Spontaneous regression of an interhemispheric arachnoid cyst: illustrative case.

BACKGROUND: Intracranial arachnoid cysts are benign collections of cerebrospinal fluid that are often asymptomatic and discovered incidentally. An interhemispheric location of these lesions is rare, with only a few such cases reported in the literature. Though spontaneous regression of arachnoid cysts has been described in other locations, to date this phenomenon has not been reported in interhemispheric fissure cysts. OBSERVATIONS: In this report, we describe a patient with a large, multiloculated interhemispheric arachnoid cyst diagnosed on prenatal ultrasound. She did not exhibit neurologic deficits or signs of increased intracranial pressure and was observed with serial imaging. After several years of observation, imaging revealed spontaneous and progressive decrease in the cyst size. LESSONS: We illustrate a case of regression of an interhemispheric arachnoid cyst in a pediatric patient. To our knowledge, this is the first reported case of spontaneous shrinkage of an arachnoid cyst in this location. Although the current presentation is rare, this reporting adds to the current understanding of natural history of arachnoid cysts and provides an example of radiographical improvement without intervention of a cyst located within the interhemispheric fissure.

Effect of distance from target on hypopituitarism after stereotactic radiosurgery for pituitary adenomas.

INTRODUCTION: Delayed hypopituitarism is the most common complication after stereotactic radiosurgery (SRS) for pituitary adenomas. The aim of this study was to investigate the relationship between the distance from the hypothalamic-pituitary axis to the treatment target and anterior pituitary function preservation after SRS. METHODS: Between 2007 and 2020, consecutive adult patients who underwent single-session SRS for non-functioning or hormone-secreting pituitary adenomas with ≥ 6 months of follow-up were included. Distance measurements between hypothalamic-pituitary axis structures and the SRS target volume were quantified on MRI. The primary outcome was anterior pituitary function preservation. Outcomes were compared using multivariable regression and area under the receiver operator characteristic curve (AUROC) analyses. RESULTS: The study cohort comprised 224 patients, who were categorized by preservation (n = 168) and no preservation (n = 56) of anterior pituitary function after SRS. The mean and median clinical follow-up durations were 53.7 (38.0) and 46.0 (17.0-75.0) months, respectively. Independent predictors of anterior pituitary function preservation were a greater distance between the center of the pituitary gland and center of the SRS target [OR 1.101 (1.000-1.213), p = 0.050], and a shorter clinical follow-up duration [OR 0.985 (0.977-0.993), p < 0.0001]. The adjusted AUROC for the distance from the center of the pituitary gland and center of the SRS target in predicting anterior pituitary function preservation was 0.595. The sensitivity, specificity, positive predictive value and negative predictive value in predicting anterior pituitary function preservation at the optimal cut-off distance of 15 mm were 30.0, 88.0, 89.9 and 26.2%, respectively. CONCLUSIONS: Greater distance between the normal pituitary gland and the SRS target is associated with anterior pituitary function preservation and increasing this distance should be a goal of adenoma resection. Larger prospective, multi-center studies are necessary to corroborate this finding and establish the effects of distance on hypopituitarism after SRS for pituitary adenomas.

Impact of histopathological classification of non-functioning adenomas on long term outcomes: comparison of the 2004 and 2017 WHO classifications.

PURPOSE: Outcomes of patients with non-functioning pituitary adenomas categorized using the 2004 and 2017 WHO classification systems are understudied. We report outcomes from the University of Virginia of patients with non-functioning pituitary adenomas categorized using both systems. METHODS: We constructed a database from all 239 patients who underwent resection of a non-functioning pituitary adenoma between 2003 and 2015 and had at least 5 years of follow-up. Pathologic diagnosis was determined under both the 2004 and 2017 WHO classification systems. We compared the rates of recurrence and progression between subtypes using univariate and multivariate Cox regression analyses. RESULTS: Nearly 30% of the tumors in our database were classified as null cell adenomas under the 2004 classification system, whereas only 10% of the tumors were classified as null cell adenomas using the 2017 classification system. Most of these tumors were reclassified as either corticotroph or gonadotroph adenomas. Despite our relatively large cohort and average follow-up of nearly 9 years, we did not detect a significant difference in recurrence and progression between subtypes. CONCLUSIONS: The majority of null cell adenomas diagnosed under the 2004 WHO classification system are reclassified as gonadotroph or corticotroph adenomas under the 2017 WHO classification system. Rates of progression and recurrence between subtypes are not as different as previously believed at our institution and require a larger cohort to further investigate.

Characterization of a paradoxical post-operative increase in serum cortisol in Cushing disease.

PURPOSE: In Cushing disease, early post-operative serum cortisol fluctuations have not been adequately characterized, and their association with initial remission and recurrence is unclear. METHODS: A retrospective cohort study of patients with Cushing disease was conducted at two institutions. A "riser" was defined a priori as a paradoxical increase in serum cortisol with an immediate incremental increase in serum cortisol over three consecutive cortisol draws separated by roughly 6-h (definition 1). Post hoc analyses used a definition of two consecutive increases (definition 2). Risers were compared to non-risers for initial remission and time-to-recurrence. RESULTS: A total of 505 patients with Cushing disease were screened, and 469 had adequate data for group assignment. Analysis of post-operative cortisol showed a subgroup of "risers" with a frequency of 3.6% for definition 1 and 42.6% for definition 2. In these patients, cortisol levels were significantly higher until approximately 36 h post-operatively, and cortisol had a significantly longer mean serum half-life. In the post hoc analysis, definition 2 risers had a lower remission rate compared to non-risers (162/196, 82.7%, versus 243/264, 92.0%) with an odds ratio of 0.41 (0.23-0.73; p = 0.003). For both definitions, recurrence was similar between groups. CONCLUSIONS: We found that almost half of Cushing disease patients experienced a temporary increase in serum cortisol level during the early post-operative period. Serum cortisol half-life was longer, and the remission rates were lower, however, recurrence rates were similar to non-risers.

Compressive epidural collection as an unusual complication of FESS for a pediatric mycetoma.

Functional endoscopic sinus surgery (FESS) is a treatment of choice for fungal sinus ball (mycetoma), which is considered safe with a very low major complication rate. We present an unusual case of a 12-year-old female, who underwent FESS for a sphenoid sinus mycetoma and which was complicated by an acute, compressive epidural fluid collection. This presumably resulted from sinus irrigation in the setting of an under-appreciated skull base and mucosal defects causing a ball-valve effect. Our patient had a temporary neurologic deficit with complete recovery, however, similar complications can have fatal consequences.

Three-dimensional printing and craniosynostosis surgery.

OVERVIEW: The goal of this study was to review the current application and status of three-dimensional printing for craniosynostosis surgery. METHODS: A literature review was performed using the PubMed/MEDLINE databases for studies published between 2010 and 2020. All studies demonstrating the utilization of three-dimensional printing for craniosynostosis surgery were included. RESULTS: A total of 15 studies were ultimately selected. This includes studies demonstrating novel three-dimensional simulation and printing workflows, studies utilizing three-dimensional printing for surgical simulation, as well as case reports describing prior experiences. CONCLUSION: The incorporation of three-dimensional printing into the domain of craniosynostosis surgery has many potential benefits. This includes streamlining surgical planning, developing patient-specific template guides, enhancing residency training, as well as aiding in patient counseling. However, the current state of the literature remains in the validation stage. Further study with larger case series, direct comparisons with control groups, and prolonged follow-up times is necessary before more widespread implementation is justified.

Long-Term Neurocognitive Outcomes in Sagittal Synostosis: The Impact of Reoperation.

INTRODUCTION: Optimal age at surgery in nonsyndromic sagittal craniosynostosis continues to be debated. Previous reports suggest that earlier age at whole vault cranioplasty more frequently requires reoperation. It is unknown, however, whether reoperation affects neurocognitive outcome. This study examined the impact of reoperation on neurocognitive outcome in children with nonsyndromic sagittal craniosynostosis using comprehensive neurocognitive testing. METHODS: Forty-seven school-age children (age 5-16 years) with nonsyndromic sagittal craniosynostosis who underwent whole-vault cranioplasty were included in this analysis. Participants were administered a battery of standardized neuropsychological testing to measure neurocognitive outcomes. RESULTS: Thirteen of the 47 participants underwent reoperation (27.7%); 11 out of the 13 reoperations were minor revisions while 2 reoperations were cranioplasties. Reoperation rate was not statistically different between patients who had earlier surgery (at age ≤6 months) versus later surgery (at age >6 months) (P > 0.05). Nonreoperated patients who had only one later-in-life surgery did not perform statistically better than reoperated patients on any outcome measure of neurocognitive function, including IQ, academic achievement, visuomotor integration, executive function, and behavior. Comparing reoperated earlier surgery patients with nonreoperated later surgery patients, reoperated earlier surgery patients had higher full-scale and verbal IQ (P < 0.05), scored higher on word reading, reading comprehension, spelling, numerical operations, and visuomotor integration (P < 0.05), and had fewer indicators of suspected learning disabilities (P < 0.01) compared to nonreoperated later surgery patients. CONCLUSION: Reoperation rate after whole vault cranioplasty was 27.7%, with few cases of repeat cranioplasty (4.2% of all patients). Reoperation was not associated with worse neurocognitive outcome. Reoperated earlier surgery patients in fact performed better in IQ, academic achievement and visuomotor integration when compared to nonreoperated later surgery patients.

Genomic and molecular characterization of pituitary adenoma pathogenesis: review and translational opportunities.

OBJECTIVE: Innovations in genomics, epigenomics, and transcriptomics now lay the groundwork for therapeutic interventions against neoplastic disease. In the past 30 years, the molecular pathogenesis of pituitary adenomas has been characterized. This enhanced understanding of the biology of pituitary tumors has potential to impact current treatment paradigms, and there exists significant translational potential for these results. In this review the authors summarize the results of genomics and molecular biology investigations into pituitary adenoma pathogenesis and behavior and discuss opportunities to translate basic science findings into clinical benefit. METHODS: The authors searched the PubMed and MEDLINE databases by using combinations of the keywords "pituitary adenoma," "genomics," "pathogenesis," and "epigenomics." From the initial search, additional articles were individually evaluated and selected. RESULTS: Pituitary adenoma growth is primarily driven by unrestrained cell cycle progression, deregulation of growth and proliferation pathways, and abnormal epigenetic regulation of gene expression. These pathways may be amenable to therapeutic intervention. A significant number of studies have attempted to establish links between gene mutations and tumor progression, but a thorough mechanistic understanding remains elusive. CONCLUSIONS: Although not currently a prominent aspect in the clinical management of pituitary adenomas, genomics and epigenomic studies may become essential in refining patient care and developing novel pharmacological agents. Future basic science investigations should aim at elucidating mechanistic understandings unique to each pituitary adenoma subtype, which will facilitate rational drug design.

Endoscopic endonasal surgery outcomes for pediatric craniopharyngioma: a systematic review.

OBJECTIVE: The goal of this study was to systematically review the outcomes of endoscopic endonasal surgery (EES) for pediatric craniopharyngiomas so as to assess its safety and efficacy. METHODS: A systematic literature review was performed using the PubMed and MEDLINE databases for studies published between 1986 and 2019. All studies assessing outcomes following EES for pediatric craniopharyngiomas were included. RESULTS: Of the total 48 articles identified in the original literature search, 13 studies were ultimately selected. This includes comparative studies with other surgical approaches, retrospective cohort studies, and case series. CONCLUSIONS: EES for pediatric craniopharyngiomas is a safe and efficacious alternative to other surgical approaches. Achieving gross-total resection with minimal complications is feasible with EES and is comparable, if not superior in some cases, to traditional means of resection. Ideally, a randomized controlled trial might be implemented in the future to further elucidate the effectiveness of EES for resection of craniopharyngiomas.

Chiari I malformation in children-the natural history.

PURPOSE: To review the natural history of asymptomatic and symptomatic pediatric Chiari I malformations with and without syringomyelia. MATERIALS AND METHODS: We reviewed the literature for case reports and case series describing the natural history of asymptomatic and symptomatic children with Chiari I malformations with and without syringomyelia. Our review included approximately 700 asymptomatic children without syringomyelia, 100 symptomatic children without syringomyelia, 22 asymptomatic children with syringomyelia, and 11 symptomatic children with syringomyelia. Symptomatic and imaging outcomes at the point of last reported follow-up were noted to describe the natural history of Chiari I malformations in children. RESULTS: Our review of about 700 asymptomatic children with CM-I without syrinx revealed that most children do not exhibit new-onset symptoms (5-6%) or syrinx (2-3%). The nearly 100 published cases of symptomatic CM-I without syrinx suggest that about half of children report symptomatic improvement (48%) and few report symptomatic worsening (7%). New-onset syrinx is rarely observed (2%). Few cases have been published about asymptomatic and symptomatic CM-I with syrinx as syringomyelia are generally regarded to be an indication for surgical intervention. Nevertheless, all 22 children with asymptomatic CM-I with syringomyelia included in this study were asymptomatic at follow-up, with syrinx resolution observed in 18 children and tonsillar herniation improvement observed in 16 children. Overall, our review of asymptomatic pediatric CM-I with or without syringomyelia suggests that its natural history is much more favorable than previously acknowledged and that the literature generally favors conservative management of these cases. CONCLUSION: Our review of asymptomatic pediatric CM-I with or without syringomyelia suggests that its natural history is much more favorable than previously acknowledged and that the literature generally favors conservative management of these cases. Further study of symptomatic pediatric CM-I is necessary to better understand its natural history.

Ganglioglioma Arising from the Septum Pellucidum: Case Report and Review of the Literature.

BACKGROUND: Gangliogliomas are low-grade neoplasms that typically affect patients under the age of 30 and present with epilepsy and symptoms of mass effect. Here, we report a case of an intraventricular ganglioglioma involving the septum pellucidum in a pediatric patient with history of optic glioma. Only one other pediatric intraventricular ganglioglioma arising from the septum pellucidum has been reported previously. CASE REPORT: The patient initially presented at 9 months of age with a pilocytic astrocytoma centered on the optic chiasm, treated with chemotherapy and radiation at 3 years of age. Routine follow-up imaging at 13 years of age revealed the development of a mass in the septum pellucidum, which was subtotally resected endoscopically because of its proximity to the fornices. Pathology confirmed a ganglioglioma positive for the BRAF V600E mutation. The tumor residual progressed and was treated with stereotactic radiosurgery. The patient was asymptomatic at her 6-month follow-up visit and the size of the nodule remained stable. LITERATURE REVIEW: Our review of the 25 previously reported intraventricular gangliogliomas found that their pre-surgical diagnoses were often incorrect, reflecting the difficulty of making the diagnosis with signs, symptoms, and imaging alone. Patients can be reassured that the prognosis is generally favorable following uncomplicated neurosurgical resection.

Deletion of 6p25.3 Is Associated with Cerebrovascular Dolichoectasia: Report of 2 Cases.

Developmental dolichoectasia of the intracranial vessels is a rare occurrence. The authors report 2 sibling pediatric patients who were born with 6p25.3 deletion, associated with carotid and vertebrobasilar dolichoectasia. MRI imaging of both children showed asymptomatic elongation and dilation of the vertebrobasilar system and "kissing" carotid arteries. A microarray analysis was also performed for both patients, which identified a 1.5-Mb deletion of 6p25.3 covering 15 genes including FOXC1, which has been implicated in defects in vascular morphogenesis.

Decrease of Serum IGF-I following Transsphenoidal Pituitary Surgery for Acromegaly.

BACKGROUND: In the immediate postoperative period following resection of growth hormone (GH)-secreting pituitary tumors, serum concentrations of GH have limited ability to predict remission of acromegaly. Since many actions of GH actions are mediated by insulin-like growth factor-1 (IGF-I), we aimed to determine the rates of fall of IGF-I during 72 h after surgical resection of pituitary tumors. METHODS: We studied patients who were undergoing pituitary surgery for acromegaly. IGF-I was measured by LC-MS and GH by immunoassay. Remission was defined by the combination of serum GH <0.4 ng/mL during oral glucose tolerance testing performed 8 weeks after the surgical procedure and normal IGF-I at ≥8 weeks. RESULTS: During the first 72 h after surgery, the mean (SD) rate of decline of IGF-I was 185 (61) ng/mL per 24 h in those who achieved remission (n = 23), with a mean (SD) apparent half-life of 55 (19) h. IGF-I had decreased to <65% of the preoperative IGF-I on postoperative day 2 in 20 of 23 remission patients (87%) vs none of 5 patients who did not achieve remission. GH was <2.7 ng/mL on day 2 in 21 of 23 remission patients (91%), but in none of the nonremission patients. The combination of IGF-I and GH on day 2 separated the remission and nonremission groups of patients. CONCLUSIONS: Rapid decline of serum IGF-I during the immediate postoperative period warrants further study as an analytically independent adjunct to GH measurement for early prediction of biochemical remission of acromegaly.

Surgical management of Cushing's disease.

BACKGROUND: Transsphenoidal selective adenomectomy is the first-line treatment for Cushing's disease. At experienced centers, early remission rates after transsphenoidal surgery range from 65 to 98%, however disease relapse frequently occurs with rates ranging from 2 to 35% at long-term follow up. METHODS: This article discusses recently reported studies on the surgical outcomes from transsphenoidal surgery for Cushing's disease. CONCLUSIONS: One of the keys to a successful long-term surgical outcome is meticulous dissection using the adenoma's pseudocapsule as a surgical plane for complete resection. MRI-negative and invasive ACTH-secreting adenomas pose particular challenges for pituitary surgeons.

Effective time window in reducing pituitary adenoma size by gamma knife radiosurgery.

OBJECTIVES: Although the effectiveness of gamma knife radiosurgery (GKRS) in controlling the size of pituitary adenomas has been well demonstrated in many studies, the time period in which significant changes in tumor size occurs has been investigated in a limited fashion. It is important to determine the therapeutic window of GKRS in treating pituitary adenomas, i.e., the effective timeframe during which significant size reduction of these tumors occurs, so that alternative treatments such as further GKRS or microsurgery might be prescribed in a timely manner if clinically indicated. METHODS: This was a nested sample of an ongoing local cohort study on GKRS for pituitary adenomas at the University of Virginia. Magnetic resonance imaging (MRI) using dedicated sequences was employed. Only patients with a baseline MRI (TP0) and at least 1 follow-up study performed in the University Hospital after GKRS were included. The follow-up scans were performed at five time-points (TP1-TP5) which were 6, 12, 24, 36 and 48 months after GKRS. The dimensional indices of the tumors were measured in three orthogonal planes, i.e., transverse (TR), antero-posterior (AP) and cranio-caudal (CC). The volumes of the tumors were estimated by using the following formula: [Formula: see text]. Tumor volume decrease by more than 25% from baseline was considered as 'shrinkage', <25% tumor size increase or decrease was considered 'static', and more than 25% increase as 'increment'. Our cohort consisted of 21 patients, with functioning adenomas in 13 subjects i.e. six adrenocorticotrophic hormone (ACTH)-secreting and seven growth hormone (GH)-secreting, and non-functioning (NF) adenomas in eight subjects. RESULTS: In 26 adenomas (8 ACTH, 9 GH and 9 NF), tumor control (tumor shrinkage or static) were achieved in 21 tumors (80.8%); 89, 75, and 78% for GH-secreting, ACTH-secreting and NF adenomas respectively, at the end of the 4-year follow-up period. Analysis of variance showed significant differences of GKRS margin dose among different types of tumors (p = 0.013), but not of baseline tumor volumes (p = 0.240). Logistic regression analysis showed no significant association of margin dose, baseline volume or tumor type with the tumor control outcome. Comparison of tumor change using dimensional indices relative to the base time point (TP0) showed that in the sample there was an average reduction of 1.290 mm at TP1 (6 months) with p values 0.155 (parametric t test) and 0.098 (non-parametric Wilcoxon signed-ranked test) respectively, showing a moderate reduction in tumor dimensional indices. The change in dimensional indices at later time points (TP2-TP5) showed an average reduction ranging from 1.930 to 2.471 mm. Significant reduction in the mean dimensional indices was firstly observed at TP2 (1 year) with p values 0.013 (t test) and 0.018 (Wilcoxon signed-rank test). Such scale of reduction in the dimensional indices appeared to be maintained along the time axis (from TP2 to TP5). CONCLUSIONS: Significant decrease in tumor dimensional indices tended to occur at 1 year post-GKRS. Although to a lesser extent, such decrease in dimensional indices continued up to the end of our follow-up period.

Calvarial Regeneration With Use of Acellular Dermal Matrix in Aplasia Cutis Congenita.

Aplasia cutis congenital (ACC) is a rare congenital anomaly, most commonly affecting the scalp, with a variable penetrance ranging from a small (<2 cm) area of missing skin to large defects characterized by absent skin, subcutaneous tissue, calvarium, and dura. Calvarial reconstruction in ACC can be challenging. Due to exposed neurologic structures, in large defects, ACC has a high mortality rate. A stable reconstruction is optimally achieved shortly after birth to minimize complications. Herein the authors present a case of a neonate with an extensive (4.5 × 7 cm) cutis aplasia defect associated with absent skin, subcutaneous tissue, calvarium, dura, and with exposed cortical surface and sagittal sinus. This defect was successfully reconstructed in a single stage with the use of an acellular dermal matrix/skin graft construct. The acelluar dermal matrix served as a scaffold for tissue ingrowth, promoting regeneration of the bony calvarium as well as soft tissue. At 18-month follow-up, the patient exhibits a 50% smaller calvarial defect as well as stable skin coverage.

Impact of supplemental vitamin K1 administration on postoperative blood component requirements after craniosynostosis repair: a prospective, placebo-controlled, randomized, blinded study.

Total cranial vault craniosynostosis repairs often require additional blood transfusions in the intensive care unit. Vitamin K1 participates in hepatic production of procoagulant proteins, and body stores of vitamin K1 are limited and dietary dependent. Surgical stress and diet interference may place infants at risk for vitamin K deficiency. Through design of a surgically stratified, randomized, placebo-controlled, blinded pilot study, we evaluated impact of vitamin K1 supplementation on coagulation parameters in infants after craniosynostosis repair. Patients received intramuscular vitamin K1 or placebo coincident with surgical incision. Serum vitamin K1 levels, protein induced in vitamin K absence-prothrombin, and factor VII were obtained at predetermined intervals after surgery. Patients received blood products in the intensive care unit in accordance with transfusion thresholds. Fifteen patients (vitamin K1 = 6, placebo = 9) completed the study procedures. Despite group assignment, patients received an average of 3 postoperative transfusions. Variations were observed with respect to intraoperative resuscitation of patients between comparably trained pediatric anesthesiologists. Thirty-three percent of patients were vitamin K1 deficient on 1 or more laboratory specimens. All breast-fed patients became deficient. Compared with placebo, elevated serum vitamin K1 levels at 6, 12, and 24 hours in the active drug group (P < 0.0001) were not associated with increased factor VII levels or reduced need for postoperative blood products. However, lack of a standardized intraoperative resuscitation plan may contribute to postoperative coagulopathy and is a major study limitation.

Nontraumatic symptomatic de novo arachnoid cyst in an adolescent patient treated with cystoperitoneal shunting: illustrative case.

BACKGROUND: Arachnoid cysts are often congenital, asymptomatic lesions detected in the pediatric population. When seen in adults, they usually occur following trauma. De novo formation of arachnoid cysts is uncommon, with only a few instances cited in the literature and most of which occurred in the pediatric population. Treatment options for these lesions include observation, craniotomy for cyst resection, microsurgical/endoscopic fenestration, or shunting. OBSERVATIONS: In this report, the authors describe a female patient with a de novo, symptomatic, enlarging middle cranial fossa arachnoid cyst detected at age 16 years. She was treated with the placement of a cystoperitoneal shunt. After surgery, she experienced clinical and radiological improvement. LESSONS: We illustrate successful shunting of a de novo arachnoid cyst in a symptomatic teen patient. Although arachnoid cysts in certain intracranial locations are more likely to produce symptoms, those in the middle cranial fossa tend to be asymptomatic. To our knowledge, this is the first reported case of a symptomatic de novo arachnoid cyst located in the middle cranial fossa in a postpubertal patient. Although the current presentation is rare, the authors demonstrate an effective surgical treatment of a symptomatic, large, de novo arachnoid cyst in a postpubertal pediatric patient.

Prognostic Factors and Nomogram for Choroid Plexus Tumors: A Population-Based Retrospective Surveillance, Epidemiology, and End Results Database Analysis.

Background: Choroid plexus tumors (CPTs) are rare neoplasms found in the central nervous system, comprising 1% of all brain tumors. These tumors include choroid plexus papilloma (CPP), atypical choroid plexus papilloma (aCPP), and choroid plexus carcinoma (CPC). Although gross total resection for choroid plexus papillomas (CPPs) is associated with long-term survival, there is a scarcity of prospective data concerning the role and sequence of neoadjuvant therapy in treating aCPP and CPC. Methods: From the years 2000 to 2019, 679 patients with CPT were identified from the Surveillance, Epidemiology, and End Result (SEER) database. Among these patients, 456 patients had CPP, 75 patients had aCPP, and 142 patients had CPC. Univariate and multivariable Cox proportional hazard models were run to identify variables that had a significant impact on the primary endpoint of overall survival (OS). A predictive nomogram was built for patients with CPC to predict 5-year and 10-year survival probability. Results: Histology was a significant predictor of OS, with 5-year OS rates of 90, 79, and 61% for CPP, aCPP, and CPC, respectively. Older age and African American race were prognostic for worse OS for patients with CPP. Older age was also associated with reduced OS for patients with aCPP. American Indian/Alaskan Native race was linked to poorer OS for patients with CPC. Overall, treatment with gross total resection or subtotal resection had no difference in OS in patients with CPP or aCPP. Meanwhile, in patients with CPC, gross total resection (GTR) was associated with significantly better OS than subtotal resection (STR) only. However, there is no difference in OS between patients that receive GTR and patients that receive STR with adjuvant therapy. The nomogram for CPC considers types of treatments received. It demonstrates acceptable accuracy in estimating survival probability at 5-year and 10-year intervals, with a C-index of 0.608 (95% CI of 0.446 to 0.77). Conclusions: This is the largest study on CPT to date and highlights the optimal treatment strategies for these rare tumors. Overall, there is no difference in OS with GTR vs. STR in CPP or aCPP. Furthermore, OS is equivalent for CPC with GTR and STR plus adjuvant therapy.