Medication Summary

Agents/modalities used in the treatment of craniopharyngiomas include (1) radiation therapy applied as proton beam radiation or external fractionated radiation, stereotactic radiation, or brachytherapy (intracavitary irradiation),^{[31, 32, 33, 34, 35]} (2) bleomycin for local intracystic chemotherapy,^{[36, 37, 38]} and (3) possible new targeted treatments that arise from the vast ongoing molecular research

Class Summary

In combination with other drugs, chemotherapeutic agents are used frequently and systemically against epithelial tumors. In the early 1970s, bleomycin was found to have encouraging results in controlling craniopharyngioma tissue in cultures. Intracavitary bleomycin reduces cyst size and toughens and thickens the cyst wall, thereby facilitating surgical excision of a cyst membrane that otherwise might fragment at the time of open craniotomy. However, reports of intracystic bleomycin use are limited. Other agents like interferon alpha are being tested.

Class Summary

Radiation creates free oxygen ions that damage cellular DNA. Cellular ability to repair DNA is lower for tumor cells than normal cells and subsequently, with each mitosis, a higher cumulative effect in tumor cells results in apoptosis. Several radiation modalities are being used for the treatment of craniopharyngioma, including proton beam treatment, external fractionated radiotherapy, stereotactic raditherapy, and more.

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Media Gallery

The adamantinomatous craniopharyngioma is a histologically complex epithelial lesion with several very distinctive morphologic features (hematoxylin-eosin, x40).
Adamantinomatous craniopharyngiomas. Peripheral palisading of the epithelium is a pronounced feature (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Frequently, the inner epithelium beneath the superficial palisade undergoes hydropic vacuolization and is referred to as the stellate reticulum (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Another distinctive feature of the adamantinomatous variant is scattered nodules of keratin. These nodules are referred to as "wet" keratin because of the plump appearance of the keratinocytes; this is in contrast to the flat, flaky keratin seen in epidermoid and dermoid cysts (hematoxylin-eosin, x100).
Adamantinomatous craniopharyngiomas. Nodules of "wet" keratin frequently calcify; in aggregate, this calcification often can be detected on CT scans and is a recognized radiologic feature of craniopharyngiomas (hematoxylin-eosin, x100).
Papillary craniopharyngioma. In contrast to the adamantinomatous variant, papillary craniopharyngiomas do not show complex heterogeneous architecture but rather are composed of simple squamous epithelium and fibrovascular islands of connective tissue (hematoxylin-eosin, x40).
Papillary craniopharyngiomas. Under high power, only simple squamous epithelium is seen in a papillary craniopharyngioma. The distinctive peripheral nuclear palisading, internal stellate reticulum, and nodules of "wet" keratin, which typify the adamantinomatous variant, are not seen in the papillary variant (hematoxylin-eosin, x100).
Rosenthal fibers in neuropils surrounding a craniopharyngioma. The brain parenchyma that surrounds both variants of craniopharyngioma is typically gliotic and often shows profuse numbers of eosinophilic Rosenthal fibers. The latter structures are composed of densely compacted bundles of glial filaments and typically are seen in astrocytic cell processes of neuropils that have been subjected to chronic compression from slowly expanding mass lesions. Rosenthal fibers are a characteristic feature of juvenile pilocytic astrocytomas (JPAs), which also may arise in the suprasellar/third ventricular region. Hence, a biopsy that samples only the surrounding neuropil of a craniopharyngioma may yield an erroneous diagnosis of JPA if the pathologist is unaware of the close association of craniopharyngioma with Rosenthal fiber formation (hematoxylin-eosin, x100).
T1-weighted MRI with gadolinium in sagittal (A) and coronal (B) views demonstrates the cystic nature of a craniopharyngioma. The calcified component is evident on axial CT imaging (C).
T1-weighted MRI with gadolinium reveals a large cystic craniopharyngioma in sagittal (A), axial (B), and coronal (C) views. There is associated elevation of the optic apparatus and displacement of the pituitary stalk.
Coronal views of T1-weighted MRI for a patient with craniopharyngioma before gross total resection (A) and at postoperative follow-up evaluation (B). There was no sign of tumor recurrence, and the patient was neurologically and endocrinologically intact.

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Author

George I Jallo, MD Professor of Neurosurgery, Pediatrics, and Oncology, Director, Clinical Pediatric Neurosurgery, Department of Neurosurgery, Johns Hopkins University School of Medicine

George I Jallo, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Medical Association, American Society of Pediatric Neurosurgeons

Disclosure: Nothing to disclose.

Coauthor(s)

Franco DeMonte, MD, FRCSC, FACS Professor of Neurosurgery, Mary Beth Pawelek Chair in Neurosurgery, The University of Texas MD Anderson Cancer Center

Franco DeMonte, MD, FRCSC, FACS is a member of the following medical societies: American Academy of Neurological Surgery, American College of Surgeons, North American Skull Base Society, Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

Gregory N Fuller, MD, PhD Professor of Pathology, Chief, Section of Neuropathology, Department of Pathology, Division of Pathology and Laboratory Medicine, The University of Texas MD Anderson Cancer Center

Gregory N Fuller, MD, PhD is a member of the following medical societies: American Association of Neuropathologists, College of American Pathologists, International Academy of Pathology, Society for Neuro-Oncology, United States and Canadian Academy of Pathology

Disclosure: Nothing to disclose.

Morris D Groves, MD, JD Associate Professor, Department of Neuro-Oncology, Division of Cancer Medicine, Associate Clinic Director for the Brain and Spine Center, The University of Texas MD Anderson Cancer Center

Morris D Groves, MD, JD is a member of the following medical societies: American Academy of Neurology, American Bar Association, American Medical Association, European Association of Neuro-Oncology, Harris County Medical Society, Houston Neurological Society, Society for Neuro-Oncology, Texas Medical Association, Texas Neurological Society

Disclosure: Received grant/research funds from Genentech for other; Received honoraria from Genentech for consulting; Received grant/research funds from GlaxoSmithKline for other; Received grant/research funds from AngioChem for other; Received grant/research funds from Pfizer/Celldex Therapeautics for other.

Meleine M Martinez-Sosa, MD Resident Physician, Department of Neurosurgery and Brain Repair, University of South Florida Morsani College of Medicine

Disclosure: Nothing to disclose.

Nir Shimony, MD Assistant Professor of Neurological Surgery, Division of Pediatric Neurosurgery, Department of Surgery, St Jude Children’s Research Hospital, Le Bonheur Neuroscience Institute, Le Bonheur Children’s Hospital, Baptist Children’s Hospital, and Semmes Murphey Clinic, University of Tennessee Health Science Center College of Medicine; Adjunct Faculty, Department of Neurosurgery, Johns Hopkins University School of Medicine

Nir Shimony, MD is a member of the following medical societies: American Association of Neurological Surgeons, American Epilepsy Society, Congress of Neurological Surgeons, International Society of Pediatric Neurosurgery, Society for Neuro-Oncology

Disclosure: Nothing to disclose.

Chief Editor

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA Professor of Pediatrics, Neurology, Neurosurgery, and Psychiatry, Medical Director, Tulane Center for Autism and Related Disorders, Tulane University School of Medicine; Pediatric Neurologist and Epileptologist, Ochsner Hospital for Children; Professor of Neurology, Louisiana State University School of Medicine

Stephen L Nelson, Jr, MD, PhD, FAACPDM, FAAN, FAAP, FANA is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Neurology, American Academy of Pediatrics, American Epilepsy Society, American Medical Association, American Neurological Association, The Society of Federal Health Professionals (AMSUS), Child Neurology Society, Southern Pediatric Neurology Society

Disclosure: Nothing to disclose.

Additional Contributors

George C Bobustuc, MD Consulting Staff, Department of Neuro-oncology, MD Anderson Cancer Center of Orlando

George C Bobustuc, MD is a member of the following medical societies: American Academy of Neurology, Texas Medical Association, Society for Neuro-Oncology, American Medical Association

Disclosure: Nothing to disclose.

Lee S Hwang University of Texas Southwestern Medical School

Disclosure: Nothing to disclose.

Acknowledgements

Jorge Kattah, MD Head, Program Director, Professor, Department of Neurology, University of Illinois College of Medicine at Peoria

Jorge Kattah, MD is a member of the following medical societies: American Academy of Neurology, American Neurological Association, and New York Academy of Sciences

Disclosure: Biogen Honoraria Consulting; Bayer Corporation Honoraria Consulting

Amy A Pruitt, MD Associate Professor of Neurology, University of Pennsylvania; Attending Neurologist, Hospital of the University of Pennsylvania

Amy A Pruitt, MD is a member of the following medical societies: American Academy of Neurology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Reference Salary Employment