Oral and Maxillofacial Surgery Cases (Sep 2020)
Craniopharyngioma with malignant transformation: A case report
Abstract
Introduction: Craniopharyngiomas are usually slow growing, benign epithelial tumors, arising from cell remnants of Rathke's pouch with its ultimate site of origin being the embryologic stomadeum. Although considered WHO grade I tumors, they are often locally aggressive. However, malignant transformation is extremely rare. Until now, 42 cases of malignant craniopharyngioma have been reported. In this article, the interdisciplinary, i.e. neurosurgical and maxillofacial, approach to a case of secondary malignant craniopharyngioma with multiple recurrences is discussed.Case report A 73-year-old woman with a history of recurrent, extracranially expanding craniopharyngioma was referred to the department of Neurosurgery following the MRI finding of a 7th tumor recurrence, located at the level of the right lateral orbital wall. Previously, the patient had already undergone five surgical resections and three radiotherapeutic treatments elsewhere. Histopathological examination revealed an adamantinomatous craniopharyngioma with sites of malignant transformation. Tumor resection with exenteration of the right eye was performed with an immediate reconstruction using a PEEK-PSI (Patient Specific Implant) and anterolateral thigh flap. Histopathological examination confirmed the diagnosis of craniopharyngioma with malignant transformation. Conclusions: Malignant transformation in a craniopharyngioma is uncommon but has a poor prognosis. The assumed correlation between radiotherapy and malignant transformation is not yet clearly established. Therefore, radiotherapy remains a good choice to reduce recurrence of a benign cranopharyngioma. PEEK implantation seems a safe option for allogenic reconstruction in patients with large maxillofacial defects. In complex cases the benefit of a multidisciplinary approach should not be underestimated.
