Craniopharyngioma - Childhood


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#MountSinaiLive: In honor of Brain Tumor Day, Peter Morgenstern, MD joins us to talk about what you need to know about brain tumors in children.
Dr. Peter Morgenstern is a board-certified neurosurgeon who specializes in pediatric neurosurgery. He has extensive training in the comprehensive care of children with brain tumors, hydrocephalus, spinal and cranial malformations including craniosynostosis and spina bifida, Chiari malformation, and other common pediatric neurosurgical conditions. He is particularly interested in applying minimally invasive techniques, such as neuroendoscopy, to pediatric neurosurgery. Throughout his career, Dr. Morgenstern has been praised for his thoughtful and level-headed approach to complex problems, and has been recognized for the excellence and compassion with which he cares for his patients. He is dedicated to guiding patients and their families through the difficult journey from diagnosis through treatment and follow-up.
Dr. Morgenstern has published numerous peer-reviewed articles and has won several grants and awards for his research, including the Kenneth Schulman Award from the AANS/CNS Joint Section on Pediatric Neurosurgery, and the Christopher Gaposchkin ’99 Research Prize from the Department of Neurological Surgery at Weill Cornell. His previous research has been funded by the Neurosurgery Research and Education Foundation and the Voices Against Brain Cancer Foundation. His current research focuses on pediatric brain tumors, clinical outcomes in pediatric neurosurgery and neuroendoscopy.
In his spare time Dr. Morgenstern enjoys spending time with his family, cycling, running, cooking, and playing music.
Request an appointment with Dr. Morgenstern: https://mshs.co/3My251o


16-year-old male with bitemporal hemianopsia and chronic headaches. There is circumscribed T1-hypointense, T2-hyperintense mass centered within the sellar/suprasellar region. The lesion demonstrates T2/FLAIR-hyperintense signal, facilitated diffusion, and a thin peripheral rim of postcontrast enhancement. There are lobular cystic components which extend into the enlarged sella turcica. This is a case of an adamantinomatous craniopharyngioma. Craniopharyngiomas occur with a bimodal distribution. The first peak occurs between 10 and 14 years of age while the second occurs in middle-aged adutls. Pediatric cases are mostly composed of the adamantinomatous subtype while the papillary subtype comprises the majority of cases in middle-aged adults. Cases occur in males and females about equally. Differential includes Rathke cleft cyst, cystic pituitary adenoma, epidermoid and intracranial teratoma.
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Endoscopic Endonasal Craniopharyngioma surgery is performed for the majority of these tumors. This is most ideal when the tumors are behind the optic chiasm (nerves from the eyes). In this video, Dr. Daniel Kelly, director of the Pacific Pituitary Disorders Center at Providence Saint John's Health Center demonstrates the endoscopic surgical removal of a craniopharyngioma while preserving the normal gland and blood vessels. This allows for a better chance of vision preservation as well as pituitary gland function. This patient did well with good pituitary gland function and vision after surgery.
https://www.pacificneuroscienc....einstitute.org/pitui
310-582-7450


12-year-old male with chronic headaches and new onset diabetes insipidus. There is a circumscribed centrally cystic lesion arising from the anterior surface of the infindibulum. The central cystic component demonstrates signal intensity slightly greater than the surrounding CSF space on the T1-weighted image. There is FLAIR hemorrhage/proteinaceous fluid centrally. The high-resolution fluid sensitive sequences demonstrate a lesion with a thin margin abutting the posterior and inferior aspect of the optic chiasm with slight displacement and deformity. The lesion demonstrates a thin enhancing margin. The general differential includes adamantinomatous craniopharyngioma, Rathke’s cleft cyst, and infindibular cyst. This is an adamantinomatous craniopharyngioma. Craniopharyngioma are though to arise via two mechanisms: as remnants of the craniopharyngeal duct, while the second is from squamous epithelial cells in the pars tuberalis of the adenohypophysis. 75% of the adamantinomatous subtype tend to be in the suprasellar compartment. NMR182
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This was presented at Neuroendocon 2021.
Though complete resection of craniopharyngioma is desirable
for lower recurrence rates, it is hindered by functional
preservation. Currently, a trend toward less radical surgical
approaches is observed. In a multicenter study of children and adolescents with craniopharyngioma, KRANIOPHARYNGEOM
2007, it was observed that the realized treatment was more
radical (p = 0.01) in patients recruited between 2001 and 2007
(38%) when compared with patients treated between 2007 and
2012 (18%).
Cystic craniopharyngiomas
present a unique challenge because of the possibility
of recurrence of cyst irrespective of the modality of initial
treatment. Almost 90% of craniopharyngiomas have a cystic
component.3 The presence of a cyst in craniopharyngioma
can account for major total bulk of the tumor, and a predominantly
cystic tumor is seen in 60% of craniopharyngiomas. The treatment of cystic craniopharyngioma in children is varied. The treatment ranges from radical excision to direct radiotherapy. As the morbidity of excision is high, more conservative approaches are used.
The traditional approach to cystic craniopharyngioma is a
variable extent of microsurgical resection. Ommaya reservoir
is often placed in the residual cyst for repeated aspiration or
intracavitary therapy. However, the malposition of reservoir
is not uncommon. In such circumstances, a neuroendoscopic
approach provides a less invasive and more accurate technique
to deal with cystic craniopharyngiomas. Since 2013, we follow
the endoscopic transventricular approach for intra- and
paraventricular cystic craniopharyngiomas.
Transventricular endoscopy is a minimally invasive treatment for cystic craniopharyngiomas. Endoscopic transventricular approach is a safe alternative for initial treatment of suprasellar cystic craniopharyngioma in children.
Konar S. Kulkarni AV, Shukla D, Mishra T, Devi BI, Peer S, Lanka V. Management options for suprasellar cystic craniopharyngioma: Endoscopic Transventricular Approach and Microsurgical Approach. J Neurosci Rural Pract 2021;12:121–127. DOI https://doi.org/10.1055/s-0041-1722839