J Korean Neurosurg Soc.  2017 Jan;60(1):108-113. 10.3340/jkns.2015.0707.022.

Malignant Transformation of Craniopharyngioma without Radiation Therapy: Case Report and Review of the Literature

• Jeong TS
• Yee GT
• Kim NR

• KMID: 2365596
• DOI: http://doi.org/10.3340/jkns.2015.0707.022

Abstract

Craniopharyngiomas exhibiting histologic malignancy are extremely rare. Herein, we report the case of a 26-year-old male patient who underwent suprasellar mass excision via an interhemispheric transcallosal approach. Histopathological examination indicated that the craniopharyngioma was of the adamantinomatous subtype. The patient received postoperative medical treatment for endocrine dysfunction and diabetes mellitus without radiation treatment. Two years after the operation, he presented with progressive visual disturbance and altered mentality. Magnetic resonance imaging revealed a huge mass in the suprasellar cistern and third ventricle. He underwent a second operation via the same approach. The histopathological examination showed an adamantinomatous craniopharyngioma with sheets of solid proliferation in a spindled pattern, indicating malignant transformation. Malignant transformation of craniopharyngioma in the absence of radiation therapy has been reported in only five cases, including this one. We present a case of malignant transformation of craniopharyngioma with a brief review of relevant literature.

Keyword

Craniopharyngioma; Malignant transformation; Radiotherapy

MeSH Terms

Adult
Craniopharyngioma*
Diabetes Mellitus
Humans
Magnetic Resonance Imaging
Male
Radiotherapy
Third Ventricle

Figure

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  Fig. 1 Brain magnetic resonance imaging before the first surgery, showing a suprasellar mass lesion with enhanced solid and multiseptated cystic components. A : Axial. B : Coronal. C : Sagittal.

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  Fig. 2 The first biopsy showing a typical adamantinomatous craniopharyngioma.

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  Fig. 3 Post-operative magnetic resonance imaging scan obtained 6 months after the first surgery, showing a small residual enhancing mass along the right anterior margin of the floor of the third ventricle. A : Axial. B : Coronal. C : Sagittal.

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  Fig. 4 Follow-up brain magnetic resonance imaging 2 years after the first surgery, showing recurrence of multiseptated huge cystic mass in the suprasellar, third, and lateral ventricles. A : Axial. B : Coronal. C : Sagittal.

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  Fig. 5 Histopathologic findings of a specimen obtained during the second surgery. Typical adamantinomatous craniopharyngioma that is highly cellular and presents with a spindled pattern (A, B; hematoxylin-eosin). The immunological tests showing overexpression of p53 (C) and Ki67 (D), and positive results for pancytokeratin (E) and vimentin (F).

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  Fig. 6 Brain magnetic resonance imaging after the second surgery showing total resection of the recurrent mass. A : Axial. B : Coronal. C : Sagittal.

Reference

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