Malignant Transformation of Craniopharyngioma after Growth Hormone Replacement Therapy

Yasuhiro Takeshima,Yasushi Motoyama,Kentaro Tamura,Young-Soo Park,Miho Kakutani,Shuichi Yamada,Hiroyuki Nakase,Fumihiko Nishimura,Ryosuke Matsuda,Ichiro Nakagawa,Yoshiaki Takamura

doi:10.31487/j.crss.2020.01.04

Abstract

Background: We present a rare case of malignant transformation of a craniopharyngioma in a patient who received growth hormone replacement therapy. Case Description: A 43-year-old male came to our department with a headache and visual disturbance and was admitted. Brain magnetic resonance imaging (MRI) revealed a giant tumor in the intra- and suprasellar region compressing the optic chiasma. Using a transcranial pterional approach, the tumor was removed as much as possible, with the residual mass removed later with a trans-sphenoidal approach. The initial histopathological diagnosis was adamantinomatous craniopharyngioma. Decompression of the optic chiasma was achieved and visual function improved, though residual tumor tissue remained in the parasellar region, without regrowth seen for more than 24 years. At the age of 67 years, the patient received growth hormone replacement therapy because of a deficiency, then 2 years later the residual tumor began to grow gradually. Trans-sphenoidal surgery was again performed to remove the growing tumor and histopathology results revealed an adamantinomatous craniopharyngioma with malignant transformation. Stereotactic radiation therapy was performed for the residual mass with a total dose of 60 Gy. Presently, 1 year later the patient has a stable condition, and the tumor remains controlled, while follow-up examinations are continuing. Conclusion: A craniopharyngioma may undergo malignant transformation with continuous exposure to administered growth hormone. Close follow-up and imaging examinations are necessary to detect a change, including tumor growth in such cases.

Highlights

A craniopharyngioma, which originates from Rathke’s pouch, a derivative of the oral ectoderm, is a benign tumor that accounts for approximately 3-3.5% of all intracranial tumors [1]
Malignant transformation of a craniopharyngioma rarely occurs, and the etiology and pathogenesis of those cases are unclear, though a previous study suggested a correlation with radiotherapy [3]
We present a case of craniopharyngioma with a subsequent malignant transformation that occurred 2 years after growth hormone (GH) replacement therapy without radiotherapy

Summary

Introduction

A craniopharyngioma, which originates from Rathke’s pouch, a derivative of the oral ectoderm, is a benign tumor that accounts for approximately 3-3.5% of all intracranial tumors [1]. Malignant transformation of a craniopharyngioma rarely occurs, and the etiology and pathogenesis of those cases are unclear, though a previous study suggested a correlation with radiotherapy [3]. There are no known reports of a case with malignant transformation associated with growth hormone (GH) replacement therapy. We present a case of craniopharyngioma with a subsequent malignant transformation that occurred 2 years after GH replacement therapy without radiotherapy. Decompression of the optic chiasma was achieved and visual function improved, though residual tumor tissue remained around the parasellar region (Figure 2). Craniopharyngioma with cytologically malignant features (Figure 5A). The tumor cells showed significant nuclear pleomorphism, hyperchromasia, and a high nuclear-cytoplasmic ratio, indicating malignant transformation (Figure 5B). To prevent growth of the residual tumor, adjuvant stereotactic radiotherapy was performed with a total dose of 60 Gy in 30 fractions (Figure 6).

Discussion

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Conclusion