64.6% demonstrated craniocervical junction instability with 83.3% ligamentous involvement. Thirty-five patients were males. 50.7% were aged between 5 and 9 years. Cranial nerve palsies occurred in 29 patients (27 had abducent palsy), 26 of which resolved within 6 months of trauma. 23.5% underwent surgery, and 76.5% were conservatively managed. Surgeries targeted hematomas, hydrocephalus, or craniocervical instability. Approaches to hematomas included transclival and far/extreme lateral suboccipital approaches. Clinical outcome was good in 75.4% and intermediate or poor in 24.6%. Logistic regression suggested an association between craniocervical junction injuries and poor or intermediate outcomes (OR 4.88, 95% CI (1.17, 27.19), p = 0.04). CONCLUSION: Pediatric RCHs are mostly traumatic and extradural. Children between 5 and 9 years old are most vulnerable. Craniocervical junction injuries, mainly ligamentous, are common in RCHs and are associated with intermediate or poor outcomes. Cervical MRI could be important in cases of trauma to rule out ligamentous injuries of the craniocervical junction. The small size of RCHs should not exempt the careful assessment of craniocervical junction instability. Cranial nerve palsies are common and usually resolve within 6 months. Conservative treatment is typical unless brainstem compression, hydrocephalus, or craniocervical junction instability exists.

Rare Case of a Clival Chondroma Resulting in Panhypopituitarism.

Chondromas are rare benign tumors composed of hyaline cartilage that can arise in various locations in the body. Their occurrence in the clivus, leading to panhypopituitarism, is exceptionally rare. This case report describes a 93-year-old female with a known clival chondroma who presented with altered mental status, presumed to be secondary to toxic metabolic encephalopathy due to an infectious cause. Further diagnostic evaluation revealed pituitary hormone levels below the normal range. This case report aims to highlight a unique case of panhypopituitarism attributed to a chondroma in the clivus with tumor extension to the sellar region, emphasizing the diagnostic challenges and treatment options for this unusual pathology.

Clival ectopic pituitary prolactinoma was successfully managed by transsphenoidal surgery: A rare case report.

Key Clinical Message: Ectopic pituitary adenoma is a rare neoplasm located in the clivus and could mimic other clival tumors. Diagnosis and treatment could be challenging. It should be considered in the differential diagnosis of clival tumors. Abstract: Ectopic pituitary adenomas (EPAs) are isolated adenomas that can be located in variable locations outside the sella turcica and have a normal-appearing pituitary gland. These tumors are rare and are thought to often arise from embryological remnants along the route of Rathke's pouch migration. EPAs are associated with a wide range of clinical manifestations depending on hormonal activity and involvement of adjacent structures, which can represent a challenge in making the diagnosis and deciding on the most appropriate management. In this case study, we report a 47-year-old male who presented with visual disturbances, a headache, and generalized weakness. Magnetic resonance imaging showed a 2 cm mass located in the clivus invading the sphenoid sinus with an intact pituitary gland. The patient underwent endoscopic transsphenoidal surgery to eradicate the mass while maintaining the integrity of the pituitary gland, which was successful and uneventful. Pathological studies were consistent with prolactinoma, with no cytological malignant features. Post-surgery, symptoms notably improved, and serum prolactin levels significantly dropped, The patient's condition was satisfactory on follow-up with no long-term complications reported. This paper contributes to the existing literature by sharing the clinical management of a challenging and uncommon case.

Assessing survival outcomes and complication profiles following surgical excision and radiotherapy as interventions for skull base chordoma: a systematic review of operative margins and surgical approaches.

INTRODUCTION: Despite their precarious behavioral classification (benign and low grade on histopathology yet behaviorally malignant), great strides have been taken to improve prognostication and treatment paradigms for patients with skull base chordoma. With respect to surgical techniques, lateral transcranial (TC) approaches have traditionally been used, however endoscopic endonasal approaches (EEA) have been advocated for midline lesions. Nonetheless, due to the rarity of this pathology (0.2% of all intracranial neoplasms), investigations within the literature remain limited to small retrospective series. Furthermore, radiotherapeutic treatments investigated to date have proven largely ineffective. METHODS: Accordingly, we performed a systematic review in order to profile surgical and survival outcomes for skull base chordoma. Fixed and random-effect meta-analyses were performed for categorical variables including GTR, STR, 5-year OS, 10-year OS, 5-year PFS, and 10-year PFS. Additionally, we pooled eligible studies for formal meta-analysis to compare outcomes by surgical approach (lateral versus midline). Statistical analyses were performed using R Studio 'metafor' package or Cochrane Review Manager. Furthermore, meta-analysis of pooled mortality rates and sub-analyses of operative margin and surgical complications were used to compare midline versus lateral approaches via the Mantel-Haenszel method. We considered all p-values < 0.05 to be statistically significant. RESULTS: Following the systematic search and screen, 55 studies published between 1993 and 2022 reporting data for 2453 patients remained eligible for analysis. Sex distribution was comparable between males and females, with a slight predominance of male-identifying patients (0.5625 [95% CI: 0.5418; 0.3909]). Average age at diagnosis was 42.4 ± 12.5 years, while average age of treatment initiation was 43.0 ± 10.6 years. Overall, I2 value indicated notable heterogeneity across the 55 studies [I2 = 56.3% (95%CI: 44.0%; 65.9%)]. With respect to operative margins, the rate of GTR was 0.3323 [95% CI: 0.2824; 0.3909], I2 = 91.9% [95% CI: 90.2%; 93.4%], while the rate of STR was significantly higher at 0.5167 [95% CI: 0.4596; 0.5808], I2 = 93.1% [95% CI: 91.6%; 94.4%]. The most common complication was CSF leak (5.4%). In terms of survival outcomes, 5-year OS rate was 0.7113 [95% CI: 0.6685; 0.7568], I2 = 91.9% [95% CI: 90.0%; 93.5%]. 10-year OS rate was 0.4957 [95% CI: 0.4230; 0.5809], I2 = 92.3% [95% CI: 89.2%; 94.4%], which was comparable to the 5-year PFS rate of 0.5054 [95% CI: 0.4394; 0.5813], I2 = 84.2% [95% CI: 77.6%; 88.8%] and 10-yr PFS rate of 0.4949 [95% CI: 0.4075; 0.6010], I2 = 14.9% [95% CI: 0.0%; 87.0%]. There were 55 reported deaths for a perioperative mortality rate of 2.5%. The relative risk for mortality in the midline group versus the lateral approach group did not indicate any substantial difference in survival according to laterality of approach (-0.93 [95% CI: -1.03, -0.97], I2 = 95%, (p < 0.001). CONCLUSION: Overall, these results indicate good 5-year survival outcomes for patients with skull base chordoma; however, 10-year prognosis for skull base chordoma remains poor due to its radiotherapeutic resistance and high recurrence rate. Furthermore, mortality rates among patients undergoing midline versus lateral skull base approaches appear to be equivocal.

[Endoscopic transnasal resection of clival meningiomas]. / Endoskopicheskoe transnazal'noe udalenie meningiom skata.

Treatment of clival meningiomas is still one of the unresolved issues in modern neurosurgery. There are several treatment strategies. These ones include various combinations of follow-up, surgical CSF drainage, tumor resection and radiotherapy. OBJECTIVE: To assess postoperative outcomes in patients with clival meningiomas. MATERIAL AND METHODS: We analyzed 18 patients with large or giant clival meningiomas. RESULTS: We assessed extent of resection using the scale by G. Frank and E. Pasquini (2002): total resection - 95-100%, subtotal - 80-95%, partial - 50-80%, extended biopsy - <50% of tumor. Total resection was achieved in 1 patient (5.5%), subtotal - 5 (27.8%), partial - 12 (66.7%). At the same time, brainstem decompression and regression of hydrocephalus were observed in all cases. Fourteen patients were followed-up. Median follow-up was 8.5 months. Seventeen patients underwent radiotherapy due to predominant partial and subtotal resection. Total focal dose ranged from 50 to 57 Gy in standard fractionation mode. None patient had residual tumor enlargement throughout the follow-up period. There were no lethal outcomes. CONCLUSION: Endoscopic transnasal access to clival meningiomas in appropriate anatomical features of tumor and surrounding structures is a full-fledged alternative to transcranial treatment in these patients. This approach provides total resection and brainstem decompression. These facts increase life expectancy without deterioration of the quality of life.

Long-Term Outcomes after Multimodal Treatment for Clival Chordoma: Efficacy of the Endonasal Transclival Approach with Early Adjuvant Radiation Therapy.

This study investigates the long-term outcomes of clival chordoma patients treated with the endonasal transclival approach (ETCA) and early adjuvant radiation therapy. A retrospective review of 17 patients (2002-2013) showed a 10-year progression-free survival (PFS) rate of 67.4%, with the ETCA group showing fewer progressions and cranial neuropathies than those treated with combined approaches. The ETCA, a minimally invasive technique, provided a similar extent of resection compared to conventional skull-base approaches and enabled safe delivery of high-dose adjuvant radiotherapy. The findings suggest that ETCA is an effective treatment for centrally located clival chordomas.

Fluoroscopic Analysis of Cannula Tip Location During Radiofrequency Thermocoagulation of the Trigeminal Ganglion.

BACKGROUND: Trigeminal neuralgia (TN) is the most common excruciating cranial neuralgia in the elderly population. Radiofrequency thermocoagulation (RFT) of the trigeminal ganglion is an alternative treatment for medically intractable patients with TN. RFT cannula tip position is an important issue since it is related to treatment outcome and patient safety. OBJECTIVES: The purpose of this study was to evaluate the fluoroscopic position of a cannula tip when a maximal stimulation-induced paresthesia was obtained and the treatment outcome using a Barrow Neurological Institute (BNI) pain scale. STUDY DESIGN: Retrospective analysis. SETTING: An interventional pain management practice in South Korea. METHODS: The final cannula tip position obtained under maximal electrical stimulation of the face was analyzed using previously saved fluoroscopic images. RESULTS: The cannula tip was located exactly in the clival line in 10 patients (29.4%) with maxillary division (V2) TN. There were 24 patients of V2 TN (70.5% ) in whom the cannula tip was located below the clival line. Over 50% of cannula tips were located at -11 mm to -15 mm below the clival line in mandibular division (V3) TN. Forty-four patients (83%) who received RFT in the trigeminal ganglion demonstrated BNI I or II. LIMITATIONS: The number of patients with V3 TN was smaller than that of V2 TN. Only short-term efficacy was evaluated, but not long-term efficacy or recurrence rate of facial pain. CONCLUSIONS: Nearly 70% of patients in V2 TN and all patients in V3 TN, the cannula tip was positioned below the clival line. RFT of the trigeminal ganglion showed a successful treatment outcome with BNI I or II in 83% of patients.

Clival intraosseous myxoma treated with an endoscopic endonasal approach: illustrative case.

BACKGROUND: Solitary lesions of the clivus, especially nonchordomatous ones, are exceptionally rare pathologies representing only 0.1%-0.2% of intracranial tumors that may present diagnostic and therapeutic challenges. Intraosseous myxomas are benign tumors arising from mesenchymal cells with an unknown pathophysiology. OBSERVATIONS: In this article, a 15-year-old male with clival intraosseous myxoma who was treated via an endoscopic endonasal surgical approach is presented. The patient became symptomatic after a baseball struck his head, leading to throbbing headaches and visual difficulties for tracking objects. Examination noted diplopia, cranial nerve VI palsy, and ptosis. After imaging revealed a tumor extending to the left cavernous sinus with bulging of the dorsal clivus against the basilar artery/ventral pons, resection was performed. Postoperatively, the patient noted ocular motility and alignment improvement without further complications. LESSONS: Clival masses present with symptoms from compromised neurovascular structures including visual disturbances and trigeminal sensory deficits. Given the rarity of these entities, patients may postpone further treatment until workup; this patient was misdiagnosed for possible concussion until several weeks passed. A PubMed database review of cranial myxoma cases was conducted to identify solitary clival intraosseous myxoma cases. This case is one of the few in which this pathology was treated through an endoscopic endonasal operative approach without complications, demonstrating its safety and effectiveness.

Lynch syndrome-associated chordoma with high tumor mutational burden and significant response to immune checkpoint inhibitors.

Chordoma is a rare malignant bone tumor arising from notochordal tissue. Conventional treatments, such as radical resection and high-dose irradiation, frequently fail to control the tumor, resulting in recurrence and re-growth. In this study, genetic analysis of the tumor in a 72-year-old male patient with refractory conventional chordoma of the skull base revealed a high tumor mutational burden (TMB) and mutations in the MSH6 and MLH1 genes, which are found in Lynch syndrome. The patient and his family had a dense cancer history, and subsequent germline genetic testing revealed Lynch syndrome. This is the first report of a chordoma that has been genetically proven to be Lynch syndrome. Chordomas usually have low TMB; however, this is an unusual case, because the TMB was high, and immune checkpoint inhibitors effectively controlled the tumor. This case provides a basis for determining the indications for immunotherapy of chordoma based on the genetic analysis. Therefore, further extensive genetic analysis in the future will help to stratify the treatment of chordoma.

Impact of Treatment Modalities upon Survival Outcomes in Skull Base and Clival Chordoma: An NCDB Analysis.

Objectives Skull base chordomas are locally aggressive malignant tumors derived from the notochord remnant. There are limited large-scale studies examining the role and extent of surgery and radiation therapy. Design Analysis of the National Cancer Database (NCDB) was performed to evaluate the survival outcomes of various treatments, and to assess for predictors of overall survival (OS). Participants This is a retrospective, population-based cohort study of patients diagnosed with a clival/skull base chordoma between 2004 and 2015 in the NCDB. Main Outcome Measures The primary outcome was overall survival (OS). Results In all, 468 cases were identified. Forty-nine percent of patients received surgery and 20.7% had positive margins. Mean age at diagnosis was 48.4 years in the surgical cohort, and 55% were males. Of the surgical cohort, 33.8% had negative margins, 20.7% had positive margins, and 45.5% had unknown margin status. Age ≥ 65 (hazard ratio [HR]: 3.07; 95% confidence interval [CI]: 1.63-5.76; p < 0.001), diagnosis between 2010 and 2015 (HR: 0.49; 95% CI: 0.26-0.90; p = 0.022), tumor size >5 cm (HR: 2.29; 95% CI: 1.26-4.15; p = 0.007), and government insurance (HR: 2.28; 95% CI: 1.24-4.2; p = 0.008) were independent predictors of OS. When comparing surgery with or without adjuvant radiation, no survival differences were found, regardless of margin status ( p = 0.66). Conclusion Surgery remains the mainstay of therapy. Advanced age (>65 years), large tumor size, and government insurance were predictors of worse OS. Whereas negative margins and the use of adjuvant radiation did not appear to impact OS, these may very well reduce local recurrences. A multidisciplinary approach is critical in achieving optimal outcomes in this challenging disease.

Acute myeloid leukemia associated with CHARGE syndrome.

CHARGE syndrome is a malformation disorder with diverse phenotypes that shows autosomal dominance with heterozygous variants in the chromodomain helicase DNA-binding 7 (CHD7) gene. Only a few cases of CHARGE syndrome accompanied by neoplasm during childhood have been reported. We report the case of a girl with CHARGE syndrome who developed acute myelogenous leukemia at 12 years old. She had mild intellectual disability, and hearing loss with inner ear malformation, myopia, astigmatism, laryngotracheal malacia, hypogonadism, and clival hypoplasia, with a history of patent ductus arteriosus. The patient was genetically diagnosed with CHARGE syndrome based on the detection of a novel heterozygous frameshift pathogenic variant in the CHD7 gene. We review the reported pediatric cases of CHARGE syndrome with malignancy and suggest a possible molecular mechanism of carcinogenesis involving pathogenic variants of the CHD7 gene.

A 13-year patient journey of infant giant clival chordoma: case report and literature review.

Chordomas are rare malignant bone tumours that develop from the ectopic remnants of the embryonic notochord. In contrast to adults, the majority in children under 16 present intra-cranially (63%). In 2006, we reported the youngest case of a large clival chordoma, a 15-week old baby, the second case to present without skull base involvement and the fourth case of chordoma in a patient with tuberous sclerosis (TS) Kombogiorgas (Childs Nerv Syst 22(10):1369-1374, 2006). In this report, we provide an update on this patient's journey through a range of therapeutic options and summarize an update of the literature, since 2006, for this patient group.

Endoscopic endonasal resection of a primary intraosseous clival myxoma: illustrative case.

BACKGROUND: An intraosseous myxoma is a rare, benign mesenchymal tumor that penetrates the bone. The occurrence of an intraosseous myxoma in the clivus is a unique presentation of the disease. OBSERVATIONS: The authors discuss the case of a 15-year-old male with a new diagnosis of a primary clival intraosseous myxoma presenting with cranial nerve VI palsy. This is the third documented case of this pathology occurring in the clivus. This patient was successfully treated with endoscopic endonasal resection of the tumor. LESSONS: Primary clival intraosseous myxomas are extremely rare, but nonetheless it is important to add it to the differential diagnosis of clival masses. This mass has a high risk of recurrence, and prior literature suggests gross total resection may improve chances of progression-free survival. However, further larger studies are needed to provide guidelines regarding proper management of this pathology.