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. 2016 Sep;3(3):173-187.
doi: 10.1093/nop/npv029. Epub 2015 Aug 30.

Multi-modality management of craniopharyngioma: a review of various treatments and their outcomes

John Varlotto  1 , Christopher DiMaio  1 , Clemens Grassberger  1 , Matthew Tangel  1 , Heath Mackley  1 , Matt Pavelic  1 , Charles Specht  1 , Steven Sogge  1 , Dan Nguyen  1 , Michael Glantz  1 , Cheng Saw  1 , Urvashi Upadhyay  1 , Richard Moser  1 , Shakeeb Yunus  1 , Paul Rava  1 , Thomas Fitzgerald  1 , Jonathan Glanzman  1 , Jonas Sheehan  1
Affiliations

Multi-modality management of craniopharyngioma: a review of various treatments and their outcomes

John Varlotto et al. Neurooncol Pract. 2016 Sep.

Abstract

Craniopharyngioma is a rare tumor that is expected to occur in ∼400 patients/year in the United States. While surgical resection is considered to be the primary treatment when a patient presents with a craniopharyngioma, only 30% of such tumors present in locations that permit complete resection. Radiotherapy has been used as both primary and adjuvant therapy in the treatment of craniopharyngiomas for over 50 years. Modern radiotherapeutic techniques, via the use of CT-based treatment planning and MRI fusion, have permitted tighter treatment volumes that allow for better tumor control while limiting complications. Modern radiotherapeutic series have shown high control rates with lower doses than traditionally used in the two-dimensional treatment era. Intracavitary radiotherapy with radio-isotopes and stereotactic radiosurgery may have a role in the treatment of recurrent cystic and solid recurrences, respectively. Recently, due to the exclusive expression of the Beta-catenin clonal mutations and the exclusive expression of BRAF V600E clonal mutations in the overwhelming majority of adamantinomatous and papillary tumors respectively, it is felt that inhibitors of each pathway may play a role in the future treatment of these rare tumors.

Keywords: craniopharyngioma; proton therapy; radiosurgery; review; surgery.

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Figures

Fig. 1.
Fig. 1.
(A) Craniopharyngioma, adamantinomatous type. This lesion is composed of squamous epithelium with peripheral palisading. Wet keratin (arrows) and stellate reticulum (star) are characteristic. Hematoxylin and eosin (H&E) stain, 200x. (B) Craniopharyngioma, papillary type. This lesion is composed of squamous epithelium (arrow) that lacks peripheral palisading, stellate reticulum or wet keratin. H&E stain, 200x.
Fig. 2.
Fig. 2.
Treatment of a patient with a craniopharyngioma with traditional opposed lateral fields and low-energy photon beam (6 MV). The tumor area was treated with a 2 cm margin. A large volume of neural tissue was treated to the 105% isodose line (orange areas) or higher. Axial, sagittal, and coronal views of the isodoses as well as a portal image view are demonstrated.
Fig. 3.
Fig. 3.
(A) Treatment of a patient with a craniopharyngioma using intensity-modulated radiation therapy with multiple non-opposed fields. Axial, sagittal, and coronal views of the isodoses as well as a room's eye view are demonstrated. (B) Treatment of a patient with a craniopharyngioma with volumetric arc therapy. Axial, sagittal, and coronal views of the isodoses as well as a room's eye view are demonstrated.
Fig. 4.
Fig. 4.
(A–C) Example of 3D-conformal proton therapy plan (A) using lateral (B), posterior and superior oblique (C) beams.
Fig. 5.
Fig. 5.
Flow diagram of treatment.
See this image and copyright information in PMC

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