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Malignant pheochromocytoma: A series of 14 cases observed between 1966 and 1990

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Abstract

We report 14 patients (9 males, 5 females) aged 15–59 years, treated for malignant pheochromocytoma. These patients were observed during the 1966–1990 period along with 68 other patients presenting benign pheochromocytomas. From the initial general presentation of the 14 patients, two groups could be individualized. In seven patients, the initial presentation seemed benign. After the excision, the recovery was complete, but patients recurred on average 7.8 yr later (range 1–22 yr). Tumors were in-traadrenal in six cases (5 single, 1 bilateral) and ex-traadrenal in one case. In the seven remaining patients, malignancy was evident from the first examination. The tumors were intraadrenal in 2 cases, ex-traadrenal in 5 cases. Frequency of extraadrenal lo-cations (6/14) was in this series significantly higher than in benign forms (9/68). Diagnosis of malignancy was based on metastases in 12 cases (lymph nodes in 5, bones in 5, liver in 4, lung in 2, brain in 1) and on peritumoral extension in 2 cases. No biological specificity was detected in urinary excretion of catecholamines or its metabolites. In 6 patients so far studied, an uptake of 131l MIBG was found in the tumor and/or metastases. Four patients received therapeutic doses of 131l MIBG and in three of them, this treatment led to a good result within a follow-up range of 12 to 66 months. The general evolution of the eleven cases followed up consisted of 5 patients who deceased with a median of 2 yr (range 1–12 yr) and 6 patients with a long survival 6.5 yr (range 2–15 yr) after the diagnosis of malignancy which corresponds to an overall survival of 14 yr (range 6–24 yr).

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References

  1. Remine W.H., Chong G.C., Van Heerden J.A., Sheps S.G., Harrison E.G. Current management of pheochromocytoma. Ann. Surg. 179:740, 1974.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  2. Van Heerden J.A., Sheps S.G., Hamberger B., Sheedy P.F., Poston J.G., Remine W.H. Pheochromocytoma: current status. Surgery 91:367, 1982.

    PubMed  Google Scholar 

  3. Scott H.W., Halter S.A. Oncologic aspects of pheochromocytoma: the importance of follow-up. Surgery 96:1061, 1984.

    PubMed  Google Scholar 

  4. Proye C., Fossati P., Lefebvre J., Decoulx M., Wemeau J.L., Fontaine P., Rwamasirabo E., Richardson A.J.C. La malignité d’un phéochromocytome est-elle prévisible? A propos de 50 patients opérés. Lyon Chir. 83:98, 1987.

    Google Scholar 

  5. Plouin P.F., Chatelier G., Delahousse Recherche, diagnostic et localisation du phéochromocytome: 77 cas dans une population de 21 420 hypertendus. Presse Med. 44:2211, 1987.

    Google Scholar 

  6. Andresen G.S., Toftdahl D.B., Lund J.O., Strandgaard S., Nielsen P.E. The incidence rate of pheochromocytoma and Conn’s syndrome in Denmark 1977–1981. J. Hum. Hypert. 2:187, 1988.

    Google Scholar 

  7. Stenstrom G., Ernest I., Tisell L.E. Long-term results in 64 patients operated upon for pheochromocytoma. Acta Med. Scand. 223: 345, 1988.

    Article  PubMed  CAS  Google Scholar 

  8. Beierwaltes W.H. Update on basic research and clinical experience with metaiodobenzylguanidine. Med. Pediat. Oncol. 15:163, 1987.

    Article  CAS  Google Scholar 

  9. Medeiros L.J., Wolf B.C., Balogh K. Adrenal pheochromocytoma a clinicopathologic review of 60 cases. Hum. Pathol. 16:580, 1985.

    Article  PubMed  CAS  Google Scholar 

  10. Peyrin L. Stratégie de l’exploration biochimique du phéochromocytome. Ann. Med. Inter. 134:235, 1983.

    CAS  Google Scholar 

  11. Favre R., De Haut M., Boudet C., Dalmaz Y., Cottet-Emard J.M., Peyrin L. Differential effect of guanethidine on dopamine and norepinephrine pools in urine, heart and the superior cervical ganglion in the rat. J. Neural. Transm. 70: 19, 1987.

    Article  PubMed  CAS  Google Scholar 

  12. Favre R., Peyrin L. Application de la Chromatographie liquide haute performance au dosage de l’acide vanyl mandélique (VMA) et de l’acide homovanyllique (HVA) urinaires. Journées de Chromatographie liquide haute performance, Paris, October 1986 (Abst.).

    Google Scholar 

  13. Badet C. Les phéochromocytomes malins: étude de 14 observations et revue de la littérature. Thèse de Médecine, Lyon, no 155, 1990 (215 f).

    Google Scholar 

  14. Mornex R., Berthezene F., Peyrin L., Tran Minh V., Martin J.P., Fulchiron D. Phéochromocytomes malins. Arch. Mal. Coeur 72:96, 1979.

    PubMed  Google Scholar 

  15. Hosaka Y., Rainwater L.M., Grant C.S., Farrow G.M., Vanheerden J.A., Lieber M.M. Pheochromocytoma nuclear deoxyribonucleic acid patterns studied by flow cytometry. Surgery 700:1003, 1986.

    Google Scholar 

  16. Tcherdakoff P., Simoni G., Milliez P. Caractères évolutifs du phéochromocytome: étude de 42 cas personnels. La Nouv. Presse Med. 3:861, 1974.

    CAS  Google Scholar 

  17. Melicow M.M. One hundred cases of pheochromocytoma (107 tumors) at the Columbia presbyterian medical center, 1926–1976. Cancer 40:1987, 1977.

    Article  PubMed  CAS  Google Scholar 

  18. Liu T.H., Chen G.S., Nan C. Clinico pathological and ultrastructural characteristics of pheochromocytomas. An analysis of 55 cases. Pathol. Res. Pract. 178:355, 1984.

    Article  PubMed  CAS  Google Scholar 

  19. Besson A., Chabloz R., Saegesser F. Vingt-sept observations vaudoises de phéochromocytomes (1926–1985). Med. Hyg. 44:2263, 1986.

    Google Scholar 

  20. Robinson R., Smith P., Whittaker S.R. Secretion of catecholamines in malignant pheochromocytoma. Br. Med. J. 1:1422, 1964.

    Article  PubMed Central  PubMed  CAS  Google Scholar 

  21. Oishi S., Sato T. Elevated serum neuron specific enolase in patients with malignant pheochromocytoma. Cancer 61:1167, 1988.

    Article  PubMed  CAS  Google Scholar 

  22. Grouzmann E., Comoy E., Bohuon C. Plasma neuropeptide Y concentrations in patients with neuroendocrine tumors. J. Clin. Endocrinol. Metab. 68: 808, 1989.

    Article  PubMed  CAS  Google Scholar 

  23. Shapiro B., Sisson J.C., Lloyd R., Nakajo M., Satterlee W., Beierwaltes W.H. Malignant pheochromocytoma: clinical, biochemical and scintigraphic characterization. Clin. Endocr. (Oxf.) 20:189, 1984.

    Article  CAS  Google Scholar 

  24. Shapiro B., Copp J.E., Sisson J.C., Eyre P.L., Wallis J., Beierwaltes W.H. Iodine 131 metaiodobenzylguanidine for the locating of suspected pheochromocytoma. Experience in 400 cases. J. Nucl. Med. 26:576, 1985.

    PubMed  CAS  Google Scholar 

  25. Ackery D.M., Tippett P.A., Condon B.R., Sutton H.E., Wyeth P. New approach to the localisation of pheochromocytoma: imaging with iodine 131 metaiodobenzyl guanidine. Br. Med. J. 280:1587, 1984.

    Article  Google Scholar 

  26. Brown M.L., Sheps S.G., Sizermore G. MIBG in the evaluation of suspected pheochromocytoma: Mayo Clinic experience. J. Nucl. Med. 25:94, 1984.

    Google Scholar 

  27. Chatal J.F., Charbonnel B. Comparison of iodobenzylguanidine imaging with computed tomography in locating pheochromocytoma. J. Clin. Endocrinol. Metab. 61:769, 1985.

    Article  PubMed  CAS  Google Scholar 

  28. Abemayor E., Harken A.H., Koop C.E. Multiple sequential pulmonary resections for metastatic pheochromocytoma with long-term survival. Am. J. Surg. 140:696, 1980.

    Article  PubMed  CAS  Google Scholar 

  29. Siddiqui M.Z., Von Eyben F.E., Spanos G.S. High voltage irradiation and combination chemotherapy for malignant pheochromocytoma. Cancer 62: 686, 1988.

    Article  PubMed  CAS  Google Scholar 

  30. Burowski R.M., Vidt D.G. Chemotherapy trials in malignant pheochromocytoma: report of two patients and review of the literature. J.Surg. Oncol. 27:89, 1984.

    Article  Google Scholar 

  31. Averbuch S.D., Steakley C.S., Young R.C., Gelmann E.P., Goldstein D.S., Stull R., Keiser J.R. Malignant pheochromocytoma: effective treatment with a combination of cyclophosphamide, vincristine and decarbazine. Ann. Intern. Med. 109:267, 1988.

    Article  PubMed  CAS  Google Scholar 

  32. Krempf M., Lumbroso J., Mornex R., Brendel A.J., Wemeau J.L., De Lisle M.J., Aubert B., Carpentier P., Fleury-Goyon M.C., Gibold C., Guyot M., Lahneche B., Marchandise X., Schlumberger M., Charbonnel B., Chatal J.F. Use of 131 I iodobenzylguanidine in the treatment of malignant pheochromocytoma. J. Clin. Endocrinol. Metab. 72:455, 1991.

    Article  PubMed  CAS  Google Scholar 

  33. Sisson J.C., Shapiro B., Beierwaltes W.H., Glowniak J.V., Nakajo M., Mangner T.J., Carey J.E., Swanson D.P., Copp J.E., Satterlee W.G., Wieland D. Radiopharmaceutical treatment of malignant pheochromocytoma. J. Nucl. Med. 24:197, 1984.

    Google Scholar 

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Authors and Affiliations

  1. Service d’Endocrinologie, Pavillon X, Hôpital Edouard Herriot, 69003, Lyon, France

    R. Mornex M.D. & C. Badet

  2. Laboratoire de Physiologie, Faculté de Médecine, 8 avenue Rockefeller, 69008, Lyon, France

    L. Peyrin

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  1. R. Mornex M.D.
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  2. C. Badet
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  3. L. Peyrin
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Mornex, R., Badet, C. & Peyrin, L. Malignant pheochromocytoma: A series of 14 cases observed between 1966 and 1990. J Endocrinol Invest 15, 643–649 (1992). https://doi.org/10.1007/BF03345808

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