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Therapie eines malignen sympathischen Paraganglioms des Zuckerkandl'schen Organs — ein Fallbericht

Therapy of a malignant sympathetic paraganglioma of the organ of Zuckerkandl — A case report

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Summary

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.

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Abbreviations

BKS:

Blutkörperchensenkungsgeschwindigkeit

DOPA:

3,4-Dihydroxyphenylalanin gamma

GT:

Gamma-Glutamyl-Transferase

GOT:

Glutamat-Oxalazetat-Transaminase

GPT:

Glutamat-Pyruvat-Transaminase

Gy:

Gray-Einheit

HPLC:

Hochdruck-Flüssigkeits-Chromatographie

IU:

International Units

J-MIBG:

Jodmetajodobenzylguanidin

MPT:

alpha-methyl-para-tyrosin (Metyrosin)

PTH:

Parathormon

SMS:

Somatostatin

TT3:

gesamtes Trijodthyronin

TT4:

gesamtes Tetrajodthyronin (Thyroxin)

TBG:

Thyroxin-bindendes Globulin

TSH:

Thyreoidea-Stimulierendes-Hormon

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Authors and Affiliations

  1. Klinische Endokrinologie, Medizinische Hochschule Hannover, Deutschland

    F. Schuppert, J. Overbeck, T. H. Schürmeyer & A. von zur Mühlen

  2. Klinik für Abdominal- und Transplantationschirurgie, Medizinische Hochschule Hannover, Deutschland

    G. F. W. Scheumann & H. Dralle

  3. Hämatologie und Onkologie, Medizinische Hochschule Hannover, Deutschland

    C. Schöber & H. J. Schmoll

Authors
  1. F. Schuppert
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  2. G. F. W. Scheumann
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  3. C. Schöber
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  4. J. Overbeck
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  5. T. H. Schürmeyer
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  6. H. J. Schmoll
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  7. H. Dralle
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  8. A. von zur Mühlen
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Schuppert, F., Scheumann, G.F.W., Schöber, C. et al. Therapie eines malignen sympathischen Paraganglioms des Zuckerkandl'schen Organs — ein Fallbericht. Klin Wochenschr 69, 937–942 (1991). https://doi.org/10.1007/BF01798547

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  • DOI: https://doi.org/10.1007/BF01798547

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