Case Of Craniopharyngioma Research Articles

PDOC-12 PEDIATRIC BRAIN TUMOR INCIDENCE, AND TREATMENT MODALITY AT KILIMANJARO CHRISTIAN MEDICAL CENTER

Abstract INTRODUCTION Pediatric brain tumors (PBT) a significant health concern, they are the primary cause of cancer-related deaths among children. PBTs in Low- and Middle-Income Countries are diagnosed late due to insufficient resources. This abstract describes Pediatric Brain tumor incidence and treatment modalities at Kilimanjaro Christian Medical Center (KCMC) Cancer Care Center (CCC), Moshi, Tanzania from December 2016 to March 2024. METHOD Data on pediatric patients treated at the CCC between Dec. 2016 and March 2024, were analyzed. Inclusion criteria were a primary brain tumor and patients aged 0-19 at the time of diagnosis. All patients were previously diagnosed by computer tomography/ magnetic resonance imaging. Variables retrospectively collected from patient’s medical records were: demographic characteristics (age, gender, Place of residence, and insurance status), previous history of malignant diseases, incidence date, and treatment modality. RESULTS Total of 748 cases of pedeatric cancer were diagnosed at CCC. Of all the pediatric cancers diagnosed, brain cancer accounted for 22(2.9%). Out of these cases of brain cancer, 63.6% were males and 36.4% were females.Majority of them came within the Kilimanjaro region. 41% were palliated while 59% had a curative intent at the time of diagnosis, 22.7% died between December 2016 to March 2024.Embryonal tumors were (40.9%) with a breakdown that includes 6 Medulloblastoma and 3 cases of Craniopharyngioma (teratoid/rhabdoid), gliomas (36.4%) with sub-types such as Ependymoma, Anaplastic Astrocytoma, Pilocytic Astrocytoma, Pineocytoma, Stem glioma, and Tectal glioma, unspecified cases (13.6%), and Pineoblastoma were (9.1%). Surgery was used in 40.9% of cases, radiotherapy in 31.8%, and chemotherapy in 72.7%. Chemoradiotherapy was used in 31.8% of cases. 13.6% of cases utilized a combined approach, including chemotherapy, radiotherapy, and surgery. CONCLUSION PBT incidence at KCMC is 2.9%, improving access to screening, accurate diagnosis and treatment modalities are essential in identifying and managing PBT in our setting.

PDOC-01 THERAPEUTIC DIFFICULTY IN THE MANAGEMENT OF A RECURRENT CRANIOPHARYNGIOMA IN A CHILD: REVIEW OF THE LITERATURE AND CASE REPORT

Abstract BACKGROUND Craniopharyngiomas are rare, benign epithelial tumors of embryonic origin in children that develop along the craniopharyngeal duct from Rathke’s pouch. The optimal treatment algorithm remains challenging. We report a child case of recurrent craniopharyngioma with therapeutic challenges. CASE PRESENTATION 12-year-old patient, followed for a multi-operated type 2 craniopharyngioma (December 2017, November 2018, and April 2022), stroke on left sylvian stenosis with right hemiplegia in 2020 during proton therapy (52 Gray), thrombolysed with good recovery after rehabilitation, sequellar panhypopituitarism. He was seen in consultation for visual field deterioration. Clinical examination showed a conscious, coherent, obese patient with a deteriorating visual field. Ophthalmological examination showed visual acuity 10/10 with slight temporal visual field amputation on the left and monocular blindness on the right due to probable optic atrophy sequelae of the craniopharyngioma. Magnetic resonance imaging revealed progression of the residual multicystic lesion in the sellar and left temporal regions, entering the sylvian valley and severely compressing the optic pathways. The tumor was removed under general anesthetic in a supine position, with release of the optic nerve, the carotid artery, and its division, and the left 3rd cranial nerve. The postoperative course is straightforward. CONCLUSION Morbidity and the assessment of sequelae are now at the forefront of the management of craniopharyngiomas. The current therapeutic approach should be reconsidered, in terms of the indications for surgery and the surgical protocol. This patient’s case is a typical example of the therapeutic difficulties involved in the management of craniopharyngiomas.

Papillary Craniopharyngioma: an integrative and comprehensive review.

Papillary craniopharyngioma (PCP) is a rare type of tumor, comprising ∼20% of all craniopharyngioma (CP) cases. It is now recognized as a separate pathological entity from the adamantinomatous type. PCPs are benign tumors, classified as WHO grade 1, characterized by non-keratinizing squamous epithelium. They typically grow as solid and round papillomatous masses or as unilocular cysts with a cauliflower-like excrescence. PCPs primarily occur in adults (95%), with increased frequency in males (60%) and predominantly affect the hypothalamus. Over 80% of these tumors are located in the third ventricle, expanding either above an anatomically intact infundibulum (strictly third ventricle tumors) or within the infundibulo-tuberal region of the third ventricle floor. Clinical manifestations commonly include visual deficits and a wide range of psychiatric disturbances (45% of patients), such as memory deficits and odd behavior. MRI can identify up to 50% of PCPs by the presence of a basal duct-like recess. Surgical management is challenging, requiring complex approaches to the third ventricle and posing significant risk of hypothalamic injury. The endoscopic endonasal approach allows radical tumor resection and yields more favorable patient outcomes. Of intriguing pathogenesis, over 90% of PCPs harbor the somatic BRAFV600E mutation, which activates the mitogen-activated protein kinase (MAPK/ERK) signaling pathway. A phase 2 clinical trial has demonstrated that PCPs respond well to BRAF/MEK inhibitors. This comprehensive review synthesizes information from a cohort of 560 well-described PCPs and 99 large CP series including PCP cases published from 1856-2023 and represents the most extensive collection of knowledge on PCPs to date.

The impact of spheno-occipital synchondrosis exposure via extended endoscopic endonasal surgery on midface growth in pediatric patients.

Pediatric extended endonasal procedures pose significant surgical challenges. Lesions from the suprasellar region to the lower clivus necessitate extensive exposure. This study examined whether drilling the spheno-occipital synchondrosis (SOS) to remove the posterior clinoid process and dorsum sellae (DS) for greater exposure affects pediatric midfacial growth. From 2014 to 2020, the authors performed endoscopic endonasal surgery (EES) in 14 patients aged 12 years or younger. The lesions consisted of 11 cases of craniopharyngioma, 1 pituitary neuroendocrine tumor, 1 Rathke's cleft cyst, and 1 Langerhans cell histiocytosis. In 8 of the 14 cases, an extended EES procedure was used by exposing the SOS to remove the posterior clinoid process and DS. Measurement of the central face was based on head MRI before and after surgery. Measuring points were the sellae-nasion (SN) plane, the foremost points of the anterior maxilla (point A), and the maximum concavity point of the mandibular symphysis (point B). The authors measured and evaluated the SNA angle (angle created by the SN plane and the NA [a line connecting point A and the nasion] plane), SNB angle (angle created by the SN plane and the NB [a line connecting point B and the nasion] plane), and the ANB angle (angle created by the NA plane and the NB plane). In addition, a comparison was made with 6 pediatric cases in which transcranial surgery was performed for craniopharyngiomas. In the extended EES group, the average preoperative age was 7 years, and the average postoperative age was 12 years. Mean preoperative angles in this group were 84° (SNA), 80.9° (SNB), and 3.1° (ANB); mean postoperative angles were 83.5° (SNA), 83.9° (SNB), and -0.4° (ANB). In the standard EES group, the average preoperative age was 9 years, and the average postoperative age was 14.5 years. Average preoperative angles in the standard EES group were 83° (SNA), 80.3° (SNB), and 2.7° (ANB); average postoperative angles were 82.7° (SNA), 81° (SNB), and 1.6° (ANB). In the transcranial surgery group, the average preoperative age was 4.5 years, and the average postoperative age was 9.8 years. Mean preoperative angles were 83.8° (SNA), 80.3° (SNB), and 3° (ANB); mean postoperative angles were 83.8° (SNA), 82.6° (SNB), and 1.2° (ANB). The only significant difference between groups was the postoperative ANB angle, which was negative in the extended EES group compared to the standard EES group, indicating the maxilla was positioned posteriorly compared to the mandible. The measurement values of the EES groups and the transcranial surgery group exhibited minimal differences, except for a significant decrease in the postoperative ANB angle in the extended EES group compared with the standard EES group. These results show that extended EES may impact midface growth. Further research is required to understand the long-term impact of SOS exposure.

The Use of Diffusion Tensor Imaging in the Differentiation and Surgical Planning of Suprasellar Hypothalamic-Opticochiasmatic Glioma and Craniopharyngioma in Children

Background and ObjectivesSuprasellar hypothalamic-opticochiasmatic glioma (HOCG) and craniopharyngioma (CP) have similar appearances on conventional MRI and are difficult to distinguish. Moreover, these tumors are situated near vital structures like the optic chiasm and hypothalamus, rendering conventional surgery susceptible to significant complications. We mainly discussed the surgical application value and diagnostic value of diffusion tensor imaging (DTI) in HOCG and CP. MethodsThe retrospective analysis of 13 cases of HOCG and 16 cases of CP was conducted. All patients underwent conventional MRI and DTI prior to surgery, and were pathologically diagnosed postoperatively. ResultsBoth CP and HOCG appeared as heterogeneous mixed signal masses on conventional MRI. For HOCGs, fiber tractography revealed two different growth patterns of the tumor: infiltrative type and inflated type. The surgical approach and risk levels differ between these growth patterns. Additionally, fiber tractography demonstrates significant differences compared to CPs. The surgical approach and extent of resection for all cases of these two tumors were guided by DTI. ConclusionDTI enhances the accuracy of HOCG and CP differentiation. Furthermore, patterns of tractography described in this study assist neurosurgeons in delineating the surgical pathway and tumor resection range without damaging important fiber bundles, thereby avoiding permanent neurological deficits and improving survival quality for patients.

Analysis of Immunohistochemical Expression of BRAF (V600E) Mutation in Serrated Colorectal Polyps: A Study from Tertiary Hospital in Oman.

Colorectal cancer (CRC) is considered one of the most common cancers in the world. Serrated polyps were found to be precursor lesions for CRC. BRAF mutation (V600E) has been strongly linked to the development of these lesions. No previous study concerning BRAF immunohistochemical expression in serrated polyps- was done in Oman. The primary objective of our study was to assess the prevalence of BRAF (V600E) mutation in serrated colorectal polyps in the Omani population. The secondary objectives were to assess the prevalence of serrated polyps and their characteristic features: type, site and size as well as the relationship between BRAF (V600E) mutation and polyp type, site and size. Ninety-one hyperplastic polyps (HP) (76.5%), 24 sessile serrated lesions (SSL) (20.2%) and 4 cases of tubular adenomas with low grade dysplasia (3.4%) were studied for BRAF (V600E) immunohistochemical expression. No case of traditional serrated adenoma (TSA) was present. Control cases of craniopharyngioma and papillary thyroid carcinoma were included. BRAF (V600E) IHC was positive in 63 of the HP polyps (69.2%), 13 SSLs (54.2%) and none of the adenomatous polyps. The majority of positive polyps (75.0%) were ≤5 mm in size, 17.9% were 5-10 mm and 7.1% were ≥10 mm in size. The majority of BRAF (V600E) positive polyps (68.1 %) were in the distal colon and 31.9 % were in the proximal colon. The majority of positive cases for BRAF (V600E) were showing multiple polyps (61.8 %). None of the tubular adenomas showed any BRAF (V600E) positivity. Serrated polyps are now well known for their potential to develop CRC. Immunohistochemistry is an easy and reproducible way to detect BRAF (V600E) mutation. Our study showed there is high prevalence (64.3%) of BRAF mutation in serrated polyps in the Omani population. The majority of these polyps- were HP and SSL; and ≤5 mm in size and located in the distal colon.

Clinical analysis of resection and dura skull base reconstruction of cranionasal communication tumor in 31 cases

Objective:To explore the methods of resection, dura and skull base repair and reconstruction of cranionasal communication tumor. Methods:Data of 31 patients with cranionasal communication tumor who underwent dura and skull base reconstruction after tumor resection from 2018 to 2022 were collected. Follow-up lasted for 3 to 41 months. Results:A total of 31 patients were enrolled, including 20 males and 11 females. The ages ranged from 19 to 74 years, with a median age of 57 years old. There were 17 benign lesions（one case of hemangioma, one case of Rathke cyst, one case of squamous papilloma, one case of craniopharyngioma, two cases of meningocele, two cases of varus papilloma, two cases of meningioma of grade Ⅰ, three cases of schwannoma, four cases of pituitary tumor） and 14 malignant lesions（one case of osteosarcoma, one case of poorly differentiated carcinoma, two cases of varus papilloma malignancy, two cases of olfactory neuroblastoma, two cases of adenocarcinoma, two cases of adenoid cystic carcinoma, four cases of squamous cell carcinoma） . Sixteen cases underwent nasal endoscopy combined with craniofacial incision and 15 cases underwent nasal endoscopy surgery alone. Complete resection of the mass and dura and skull base reconstruction were performed in all 31 patients, and free graft repair was performed in 8 cases（fascia lata in 5 cases and nasal mucosa in 3 cases）. Twenty-three cases were repaired with pedicled flaps（septal mucosal flap alone in 11 cases, septal mucosal flap combined with free graft in 6 cases, and cap aponeurosis combined with free graft in 6 cases）. Eight out of 31 patients underwent skull base bone repair. Postoperative cerebral hemorrhage occurred in 1 case, cerebrospinal fluid leakage in 1 case, intracranial infection in 2 cases. All patients were successfully treated without severe sequelae. Cerebrospinal fluid leakage and intracranial infection occurred in one patient after radiotherapy, who recovered after conservative treatment. All 17 patients with benign lesions survived. Thirteen out of 14 patients with malignant lesions received radiotherapy after surgery, nine survived without recurrence, five cases recurred, of which 2 survived with tumor, one underwent reoperation and 2 died. Conclusion:Cranionasal communication tumors are high-risk diseases of anterior and middle skull base, and various surgical repair methods could be selected after complete resection of the tumor. Successful reconstruction and multidisciplinary cooperation are crucial for treatment outcome.

P.142 The use of intraoperative magnetic resonance imaging for endoscopic transnasal transsphenoid surgery in children

Background: Sellar and suprasellar pediatric lesions are uncommon. Endoscopic transnasal transphenoidal surgery (ETTS) is the preferred treatment, but early post-op MRI is hindered by sphenoidal packing. This study aims to assess iMRI safety and efficacy in pediatric ETTS cases. Methods: We performed a retrospective review from Jan 01, 2015 to Dec 31, 2022, evaluating use of iMRI. We determined if the goals of the surgery (biopsy, cyst decompression, subtotal resection, gross total resection) were met, and iMRI’s influence on surgery outcomes. We examined patient age, surgery duration, length of stay, histopathology results, surgical complications, post-op MRIs within 1 month, and tumor progression/recurrence. Results: Over eight years, 20 pediatric ETTS procedures, 14 with iMRI, were conducted. Achieving goals in 13 cases, iMRI prompted extra surgery once. Two adenomas progressed, requiring a second surgery, and craniopharyngioma cases had complications, needing further interventions. Hospital stays varied (1-9 days), with a mean surgery duration of 6 hours and 47 minutes. The study underscores iMRI’s potential impact, stressing the necessity for more research in pediatric transsphenoidal surgeries. Conclusions: While intraoperative MRI in pediatric transsphenoidal surgeries may aid goal verification, this small study doesn’t conclusively demonstrate improved outcomes. Complication rates align with non-IMRI procedures, highlighting the need for further research.

Demographic Profile of CNS Tumors and Histopathological Evaluation of Pituitary Lesions: An Institutional Study

Primary Central Nervous System tumors (CNS trs) are a diverse group of neoplasm showing significant geographic variations in incidence, morbidity, mortality, and survival. Pituitary adenomas (PA) recently termed as Pituitary Neuroendocrine Tumours (PitNET) are the commonest mass in the sellar region, can produce local, and distant effects.1 However, some developmental, inflammatory, and neoplastic lesions may primarily or secondarily involve the sella, mimicking pituitary tumors; recognition of which is crucial for the appropriate management. Aims: To evaluate the histopathological profile of primary CNS trs and to correlate with age, sex and site at presentation. Pituitary lesions are further analyzed to find out prevalence of non adenomatous lesions in pituitary Materials and methods: Histologically diagnosed 79 primary CNS trs and 18 pituitary lesions are analyzed. Results: Meningioma is the commonest brain and CNS trs. The PA being the second commonest CNS trs, constitute 17% of CNS trs, 22% of brain tumors and 78% of pituitary lesions. Other sellar lesions were single cases of Rathkeys cleft cyst (RCC), Arachnoid cyst, craniopharyngioma and pituitary apoplexy. Conclusion: This retrospective study established a baseline profile of brain tumors and sellar lesions based primarily on the histopathological experience at a tertiary care hospital in the Southern Kerala.

The role of endoscopic endonasal salvage surgery in recurrent or residual craniopharyngioma after a transcranial approach: a systematic review.

The management of craniopharyngiomas is challenging due to their high rate of recurrence following resection. Excision of recurrent tumors poses further surgical challenges due to loss of arachnoidal planes and adherence to anatomical structures. The endoscopic endonasal approach (EEA) offers a favorable alternative to transcranial approaches for primary craniopharyngiomas. However, the safety and efficacy of EEA for recurrent tumors, specifically after a prior transcranial approach, needs further investigation. We performed a systematic review using PubMed to develop a database of cases of recurrent craniopharyngiomas previously treated with a transcranial approach. Fifteen articles were included in this review with a total of 75 cases. There were 50 males and 25 females with a mean age of 38years (range 2-80). One prior transcranial surgery was done in 80.0% of cases, while 8.0% had two and 12.0% had more than two prior surgeries. Radiotherapy after transcranial resection was given in 18 cases (24.0%). Following EEA, vision improved in 60.0% of cases, and vision worsened in 8.6% of the cases. Of cases, 64.4% had pre-existing anterior hypopituitarism, and 43.8% had diabetes insipidus prior to EEA. New anterior hypopituitarism and diabetes insipidus developed in 24.6% and 21.9% of cases, respectively following EEA. Gross total resection (GTR) was achieved in 64.0%, subtotal resection in 32.0%, and partial resection in 4.0% revision EEA cases. GTR rate was higher in cases with no prior radiotherapy compared to cases with prior radiotherapy (72.0% vs 39.0%, p = 0.0372). The recurrence rate was 17.5% overall but was significantly lower at 10.0% following GTR (p = 0.0019). The average follow-up length was 41.2months (range, 1-182months). The EEA can be utilized for resection of recurrent or residual craniopharyngiomas previously managed by a transcranial approach.

Serum sodium level fluctuations following the resection of childhood-onset craniopharyngioma.

Craniopharyngiomas are low-grade malignancies (WHO I) in the sellar region. Most cases of childhood-onset craniopharyngioma are adamantinomatous craniopharyngioma, and neurosurgery is the treatment of choice. Affected patients have postoperative complications, including water and electrolyte disturbances, because these malignancies develop near the hypothalamus and pituitary gland. Determining postoperative serum sodium fluctuation patterns in these patients can reduce postoperative mortality and improve prognosis. To measure changes in serum sodium levels in pediatric patients who underwent craniopharyngioma surgery and identify influencing factors. This retrospective study measured the serum sodium levels of 202 patients aged 0-18 years who underwent craniopharyngioma resection in Beijing Tiantan Hospital and Beijing Children's Hospital and identified predictors of severe hyponatremia and hypernatremia. The mean age of the cohort was 8.35±4.35 years. The prevalence of hypernatremia, hyponatremia, and their severe forms (serum Na+ >150mmol/L and serum Na+ <130mmol/L) within 14 days after surgery was 66.3%, 72.8%, 37.1%, and 40.6%, respectively. The mean postoperative serum sodium level showed a triphasic pattern, characterized by two peaks separated by a nadir. Sodium levels peaked on days 2 (143.6±7.6mmol/L) and 14 (143.2±6.7mmol/L) and reached their lowest on day 6 (135.5±7.5mmol/L). A total of 31 (15.3%) patients met the diagnostic threshold for hyponatremia and hypernatremia of the triphase response, whereas 116 (57.4%) patients presented this pattern, regardless of met the diagnostic criteria or not. The prevalence of severe hyponatremia varied depending on preoperative endocrine hormone deficiency, tumor status (primary or recurrent), and surgical approach. Serum sodium levels after craniopharyngioma resection in children showed a triphasic pattern in most cases. The risk of postoperative hyponatremia varied depending on preoperative endocrine hormone deficiency, tumor status (primary or recurrent), and surgical approach.

Craniopharyngioma – case report and literature review

Craniopharyngiomas (Erdheim tumors) are rare tumors with low histological malignancy. Craniopharyngiomas occur at a rate of 0.5 to 2 cases per million people per year, accounting for 30 to 50% of all cases occurring in childhood and adolescence. Craniopharyngioma accounts for 1.2 to 4% of all intracranial tumors in children [1, 2, 3, 4]. Clinical manifestations usually result from increased intracranial pressure, visual disturbances and endocrine disorders. Although long-term survival is high, patients' quality of life is poor, and patients' neuropsychological functions are often impaired. Choosing an appropriate treatment method, is a major challenge and requires the cooperation of physicians from different specialties [5, 6, 7, 8, 9]. Research priorities should be efforts to make information available on cases of Craniopharyngioma, as these tumors are extremely rare. The purpose of this article is to present a case of a boy with a tumor of the periventricular region based on the analysis of medical records and to analyze the current state of knowledge about craniopharyngioma.

Effect of Resection and Surgical Experience on Survival in Patients with Craniopharyngiomas: Endoscopic Transsphenoidal Surgery in Series of 31 Cases.

To share the surgical outcomes of 31 patients who underwent endoscopic endonasal transsphenoidal surgery (EETS) at a single center. This retrospective analysis of 31 craniopharyngioma cases (2013-2022) with a minimum 6-month follow-up included demographic data, preoperative findings, postoperative resection volumes, recurrence rates, pathological diagnoses, and complications. Herein, 34 EETS surgeries were performed on 31 patients (12 males, 19 females). The presenting symptoms included visual loss (58%), hypopituitarism (54.8%), and diabetes insipidus (25.8%). Gross total resection was achieved in 87% of the patients, with 64.5% total and 22.5% near-total resection. Total resection prevented recurrences, contrasting with 75% recurrence in the subtotal resection patients (p=0.000). The primary patients showed 73.1% total resection, while only 20% of the recurrent patients achieved it (p=0.049). When comparing the first 16 cases with the last 15 cases in terms of surgical experience, the rates of resection (p=0.040) and recurrence-free survival (p=0.020) in the last 15 cases were statistically significant. Patients with preoperative visual loss demonstrated 94.4% improvement or stability postoperatively. Postoperative complications included hypopituitarism (71.4%), permanent diabetes insipidus (60.8%), worsening vision (6.5%), cerebrospinal fluid leakage (9.7%), meningitis (6.5%), and a 3.2% perioperative mortality rate. This study underscores the role of surgical resection in craniopharyngiomas, emphasizing the impact of surgical experience on recurrence-free survival. Primary surgery, with minimal complications and maximal resection, is crucial in managing recurrence challenges. Endoscopic endonasal transsphenoidal surgery, particularly in experienced centers, offers advantages such as panoramic vision and access to the third ventricle base, facilitating total and near-total resection and extending recurrence-free survival.

Endonasal endoscopic surgical treatment of craniopharyngiomas in adult patients. Our experience

Objective: to estimate the results of surgical treatment of adult patients with craniopharyngiomas (CP), who underwent endoscopic endonasal surgical treatment. Materials and methods. Treatment of 69 adult patients with CPs who underwent endonasal endoscopic surgery from 2014 to 2021 is described. Gender division: women 42% (60.9%), men 27 (39.1%). The minimum age of patients is 19 years (women 19, men 20), the maximum age is 73 years (women 73, men 66). The mean age of all patients in the group was 46.7±14.5 years, women 47.6±15.5 years, men 45.1±12.6 years. In 52 patients (75.4%) the surgical intervention was primary, and in 17 patients (24.6%) it was secondary. All patients were estimated for preoperative and postoperative endocrine and neuro-ophthalmological status in dynamics. Results. No hemorrhagic or access-associated complications have been reported. Major complications: postoperative CSF leak - 9 patients (13%), meningitis - 10 patients (14.5%), 7 out of 8 patients with CSF leak had meningitis. 7 out of 10 patients with meningitis also had CSF leak. Other complications: acute tension pneumocephalus (2 cases, 2.9%), electrolyte disturbances (45 cases, 65.2%), severe diencephalic dysfunction (SDD) (4 cases, 5.8%). The postoperative mortality rate was 3 cases (4.35%), of which 1 due to meningitis (33.3% of all deaths), due to SDD 2 cases (66.6%). The dynamic changes of visual function were as follows: improvement in 36 patients (66.7% of all patients with preoperative visual impairment), no significant change in 7 patients (13%), deterioration in 10 patients (18.5%), 4 of which were transient. 40 patients had hypopituitarism prior to surgery, 10 of whom also had diabetes insipidus. A worsening of hypopituitarism was observed in 13 patients (32.5% of all patients with preoperative hypopituitarism) and an onset of hypopituitarism in 18 patients (26.1%). The onset of diabetes insipidus in the postoperative period occurred in 23 patients (33.3%), 7 of whom with regression. Conclusions. Endonasal endoscopic approach is no longer an alternative for surgical treatment of certain forms of craniopharyngiomas, but it is a method of choice for most CP cases. The postoperative mortality is low. The quality of life of the operated patients is satisfactory due to a high percentage of improvement in visual function and preservation of endocrine function. The rates of CSF leak and purulent-septic complications correlate with the data of the world literature. Modern standards of treatment require the establishment of a multidisciplinary team of specialists in healthcare institutions to personalize the treatment process in patients with CPs.

Surgical outcomes and follow-up results of 53 pediatric craniopharyngioma cases: a single-center study.

Craniopharyngiomas are locally invasive neoplasms, and they cause potential lifelong morbidity because of their tendency for local recurrence. Despite advancements in endoscopic techniques, gross-total resection (GTR) of tumors with invasion or adhesion to important surrounding anatomical structures is extremely difficult. The authors present a single-center study that evaluated the impact of the endoscopic endonasal approach (EEA) on the surgical outcomes of pediatric craniopharyngiomas, the factors affecting the resection rate, and recurrence. A total of 44 pediatric patients (age ≤ 18 years) who were treated via the EEA for craniopharyngioma from August 1997 to June 2022, as well as their 53 operations, were included in this study. The preoperative radiological configuration and surgical data of these cases were assessed. Also, preoperative and postoperative clinical (endocrinologic, neurological, and ophthalmological), hypothalamic, physical and social development, and neurocognitive assessment data were described. In total, 37 cases (69.8%) had no history of operation beforehand. The most common symptoms at presentation were endocrine disturbances (98.1%), headache without vomiting (84.3%), and visual disturbance (51%). Cases were classified as infrasellar (1.9%), sellar (32.1%), sellar-suprasellar (52.8%), and suprasellar (13.2%) localization. GTR was achieved in 34/53 cases (64.1%). The rate of GTR was higher in infrasellar and sellar tumors compared with sellar-suprasellar and suprasellar tumors (p = 0.003), and preoperative hypothalamic involvement was associated with lower likelihood of GTR (p = 0.024). Moreover, with experience, the rate of GTR increased (p = 0.037). Postoperative complications, other than endocrine impairment, occurred in 10/53 cases (18.9%). The mean duration of follow-up was 53.57 months. At follow-up, 21/53 (39.6%) cases presented with tumor recurrence. The 5-year progression-free survival (PFS) rate was 48.5%. There was a statistically significant difference between the GTR and other-than-GTR groups in terms of PFS (p < 0.001). According to univariate analysis, smaller tumor (p = 0.017), infrasellar and sellar localization (p = 0.031), and GTR (p < 0.001) were significantly associated with decreased rate of recurrence. Also, there was a statistically significant association between the recurrence rate and adhesion strength of the tumor (p < 0.001). This retrospective cohort study revealed surgical indications for EEA, as well as factors affecting the resection rate, recurrence, and quality of life during the follow-up period of the included cases. The authors believe that GTR should be the goal for craniopharyngioma treatment, but the authors' treatment approach was to provide a balance between radical surgery with maximum safety and adjuvant treatment for long-term disease control.

Postoperative hypothalamic-pituitary dysfunction and long-term hormone replacement in patients with childhood-onset craniopharyngioma.

Hypothalamic-pituitary axis dysfunction is a common complication in post-operative craniopharyngioma(CP) patients, and it greatly impacts the long-term quality of life of such patients. To better understand the effects of postoperative hypothalamic-pituitary dysfunction and long-term hormone replacement therapy in patients with childhood CP, we assessed approximately 200 patients with childhood-onset CP postoperatively. Clinical details of patients with childhood-onset CP who underwent sellar tumor resection in Beijing Children's Hospital and Beijing Tiantan Hospital from 2018 to 2019 were retrieved retrospectively. The participants were followed up to assess the effects of post-operative long-term hormone replacement therapy and assess the tumor recurrence rate. The median age of admission was 8.1 (1.8, 14.3) years. Headache (45.5%), visual impairment (39.5%), and nausea (33.0%) were the most common clinical manifestations. ACP accounted for 95% of all CP cases. The incidence of central adrenal insufficiency and central hypothyroidism within the first week after surgery was 56.2% and 70.3%, respectively. At the same time 85.5% of the patients required at least one dose of desmopressin to control urine output. Total survival and tumor recurrence rates were 98.6% and 26.1%, respectively, with a median follow-up time of 29.7 (19.0, 40.3) months. During the follow-up period, 28.1% patients met the diagnostic criteria for short stature, while 54.4% fit the criteria for obesity. In addition, 94.4% of the patients were taking at least one kind of hormone substitution, and 74.7% were taking three or more. The prevalence of levothyroxine, glucocorticoid, desmopressin, and growth hormone replacement therapy was 87.3%, 77.5%, 78.9% and 31.0%, respectively. The proportion of patients treated with the substitutive combination of levothyroxine, hydrocortisone, and desmopressin was 54.9%. This study is a large-sample systematic postoperative endocrine function evaluation of patients with childhood-onset CP. Due to the high prevalence of post-operative hypothalamic-pituitary dysfunction, patients with CP usually require long-term multiple hormone substitution therapy. Individualized management and accurate hormone replacement dosage for postoperative childhood-onset CP patients are of great importance.

Patterns of Care in Craniopharyngioma: Clinical Outcomes After Surgery and Radiation Therapy in a Real-World Setting

Craniopharyngioma (CP) is a benign neuroepithelial tumor generally treated with maximal safe resection and radiation therapy (RT) in incompletely resected CP or in recurrent tumors to achieve long-term control. We analyzed the clinical outcomes of patients with CPs treated with a multimodality approach. A retrospective clinical audit of histologically proven CPs registered between 2008 and 2019 at a specialized neuro-oncology center in India was performed. Time-to-event outcomes (overall survival [OS] and progression-free survival [PFS]) were analyzed. One hundred and twenty-two patients with CP were analyzed. The median age of the population was 14 years (interquartile range [IQR], 8-26) with a significant male preponderance. Gross total resection was achieved in only 25% of patients. At a median follow-up of 57.1 months (IQR, 27.8-87.8), 5-year estimates of PFS and OS were 52% (95% confidence interval, 46%-63.4%) and 85.8% (95% confidence interval, 78.6%-93%), respectively. Recurrence or progression was observed in 48 of 122 patients (39.3%) at a median time of 84.4 months (IQR, 24.7-174.8). On multivariate analysis, the absence of residual disease (P= 0.004), near-total resection (P= 0.035), and use of up-front adjuvant RT (P < 0.001) significantly improved the 5-year PFS, whereas the absence of extracavernous extension (P= 0.058) and any use of postoperative RT (P= 0.026) significantly improved the 5-year OS. This study represents one of the largest single-institutional series of CPs, showing improved PFS with up-front adjuvant RT in most cases of CP. Deferring adjuvant RT should be considered only in patients with no evidence of residual disease (as shown on dedicated sellar imaging) after primary surgery.

Clinico-epidemiological profile and treatment outcome of craniopharyngioma: a case series

Craniopharyngioma is a rare type of benign (non-cancerous) brain tumor that primarily affects children and young adults. It arises from remnants of the Rathke's pouch, which is an embryonic structure that forms during the development of the pituitary gland. Craniopharyngiomas typically develop near the pituitary gland, in the area known as the sellar region. These tumors can cause various symptoms depending on their size and location, including hormonal imbalances, vision problems, headaches, and growth abnormalities. The management of craniopharyngioma involves a multidisciplinary approach, including surgery, radiation therapy, and hormone replacement therapy. Long-term outcomes can be influenced by the tumor's size, location, and the treatment modality used. Therefore, early recognition, accurate diagnosis, and appropriate treatment are crucial for optimizing the quality of life and long-term prognosis for individuals with craniopharyngioma. A detailed review of a number of craniopharyngioma cases is presented in this case series publication, with an emphasis on the clinical presentation, diagnostic methods, therapeutic options, and long-term results. This study's goal is to give doctors and researchers who work with patients who have this difficult malignancy useful information.

Cystic Craniopharyngiomas Treated by Ommaya Reservoir Implantation About 4 Cases Including 2 Cases of Intracystic Injection of Bleomycin

<i>Introduction. </i>Cystic craniopharyngiomas are the most common form of craniopharyngiomas. The treatment is still debated. The less aggressive and multidisciplinary approach is more recommended. <i>Methods. </i>We describe 4 cases of cystic craniopharyngiomas managed in our department by implantation of an Ommaya reservoir. The diagnosis was retained on imaging and in front of the appearance of the cyst puncture fluid. Intracystic chemotherapy with bleomycin was performed in 2 cases. <i>Results. </i>Patient’s age was 3, 34, 51 and 65 years. There were 2 female and 2 male patients. Clinical signs were dominated by headache (4 cases) and visual disturbances (4 cases). The Ommaya reservoir was placed with an endoscope in 2 cases. Postoperative insipid diabetes was noted in these 2 cases. A puncture of the Ommaya reservoir was performed on average every 3 weeks (2 cases) in the absence of Bleomycin. Infection was noted in these 2 cases. The evolution was favorable in 3 cases including the 2 cases of Bleomycin injection. <i>Conclusion. </i>Ommaya reservoir implantation was a satisfactory alternative to surgical excision of craniopharyngiomas. The use of the endoscope increased the risk of developing insipid diabetes. Close punctures of the reservoir increased the risk of infection. Intra-cystic Bleomycin stopped the punctures of the reservoir.

Long-Term Endocrinologic Follow-Up of Children with Brain Tumors and Comparison of Growth Hormone Therapy Outcomes: A SingleCenter Experience.

Brain tumors in childhood carry a high risk for endocrine disorders due to the direct effects of the tumor and/or surgery and radiotherapy. Somatotropes are vulnerable to pressure and radiotherapy; therefore, growth hormone deficiency is one of the most frequent abnormalities. This study aimed to evaluate endocrine disorders and recombinant growth hormone treatment outcomes in brain tumor survivors. In this study, 65 (27 female) patients were classified into 3 groups as craniopharyngioma (n = 29), medulloblastoma (n = 17), and others (n = 19). "Others" group included astrocytoma, ependymoma, germinoma, pineoblastoma, and meningioma patients. Anthropometric data and endocrine parameters of patients and their growth outcome with/without recombinant growth hormone therapy were collected from medical records, retrospectively. Mean age at the first endocrinological evaluation was 8.7 ± 3.6 years (range: 1.0- 17.1 years). Height, weight, and body mass index standard deviation score, mean ± standard deviation (median) values were -1.7 ± 1.7 (-1.5), -0.8 ± 1.9 (-0.8), and 0.2 ± 1.5 (0.4), respectively. Hypothyroidism (central 86.9%, primary 13.1%) was detected during follow-up in 81.5% of patients. Primary hypothyroidism in medulloblastoma (29.4%) was significantly higher compared to other groups (P = .002). The frequency of hypogonadotropic hypogonadism, central adrenal insufficiency, and diabetes insipidus was significantly high in the craniopharyngioma cases. In our study, endocrine disorders other than growth hormone deficiency were also frequently observed. In craniopharyngioma cases, the response to recombinant growth hormone therapy was satisfactory. However, there was no improvement in height prognosis during recombinant growth hormone therapy in medulloblastoma patients. A multidisciplinary approach to the care of these patients, referral for endocrine complications, and guidelines on when recombinant growth hormone therapy is required.