title: Recurrent and metastatic clivus chordoma: systemic palliative therapy retards disease progression.
abstract: We report on a male patient with progressive and metastatic clivus chordoma treated over a period of 9 years by a multidisciplinary approach. Within the first 4 years, the patient underwent surgery four times. Thereafter, he received radiotherapy and subsequent chemotherapy. Stabilization of disease was achieved repeatedly for variable periods under local radiotherapy, systemic chemotherapy, immunomodulatory and anti-angiogenic therapy with isotretinoin and interferon-alpha, followed by thalidomide. Due to the occurrence of brain and lung metastases 8 years after initial diagnosis, liposomal doxorubicin was added to thalidomide. At the last follow-up control the patient had stable disease, with no progression of the intracranial tumor and regression of pulmonary metastases. He is in a good physical, psychological and neurological condition with a Karnofsky score of 80. Our observations show that multimodal therapy including a systemic palliative approach is associated with long quiescent intervals in recurrent chordoma and with regression of its metastases. Use of substances with high efficacy on tumor tissue and low toxicity, allowing long-term administration, seems promising in similar situations.
authors.affiliation: Clinical Division of Oncology, Department of Medicine I, Medical University Vienna, General Hospital (AKH), Vienna, Austria.
authors.forename: Katharina
authors.initials: K
authors.lastname: Schönegger
authors.forename: Ellen
authors.initials: E
authors.lastname: Gelpi
authors.forename: Daniela
authors.initials: D
authors.lastname: Prayer
authors.forename: Karin
authors.initials: K
authors.lastname: Dieckmann
authors.forename: Christian
authors.initials: C
authors.lastname: Matula
authors.forename: Marco
authors.initials: M
authors.lastname: Hassler
authors.forename: Johannes A
authors.initials: JA
authors.lastname: Hainfellner
authors.forename: Christine
authors.initials: C
authors.lastname: Marosi
mesh_terms: D002817:Chordoma / Q000000981:diagnostic imaging / Q000556:secondary* / Q000628:therapy*; D003131:Combined Modality Therapy; D003388:Cranial Fossa, Posterior / Q000000981:diagnostic imaging / Q000473:pathology; D018450:Disease Progression; D006801:Humans; D017567:Karnofsky Performance Status; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy*; D010166:Palliative Care; D011859:Radiography; D012008:Recurrence; D019292:Skull Base Neoplasms / Q000000981:diagnostic imaging / Q000473:pathology / Q000628:therapy*; D016896:Treatment Outcome