title: Recurrent malignant Triton tumour: first report on a long time survivor.
abstract: Malignant Triton tumour represents an extremely rare mesenchymal malignancy exhibiting histopathologic patterns of peripheral nerve sheath tumours and rhabdomyoblastic components, the latter usually determining the mostly fatal outcome. We report on a 26-year old patient diagnosed with malignant Triton tumour who developed multiple recurrences despite repeated aggressive surgery, chemo- and radiotherapy during an 8-year period. After Northern blotting analysis of an excised in-transit metastasis had revealed expression of retinoic receptors alpha and gamma, the patient received experimental treatment with isotretinoin and interferon-alpha for one year and remains without any evidence of disease for more than three years. This is the first report on a long-term survivor of multiple recurrences of malignant Triton tumour.
authors.affiliation: Clinical Division of Oncology, Department of Medicine I, University Hospital of Vienna, 18-20 Waehringer Guertel, A-1090 Vienna, Austria. wolfgang.koestler@akh-wien.ac.at
authors.forename: Wolfgang J
authors.initials: WJ
authors.lastname: Köstler
authors.forename: Gabriele
authors.initials: G
authors.lastname: Amann
authors.forename: Thomas W
authors.initials: TW
authors.lastname: Grunt
authors.forename: Christian F
authors.initials: CF
authors.lastname: Singer
authors.forename: Sonja M
authors.initials: SM
authors.lastname: Schneider
authors.forename: Thomas
authors.initials: T
authors.lastname: Brodowicz
authors.forename: Sandra
authors.initials: S
authors.lastname: Tomek
authors.forename: Christoph C
authors.initials: CC
authors.lastname: Zielinski
mesh_terms: D000328:Adult; D000970:Antineoplastic Agents / Q000627:therapeutic use*; D015152:Blotting, Northern; D003131:Combined Modality Therapy; D006801:Humans; D008297:Male; D009364:Neoplasm Recurrence, Local; D009442:Neurilemmoma / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D010524:Peripheral Nervous System Neoplasms / Q000188:drug therapy / Q000378:metabolism / Q000473:pathology*; D012333:RNA, Messenger / Q000378:metabolism; D012334:RNA, Neoplasm / Q000378:metabolism; D011879:Radiotherapy Dosage; D018168:Receptors, Retinoic Acid / Q000235:genetics / Q000378:metabolism; D000072482:Retinoic Acid Receptor alpha; D017741:Survivors; D000097768:Retinoic Acid Receptor gamma
chemical_list: D000970:Antineoplastic Agents; D012333:RNA, Messenger; D012334:RNA, Neoplasm; D018168:Receptors, Retinoic Acid; D000072482:Retinoic Acid Receptor alpha; D000097768:Retinoic Acid Receptor gamma; C000607071:RARA protein, human