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High incidence of malignant pheochromocytoma in a surgical unit. 26 cases out of 100 patients operated from 1971 to 1991

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Abstract

In this surgical series of 100 pheochromocytomas (PH), where 91 cases were studied and followed up, the frequency of malignancy appears high (29%) on macroscopic criteria observed at the first operation (25 out 26 cases of malignant PH) or thanks to the occurence of metastases (1 case out of 26 malignant PH). While some clinical signs are suggestive, there is no excretory profile predictive of malignancy. Isolated or predominant secretion of dopamine is not specific of malignancy event if it occurs more frequently and severely in malignant PH. Bilateral involvement, occurring in the setting of phacomatosis or of multiple endocrine neoplasia (MEN II), or in a sporadic case, is not a predictor for malignancy. Contrarily, extraadrenal involvement is a major feature indicative of malignancy. A CT scan which demonstrates possible locoregional invasion, and a MIGB scan revealing distant localizations raise the suspicion of malignancy. The preferred surgical approach is via a laparotomy, thus allowing the exploration of all possible abdominal chromaffin site and the search for intraabdominal metastases. Intraoperative MIBG scanning checks the completeness of the excision. Flow cytometric DNA ploidy studies have been performed in 25 of our patients. The follow up of benign or malignant PH, after excision “for cure” must be life-long: clinically, biologically and by MIGB scan. Persistence of the disease after surgery or recurrence with unresectable metastases can benefit from MIGB and chemotherapy.

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References

  1. Linnoila R.I., Keiser H.R., Steinberg S.M., Lack E.E. Histopathology of benign versus malignant sympathoadrenal paragangliomas: Clinicopathologic study of 120 cases including unusual histologic features. Hum. Pathol. 21:1168, 1990.

    Article  PubMed  CAS  Google Scholar 

  2. Hosaka Y., Rainwater L.M., Grant C.S., Farrow G.M., Van Heerden J.A., Lieber M.M. Pheochromocytoma: Nuclear deoxyribonucleic acid pattern by flow cytometry. Surgery 100:1003, 1986.

    PubMed  CAS  Google Scholar 

  3. 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 

  4. Hauss J., Pircher W., Spiegel H.U., Kertelge E., Roessner A., Vetter H. Pheochromozytombehandlung: Wandel von Diagnostik und Therapie. Acta Med. Austr. 4:95, 1988.

    Google Scholar 

  5. Badet C. Fr Les phéochromocytomes malins. Etude de 14 observations. Revue de la littérature Thèse Lyon, 1990. University of Lyon, Ed., Lyon.

    Google Scholar 

  6. Thompson N.W., Alio M.D., Shapiro B., Sisson J.C., Beierwaltes W. Extra adrenal and metastatic pheochromocytoma: the role of 131 I metaiodobenzylguanidine (131 IMIBG) in localisation and management. World J. Surg. 8:605, 1984.

    Article  PubMed  CAS  Google Scholar 

  7. Proye C., Vérin P., Sautier M., Thevenin D., Fontaine P., Vantyghem M.C., Wemeau J.L. Expérience de 60 interventions pour phéochromocytome. Rev. Franc. End. Clin. 30:291, 1989.

    Google Scholar 

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

    PubMed  Google Scholar 

  9. Tison E., Fontaine P., Cantineau D., Vantyghem M.C., Lecomte-Houcke M., Proye C., Lefebvre J. Phéochromocytome et maladie de von Hippel Lindau. A propos d’un cas avec revue de la littérature. Sem Hôp Paris 65:941, 1989.

    Google Scholar 

  10. Tison E., Marchand X., Kacet S., Proye C., Lekieffre J. Phéochromocytome malin associé à une maladie de Recklinghausen. A propos d’un cas. Ann. Cardiol. Angéiol. 35:551, 1986.

    CAS  Google Scholar 

  11. Van Heerden J.A., Roland C.F., Carnery A., Sheps S.G., Grant C.S. Long term evaluation following resection of apparently benign pheochromocytomas/paragangliomas. World J. Surgery 14:325, 1990.

    Article  Google Scholar 

  12. Proye C., Fossati P., Fontaine P., Lefebvre J., Decoulx M., Wemeau J.L., Dewailly D., Rwamasirabo E., Cecat P. Dopamine secreting pheochromocytoma. An unrecognized entity? Classification of pheochromocytomas according to their type of secretion. Surgery 100:1154, 1986.

    PubMed  CAS  Google Scholar 

  13. Fontaine P., Romon M., Dewailly D., Veret E., Racadot-Leroy N., Proye C., Fossati P. Le phéochromocytome à dopamine. Rev. Franc. End. Clin. (Paris) 26:41, 1985.

    Google Scholar 

  14. Fossati P., Romon-Rousseaux M., Racadot A., Racadot-Leroy N., Proye C., Wemeau J.L., Bethouart M., Mazucca M. Le phéochromocytome à dopamine. Presse Med. 11:1607, 1982.

    CAS  Google Scholar 

  15. Goldstein D.S., Stull R., Eisenhofer G., Sisson J., Weder A., Averbuch S.D., Keiser H.R. Plasma 3–4 dihydroxphenylalanine (Dopa) and catecholamines in neuroblastoma and pheochromocytoma. Ann. Intern. Med. 705:887, 1986.

    Article  Google Scholar 

  16. Tippe P.A., Mc Ewan A.J., Acrery D.M. A reevaluation of dopamine excretion in pheochromocytoma. Clin. Endocrinol. (Oxf.) 25:401, 1986.

    Article  Google Scholar 

  17. Serrano P.A., Chavez-Laba B., Sanchez-Torres G. Dopamine excretion in benign pheochromocytoma. Biomedicine 29: 229, 1978.

    PubMed  CAS  Google Scholar 

  18. Vinik A.I., Shapiro B., Thompson N.W. Plasma gut hormone levels in 37 patients with pheochromocytomas. World J. Surgery 10:593, 1986.

    Article  CAS  Google Scholar 

  19. 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 

  20. Heath H., Edis A.J. Pheochromocytoma associated with hyperalcemia and ectopic secretion of calcitonin. Ann. Intern. Med. 91:208, 1979.

    Article  PubMed  Google Scholar 

  21. Altergott R., Barbato A., Lawrence C.A., Paloyan E., Freeark R.J., Prinz R.A. Spectrum of catecholamine secreting tumours of the organ of Zuckerkandel. Surgery 98:1121, 1985.

    PubMed  CAS  Google Scholar 

  22. Scott Jr., H.W., Reynolds V., Green N., Page D., Oates I.A., Robertson D., Roberts S. Clinical experience with malignant pheochromocytoma. SGO 154:801, 1982.

    PubMed  Google Scholar 

  23. Michel F., Gattegno B., Sicard J.F., Rolan J., Thibaut P. A propos d’une observation de phéochromocytome vésical malin. Conduite diagnostique et thérapeutique. Ann. Urol. 24:396, 1990.

    CAS  Google Scholar 

  24. Ochi K., Yostllora S., Morita M., Tarenchi M. Phechromocytoma of the bladder. Urology 17:228, 1981.

    Article  PubMed  CAS  Google Scholar 

  25. Das S., Lowe P. Malignant pheochromocytoma of the bladder. J. Urol. 123: 282, 1980.

    PubMed  CAS  Google Scholar 

  26. Chatal J.F., Charbonnel B. Comparaison de la scintigraphie à la MIBG et de la tomodensitométrie pour localiser les phéochrocytomes. Ann. Endocrinol. (Paris) 46:125, 1985.

    Google Scholar 

  27. Gough I.R., Thompson N.W., Shapiro B., Sisson J.C. Limitations of 131 I-MIBG scintigraphy in locating pheochromocytomas. Surgery 90:115, 1985.

    Google Scholar 

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

    PubMed  CAS  Google Scholar 

  29. Swensen S.J., Brown M.L., Sheps S.G., Siezmore G.W., Gharis H., Grant C.S., Van Heerden I. Use of 131 I-MIBG Scintigraphy in the evaluation of suspected pheochromocytoma. Mayo Clin. Proc. 60: 299, 1985.

    Article  PubMed  CAS  Google Scholar 

  30. Proye C., Thevenin D., Cecat P., Petillot P., Carnaille B., Vérin P., Sautier M., Racadot N. Exclusive use of calcium channel blockers in preoperative and intraoperative control of pheochromocytomas: Hemodynamics and free catecholamine assays in 41 consecutive patients. Surgery 106:1149, 1989.

    PubMed  CAS  Google Scholar 

  31. Cullen M.L., Staren E.D., Strauss A.R., Doolas A., Shah R., Patel S., Economou S.G. Pheochromocytoma: operative strategy. Surgery 98: 927, 1985.

    PubMed  CAS  Google Scholar 

  32. Irvin G.L. III, Fishman L.M., Sher J.A., Yeung L.K., Irani H. Pheochromocytoma. Lateral versus anterior operative approach. Ann. Surg. 209:774, 1989.

    Article  PubMed Central  PubMed  Google Scholar 

  33. Schroder H.D., Johannsen L. Demonstration of S 100 protein in sustentacular cells of pheochromocytomas and paragangliomas. Histopathology 10:1023, 1986.

    Article  PubMed  CAS  Google Scholar 

  34. Hassoun J., Monges G., Giraud P., Henry J.F., Charpin C., Payan H., Toga M. Immunohistological study of phheochromocytomas. An investigation of Methionin-Enkephalin, Vasoactiv intestinal peptide, Somatostatin corticotropin, Endorphin and Calcitonin in 16 tumors. Am. J. Pathol. 114:56, 1984.

    PubMed Central  PubMed  CAS  Google Scholar 

  35. Lloyd R.V., Shapiro B., Sisson J.C., Kalff V., Thompson N.N., Beierwaltes W.D. An immunohistochemical study of pheochromocytomas. Arch. Pathol. Lab. Med. 108:541, 1984.

    PubMed  CAS  Google Scholar 

  36. 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 

  37. Brennan M.F., Keiser A.R. Persitent and recurrent pheochromocytoma: the role the surgery. World J. Surg. 6:397, 1982.

    Article  PubMed  CAS  Google Scholar 

  38. Averbuch S.D., Steadkley C.S., Young R.C., Gelmann E.P., Golstein D.S., Stull R.S., Keiser H.R. Malignant pheochromocytoma: Effective treatment with a combination of Cyclophosphamide, Vincristine, and Dacabarine. Ann. Intern. Med. 109:267, 1988.

    Article  PubMed  CAS  Google Scholar 

  39. Charbonnel B., Chatal J.F., Brendel A.J., Lanehche B., Lumbroso J., Marchandise X., Mornex R., Schlumberger M., Wemeau J.L. Le traitement des phéochromocytomes malins par la 131 I-Métaiodobenzylguanidine. Ann. Endocrinol. (Paris) 49:344, 1988.

    CAS  Google Scholar 

  40. Decoulx M., Wemeau J.L., Racadot-Leroy N., Grimbert I., Proye C., Plane C. L’alpha méthyl paratyrosine dans le traitement des phéochromocytomes malins. Rev. Med. Int. 8:383, 1987.

    Article  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of General and Endocrine Surgery, France

    C. Proye (Surgical Professorial Unit), M. Vix (Surgical Professorial Unit) & A. Goropoulos (Surgical Professorial Unit)

  2. Department of Pathology, Lille Cedex, France

    P. Kerlo & M. Lecomte-Houcke

Authors
  1. C. Proye
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  2. M. Vix
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  3. A. Goropoulos
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  4. P. Kerlo
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  5. M. Lecomte-Houcke
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Proye, C., Vix, M., Goropoulos, A. et al. High incidence of malignant pheochromocytoma in a surgical unit. 26 cases out of 100 patients operated from 1971 to 1991. J Endocrinol Invest 15, 651–663 (1992). https://doi.org/10.1007/BF03345810

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