title: [Therapy of a malignant sympathetic paraganglioma of the organ of Zuckerkandl--a case report].
abstract: We present a case report on a 35-year-old patient in whom a malignant sympathetic paraganglioma of the organ of Zuckerkandl was the cause of severe hypertension with excessive perspiration at night. Since curative surgery was not possible medical treatment was initiated. Interferon alfa 2b (Intron A, Essex Pharma) and the somatostatin-analogue SMS 201-995 (Sandostatin, Sandoz) had no effect on catecholamine production and progression of the tumor. Treatment with alpha-methyl-para-tyrosin (MPT, [Metyrosin], Demser, MSD) turned out to be an effective and well tolerable therapy in this patient with peritoneal carcinosis. Clinical and hormonal progression of the paraganglioma resumed only after two years of therapy, which constitutes the longest documented period of time of successful MPT treatment. The superior efficacy of MPT in our patient should encourage postoperative medical treatment with MPT in malignant pheochromocytoma or malignant paraganglioma, particularly when the tumor turns out to be resistent to alpha blocking drugs.
authors.affiliation: Klinische Endokrinologie, Medizinische Hochschule Hannover.
authors.forename: F
authors.initials: F
authors.lastname: Schuppert
authors.forename: G F
authors.initials: GF
authors.lastname: Scheumann
authors.forename: C
authors.initials: C
authors.lastname: Schöber
authors.forename: J
authors.initials: J
authors.lastname: Overbeck
authors.forename: T H
authors.initials: TH
authors.lastname: Schürmeyer
authors.forename: H J
authors.initials: HJ
authors.lastname: Schmoll
authors.forename: H
authors.initials: H
authors.lastname: Dralle
authors.forename: A
authors.initials: A
authors.lastname: von zur Mühlen
mesh_terms: D000328:Adult; D001012:Aorta, Abdominal / Q000473:pathology; D003131:Combined Modality Therapy; D004298:Dopamine / Q000652:urine; D004837:Epinephrine / Q000652:urine*; D006801:Humans; D006973:Hypertension / Q000188:drug therapy*; D008207:Lymphatic Metastasis; D008297:Male; D008781:Methyltyrosines / Q000627:therapeutic use*; D009360:Neoplastic Cells, Circulating; D009638:Norepinephrine / Q000652:urine*; D010236:Paraganglioma, Extra-Adrenal / Q000188:drug therapy* / Q000601:surgery*; D012186:Retroperitoneal Neoplasms / Q000188:drug therapy* / Q000601:surgery*; D019805:alpha-Methyltyrosine
chemical_list: D008781:Methyltyrosines; D019805:alpha-Methyltyrosine; D004298:Dopamine; D009638:Norepinephrine; D004837:Epinephrine
vernacular_title: Therapie eines malignen sympathischen Paraganglioms des Zuckerkandl'schen Organs--ein Fallbericht.