Xanthogranuloma, Rathke

Hermann Muller; Ursel Gebhardt; Andreas Faldum; Monika Warmuth-Metz; Torsten Pietsch; Fabian Pohl; Gabriele Calaminus; Niels Sorensen

doi:10.1530/endoabs.32.P885

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Endocrine Abstracts (2013) 32 P885 | DOI: 10.1530/endoabs.32.P885

ECE2013 Poster Presentations Pituitary – Clinical (<emphasis role="italic">Generously supported by IPSEN</emphasis>) (127 abstracts)

Xanthogranuloma, Rathke‘s cyst, and Childhood Craniopharyngioma: results of prospective multinational studies of children and adolescents with rare sellar malformations

Hermann Müller ¹ , Ursel Gebhardt ¹ , Andreas Faldum ² , Monika Warmuth-Metz ³ , Torsten Pietsch ⁴ , Fabian Pohl ⁵ , Gabriele Calaminus ⁶ & Niels Sörensen ^1,

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¹Department of Pediatrics, Klinikum Oldenburg, Oldenburg, Germany; ²Institute of Biostatistics and Clinical Research, Westphälische Wilhelms University, Münster, Germany; ³Department of Neuroradiology, University Hospital, Würzburg, Germany; ⁴Institute of Neuropathology, University Hospital, Bonn, Germany; ⁵Department of Radiooncology, University Hospital, Regensburg, Germany; ⁶Department of Pediatric Oncology, Münster, Germany; ⁷Department of Neurosurgery, Evangelisches Kranklenhaus, Oldenburg, Germany.

Background: SUB:Pituitary – Clinical (Generously supported by IPSEN)Craniopharyngioma (CP), Rathke’s cyst (RC) and xanthogranuloma (XG) are closely related rare sellar masses, which share common embryogenic origin. Treatment strategies in children lack consensus, especially in terms of surgical and radiooncological treatment options.

Objective: To study clinical manifestations and treatment-related outco-me in RC, XG, and CP patients.

Patients and methods: Multicentre surveillance trial. Inclusion criteria were: i) histological diagnosis of CP, XG, or RC; ii) diagnosis. Main Outcome: overall survival, event-free survival (OS, EFS), quality of life (QoL). 14 RC, 14 XG, and 117 CP patients were included in the study.

Results: Five-Year OS rates are 1.00±0.00 in RC and XG; 0.97±0.02 in CP.5-year EFS are 0.85±0.10 in RC, 1.00±0.00 in XG, and 0.50±0.05 in CP. Surgical resection of XG results in complete remission without recurren-ce. Recurrences occur in RC (14%) and CR (59%), but can be efficiently treated by irradiation, reoperation, and/or intracystic treatment. Severe hypothalamic sequelae such as obesity and others effecting QoL are predominant in CP due to pre-surgical involvement (59%) and post-surgical lesions (44%) of posterior hypothalamic structures. Centres with lower neurosurgery patient load use more radical surgical approaches to treat CP, resulting in higher rates of obesity and reduced QoL. In spite of 46% anterior hypothalamic involvement, severe obesity is not encountered in XG.

Conclusions: Treatment of choice in XG and RC is radical surgery. In CP involving hypothalamic structures, less radical surgical approaches preserving hypothalamic integrity are recommended. Owing to frequent relapses, regular imaging during follow-up is recommended for CP and RC. Treatment of patients with sellar masses should be confined to experienced multidisciplinary teams. Due to the rareness of the diseases, international scientific collaboration (i.e. international trials) is recommended in order to achieve reliable results based on evaluation of larger cohorts.