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Endocrine Abstracts (2016) 41 EP249 | DOI: 10.1530/endoabs.41.EP249

Clinical case reports - Pituitary/Adrenal

Spinal metastasis in childhood-onset craniopharyngioma: Case report, review of the literature and experiences in the German childhood-onset craniopharyngioma registry

Martin Jaspers1,2, Anika Hoffmann1, Kristin Lohle1, Angela Brentrup3 & Hermann L Müller1

1Department of Pediatrics, Klinikum Oldenburg, Medical Campus University Oldenburg, Oldenburg, Germany; 2UMCG, Groningen, The Netherlands; 3Department of Neurosurgery, University Hospital Münster, Münster, Germany.

Background: Remote recurrence and metastasis are unusual complications in childhood-onset adamantinomatous craniopharyngioma mainly occurring either along a previous surgical route or by seeding via cerebrospinal fluid.

Case description: An 11-year-old female patient initially presented with headache and neck pain as well as nausea over the course of 2 months. A sellar/suprasellar mass (4.0 cm×4.0 cm 5.0 cm) was detected on magnetic resonance imaging (MRI). Initial surgery resulted in complete resection (CR) based on intraoperative microscopic inspection. CR was confirmed by postoperative MRI. The tumor was histologically determined to be an adamantinomatous craniopharyngioma. Seven years after initial CR, the patient presented with back pain spreading to the ventral side of the upper legs as well as a loss of strength. MRI showed a spinal neoplasm at the level of T12/L1 without any sign of local sellar/suprasellar recurrence. The patient underwent a spinal tumor resection without complications. Histological analysis confirmed the spinal tumor to be a metastasis of the initial adamantinomatous craniopharyngioma. Clinical complaints due to the spinal metastasis ceased after CR. Currently, the patient is in complete remission 9 years after CR of the sellar/suprasellar craniopharyngioma and 2 years after CR of a spinal metastasis of craniopharyngioma.

Only one case of an adult patient with spinal metastasis of an adamantinomatous craniopharyngioma has been reported in the literature up to now. Our case represents the first case of childhood-onset craniopharyngioma with spinal metastasis in the total cohort of 582 patients, recruited prospectively in the German childhood craniopharyngioma registry.

Conclusions: We report the first case of remote spinal recurrence of a childhood-onset adamantinomatous craniopharyngioma. Spinal metastasis is a very rare complication in childhood-onset craniopharyngioma and should be considered in long-term follow-up of childhood-onset craniopharyngioma patients with peripheral neurological complaints and symptoms.

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