Scala et al., from the Istituto Giannina Gaslini, report on a child who developed rapidly progressive moyamoya syndrome (MMS) after proton beam therapy (PBT) for a clivus chordoma. A combined indirect revascularization procedure by encephalo-duro-arterio-synangiosis (EDAS) and encephalo-myo-synangiosis (EMS) was performed with good neuroradiological and clinical outcome.
Regardless of the presence of known risk factors for MMS, strict neuroimaging surveillance is indicated in all patients treated with radiotherapy, including those receiving PBT. We suggest that early revascularization procedure should be considered in patients with worsening symptoms and/or sign of neuroradiological progression of cerebral vasculopathy. This management of MMS could lower the risk of permanent neurological deficits and improve patients' quality of life 1).
An infrasellar craniopharyngioma involving the sphenoid sinus and clivus 2).
The patient is a 44-year-old man who underwent resection of a posterior nasopharynx tumor 12 years earlier via left lateral rhinotomy approach. The final pathological analysis indicated the tumor was a craniopharyngioma, and the patient subsequently underwent focal radiation. The patient returned to medical attention complaining of dysequilibrium. A neurologic exam was nonfocal. Magnetic resonance imaging revealed a clival mass, separate from the sella turcica, with imaging characteristics concerning for chordoma or primary bone tumor. The lesion was resected via an endoscope-assisted endonasal transsphenoidal approach, with gross total resection achieved. Intraoperatively, the mass was noted to erode through the posterior nasopharynx, without extension superiorly into the sella or posteriorly through the clival dura (i.e., lesion was infrasellar). The final pathological results indicated the tumor was adamantinomatous craniopharyngioma 3).
Ectopic craniopharyngioma is a rare entity. The authors present a very rare case of an ectopic clival craniopharyngioma completely separate from the sella turcica.A 44-year old woman presented with abducens palsy. A MR imaging study and a CT scan revealed a cystic clival lesion separate from the sella turcica. Surgical resection was performed successfully with flexible endoscope-assisted procedure using an endonasal transsphenoidal approach. No evidence of involvement of the sellar region was found according to radiological, intra-operative, and clinical findings.A review of the literature revealed no other such cases. The discussion includes the formation of craniopharyngioma from the ectopic Rathke's pouch remnants and the surgical approach for clival lesions. We believe that our approach provides good results with minimal invasiveness for some clival lesions 4).
An unusual case of entirely infrasellar craniopharyngioma mimicking a clival chordoma is described. Only 22 cases of craniopharyngioma with nasopharyngeal extension have been reported in the literature. Of the reported cases, most were primarily intracranial with secondary downward extension; only two were thought to originate from an infrasellar location. The present case is another example of an entirely infrasellar craniopharyngioma, with extensive clival destruction, mimicking a clival chordoma. Relevant literature on the subject is reviewed 5).