Craniopharyngiomas and pituitary adenomas are two types of brain tumors. Learn about their similarities and differences.
Craniopharyngiomas are rare, slow-growing tumours located in the sellar region of the brain with a worldwide incidence rate of 1.34 per million. Here, a case is discussed that adds to the literature ...
Craniopharyngiomas (CPs) are rare primary brain epithelial tumors arising in the suprasellar region from remnants of Rathke's pouch. About 50% originate at the level of the third ventricle floor, inc...
CRANIOPHARYNGIOMA. HISTORY. CRANIOPHARYNGIOMA Mclean in 1930, Frazier and Alpes in 1931 Cushing in 1932 . CUSHING “ MOST FORBIDDING OF THE INTRACRANIAL TUMORS ” RUTKA “ THERE IS PERHAPS NO OTHER BRAIN TUMOR THAT EVOKES MORE PASSION, EMOTION, AND CONTROVERSY AS CRANIOPHARYNGIOMA ”.
Introduction Craniopharyngiomas are one of the most frequently diagnosed hypothalamo-pituitary tumors in childhood. The adamantinomatous histological subtype accounts for most pediatric cases, while the papillary variant is almost exclusively diagnosed in adults. Here, we report a case of papillary craniopharyngioma in a very young child, confirmed by molecular tissue analysis. Case report A 4-year-old girl was being investigated for symptomatic central hypothyroidism. Brain MR imaging revealed a large solid/cystic suprasellar mass, splaying the optic chiasm and measuring 3 × 1.9 × 2.3 cm. The patient underwent a transsphenoidal near total resection of the lesion, which was encased within a tumor capsule. Post-operatively, the patient developed transient diabetes insipidus but otherwise recovered well. The pathology of the lesion was consistent with a papillary craniopharyngioma with regions of stratified squamous epithelium accompanied by superficial goblet cells and ciliated cells. Subsequent next-generation sequencing analysis of the lesion confirmed the presence of a BRAF V600E mutation (BRAFc.1799T>A p. (Val600Glu). To date, she remains free from progression 1 year following surgery. Conclusion This is the youngest case published to date of papillary craniopharyngioma with a confirmed BRAF V600E mutation. The case encourages discussion about the most appropriate adjuvant therapy for tumor progression in such cases, given the risks of radiotherapy to the developing brain and the increasing availability of oral BRAF inhibitor therapy.
Luiz Augusto Marcio Marques, also known as Guto, is a man from Passo Fundo, Brazil who has faced many challenges in his life. At a young age, Guto started experiencing intense headaches that affected his daily life and schooling. After numerous misdiagnoses, he was finally diagnosed with a rare brain tumor called craniopharyngioma. Guto underwent surgery to remove part of the tumor, but it resulted in his growth being stunted. Even though he faced difficulties and struggled with acceptance, Guto is now grateful to be alive after multiple surgeries and chemotherapy. He still has a small tumor, but his headaches have disappeared.
Even though ACP is a benign tumor, treatment is challenging because of the tumor's eloquent location. Today, with the exception of surgical intervention and irradiation, further treatment options are...
Pediatric adamantinomatous craniopharyngiomas (ACPs) are histologically benign brain tumors that often follow an aggressive clinical course. Arising in the sellar/suprasellar region, they grow in close proximity to critical neurological and vascular structures and can result in significant neuroendocrine morbidity. First-line treatment often involves surgical resection with or without radiotherapy and has been associated with significant morbidity and poor quality of life outcomes. As a result, the discovery of alternative effective and safe treatments is clearly desirable. In recent years, laboratory studies have harnessed sophisticated techniques to identify the upregulation of several markers that may represent potential therapeutic targets. These targets include IL-6, PD1/PD-L1, MEK, IDO-1, and others. Agents that target these pathways exist, and there is an opportunity to investigate their potential efficacy in the treatment of ACP. Trials investigating some of these agents as monotherapy and in combination for the treatment of pediatric ACP are underway or in development. If positive, these trials may result in a paradigm shift in treatment that will hopefully result in reduced morbidity and better outcomes for patients.
Features are consistent with an adamantinomatous craniopharyngioma, which was proven histologically at resection.
OBJECTIVE Craniopharyngiomas with a predominant cystic component are often seen in children and can be treated with an Ommaya reservoir for aspiration and/or intracystic therapy. In some cases, cannulation of the cyst can be challenging via a stereotactic or transventricular endoscopic approach due to its size and proximity to critical structures. In such cases, a novel placement technique for Ommaya reservoirs via a lateral supraorbital incision and supraorbital minicraniotomy has been used. METHODS The authors conducted a retrospective chart review of all children undergoing supraorbital Ommaya reservoir insertion from January 1, 2000, to December 31, 2022, at the Hospital for Sick Children, Toronto. The technique involves a lateral supraorbital incision and a 3 × 4–cm supraorbital craniotomy, with identification and fenestration of the cyst under the microscope and insertion of the catheter. The authors assessed baseline characteristics and clinical parameters of surgical treatment and outcome. Descriptive statistics were conducted. A review of the literature was performed to identify other studies describing a similar placement technique. RESULTS A total of 5 patients with cystic craniopharyngioma were included (3 male, 60%) with a mean age of 10.20 ± 5.72 years. The mean preoperative cyst size was 11.6 ± 3.7 cm3, and none of the patients suffered from hydrocephalus. All patients suffered from temporary postoperative diabetes insipidus, but no new permanent endocrine deficits were caused by the surgery. Cosmetic results were satisfactory. CONCLUSIONS This is the first report of lateral supraorbital minicraniotomy for Ommaya reservoir placement. This is an effective and safe approach in patients with cystic craniopharyngiomas, which cause local mass effect but are not amenable to traditional Ommaya reservoir placement stereotactically or endoscopically.
Obesity is a common complication after craniopharyngioma therapy, occurring in up to 75% of survivors. Its weight gain is unlike that of normal obesity, in t...
Intraventricular craniopharyngiomas are rare but certainly reported. Like this case, they are usually located in the third ventricle. Editors note: Unfortunately additional diagnostic informat...
Even though ACP is a benign tumor, treatment is challenging because of the tumor's eloquent location. Today, with the exception of surgical intervention and irradiation, further treatment options are...
Pediatric adamantinomatous craniopharyngiomas (ACPs) are histologically benign brain tumors that often follow an aggressive clinical course. Arising in the sellar/suprasellar region, they grow in close proximity to critical neurological and vascular structures and can result in significant neuroendocrine morbidity. First-line treatment often involves surgical resection with or without radiotherapy and has been associated with significant morbidity and poor quality of life outcomes. As a result, the discovery of alternative effective and safe treatments is clearly desirable. In recent years, laboratory studies have harnessed sophisticated techniques to identify the upregulation of several markers that may represent potential therapeutic targets. These targets include IL-6, PD1/PD-L1, MEK, IDO-1, and others. Agents that target these pathways exist, and there is an opportunity to investigate their potential efficacy in the treatment of ACP. Trials investigating some of these agents as monotherapy and in combination for the treatment of pediatric ACP are underway or in development. If positive, these trials may result in a paradigm shift in treatment that will hopefully result in reduced morbidity and better outcomes for patients.
This illustration was produced for the Johns Hopkins University’s Suburban Hospital Magazine, New Directions, Fall 2012 issue. A craniopharyngioma is a benign tumor that develops near the pituitary gland (a small endocrine gland at the base of the brain). This tumor most commonly affects children 5 – 10 years of age. Adults can sometimes be […]
OCEAN CITY – Amy Wood remembers having an almost out-of-body experience the moment her 4- year-old son’s pediatrician uttered the words that changed her
Ray Rice was diagnosed with a low grade (non-cancerous) Craniopharyngioma in 1998. He was just 12 years old when he was told he would need brain surgery
Craniopharyngiomas are complex and challenging tumors of the central nervous system (CNS) which require multidisciplinary and tailored management plans. An extensive variety of clinical presentations, treatment options, and patient outcomes exists. It is crucial to accurately and effectively distinguish the clinical status and the patient’s specific roadmap to ensure proper disease status identification, patient-tailored care, follow-up, doctor-doctor and doctor-patient communication, and research. The author proposes the four-tier STAR/HOP craniopharyngioma clinical coding system to facilitate case definitions, adequate reporting, and sound communication among neurosurgeons and other healthcare professionals. The new system utilizes symbols and conventions encompassing simple numerical tentative rules and relevant clinical concerning the tumor surgeries, adjuvant therapies, shunt systems, and access Ommaya devices with flexibility in giving extra information of shunt and Ommaya functionality, timing of radiation therapy, and different grades of tumor excision and recurrences as well. The HOP component provides a broad, yet simple description of hypothalamic, ophthalmological, and pituitary functions which are crucial to assess in each craniopharyngioma patient. Such a system could represent a new template for new clinical coding systems for brain tumors in general, and craniopharyngiomas in particular, and may eventually affect the World Health Organization (WHO) classification of brain tumors at some point. The system is easy to grasp, clinically practical, simple, and easy to communicate.
While classified as benign, craniopharyngiomas pose a great challenge. Find out why these pituitary tumors are "the most formidable of intracranial tumors."
Features are consistent with an adamantinomatous craniopharyngioma, which was proven histologically at resection.
Jacob Barnes, of Ohio, stopped growing at age 12 - staying at 5'2
Clinical Endocrinology is a journal publishing papers dealing with the clinical aspects of endocrinology and molecular endocrinology.
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YACOLT — Macey Bodily is a sweet, easy-going 7-year-old who loves to swim in the family pool and get manicures.
Adamantinomatous craniopharyngiomas (adaCP) cause hypothalamic pituitary dysfunction. Elucidation of pathomechanisms underlying tumor progression is essential for the development of targeted chemothe...
Open access peer-reviewed chapter
Dehaut overcame brain surgery to give back to wrestling.
INTRODUCTION: Activating mutations in exon 3 of the β-catenin gene are involved in the...
Open access peer-reviewed chapter
Features are consistent with an adamantinomatous craniopharyngioma, which was proven histologically at resection.
Clinical Endocrinology is a journal publishing papers dealing with the clinical aspects of endocrinology and molecular endocrinology.
