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.
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.
Malignant craniopharyngioma is uncommon but has a poor prognosis.
The role of radiotherapy in malignant transformation is not yet clearly established.
PEEK-PSI appears a safe option for allogenic reconstruction of large maxillofacial defects.
A multidisciplinary approach can benefit the patient when tumor expansion is beyond a surgeon’s field of expertise.
Craniopharyngiomas are slow growing, benign epithelial tumors, arising from cell remnants of Rathke’s pouch [ ].They can be divided in two different subtypes, the adamantinomatous and the papillary squamous type, each with distinct histopathological characteristics [ ].
Although craniopharyngiomas are considered WHO grade I tumors, they often show aggressive local behavior like hypothalamic brain invasion and multiple recurrences, with the vast majority being confined to the intracranial compartments [ , ]. Malignant transformation in a craniopharyngioma is extremely rare and limited to the adamantinomatous subtype [ ]. It was first reported in 1973 by Salyer et al. [ ]. Since then, 42 cases of malignant craniopharyngioma have been described [ , ]. Radiotherapy has been suggested as an inducing factor [ , ].
In this article, a case of secondary malignant craniopharyngioma with multiple recurrences and extracranial expansion is discussed. We will focus on the interdisciplinary, i.e. neurosurgical and maxillofacial, approach to the 7th tumor recurrence, occurring at the level of the right orbit, the greater sphenoid wing and the dura of the anterior temporal pole. A broad tumor resection with exenteration of the right eye, removal of the affected dura over the anterior temporal base and reconstruction with a PEEK-PSI and anterolateral thigh flap was performed by a team of maxillofacial- and neurosurgeons.
A 73-year-old woman with a history of recurrent craniopharyngioma was referred to the department of Neurosurgery following the MRI finding of a 7th tumor recurrence. The lesion was located in the right lateral orbital wall, invading the greater sphenoid wing and bulging into the temporal base and middle cranial fossa. The complete oncological history is displayed in Fig. 1 .
The patient presented with right retro-orbital pain. Clinically, there were no eye movement disturbances, nor signs of exophthalmia. Due to previous radiotherapy, vision was almost completely lost in the right eye. MRI showed an increased volume of a tumor remnant in the right lateral orbital wall, extending into the lateral part of the orbit. Anterior, the mass reached to the eyeball, posterior to 4–5mm before the superior orbital fissure. Tumor growth through the posterolateral orbital wall was seen, bulging into the middle cranial fossa with presumably meningeal invasion. The greater sphenoid wing was invaded up to the inferior orbital fissure. Furthermore, there was limited intradural bulging of the mass into the temporal fossa. Dimensions of the tumor were estimated at 42 × 26 × 30mm ( Fig. 2 ).
Further staging with a CT of the orbits, thorax and abdomen was performed without evidence of metastatic disease. The case was discussed multidisciplinary and despite the potential meningeal involvement, the lesion was considered resectable. A combined neurosurgical and maxillofacial surgical intervention was planned with resection of the former temporal craniotomy bone flap, the right lateral and superior orbital wall, the posterior orbital floor, a part of the zygomatic body, the lateral wall of the maxillary sinus and the skull base from sphenoid till orbital fissure, along with the right eyeball. This trajectory was simulated using 3D-models ( Fig. 3 ). Furthermore, a PEEK-PSI was designed based on the CT data of the surgical plan.