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“The” pheochromocytoma: A benign, intra-adrenal, hypertensive, sporadic unilateral tumor. Does it exist?

  • International Association of Endocrine Surgeons—Manuscripts Presented at the 35th World Congress of the International Society of Surgery, Hong Kong, August 1993
  • Published:
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Abstract

This study aims to examine the frequency of the pheochromocytoma (pheo), defined as a “benign, intra-adrenal, hypertensive, sporadic, unilateral tumor.” Three large series amounting to 310 subphrenic chromaffin tumors operated over periods of 17, 23, and 41 years, respectively, have been reviewed. Among those combined 310 pheos, 48 (15.5%) were malignant (i.e., metastatic) and 262 (84.5%) were benign; 42 (13.5%) were ectopic (35% of malignant tumors versus 9.5% of benign tumors); 230 (74.2%) were hypertensive (74% of benign tumors versus 73% of malignant tumors); 29 (9.4%) were bilateral, including 23 patients with a family history; 41 (13.2%) of patients had MEN II type A or B syndrome; and 20 (6.5%) occurred in a phacomatosis setting. Some of the patients had a non-MEN family history of pheo (n=5), seemingly sporadic hyperparathyroidism (n=4), or other associated neuroendocrine tumor (n=9). Sometimes several of these features were combined. Finally 125 (40.3%) cases fitted the classic description of the tumor, 47.0% at the time of initial presentation and 40.3% at the end of follow-up. Late occurrence of metastases or metachronous diagnosis of familial disease make lifelong follow-up mandatory. Genetic studies may be indicated in pheochromocytoma patients.

Résumé

Le but de cette étude a été d'évaluer la fréquence du phéochromocytomes (pheo), défini par l'étiquette historique de “tumeur bénigne sporadique, localisé dans la surrénale, responsable d'hypertension”. Trois séries importantes, comprenant 310 tumeurs comportant des tissus chromaffines sous-phréniques, opérées pendant des périodes de 17, 23, et 41 ans, ont été revues. Parmi celles-ci, 48 (15.5%) étaient malignes, i.e. métastatiques, et 262 (84.5%) étaient bénignes, 42 (13.5%) étaient ectopiques (35% des tumeurs malignes vs 9.5% des tumeurs bénignes), 230 (74.2%) étaient responsables d'hypertension, (74% des tumeurs bénignes vs 73% des tumeurs malignes), 29 (9.4%) étaient bilatérales, parmi lesquelles 23 étaient des tumeurs familiales. Quarante et une (13.2%) avaient un syndrome MEN II A ou B, 20 (6.5%) étaient associées avec une phacomatose. D'autres avaient des antécédents familiaux de phéo non MEN (n=5) ou d'hyperparathyroïdie apparemment sporadique (n=4) ou d'autres tumeurs neuro-endocrines (n=9). Toutes caractéristiques confondues, et parfois combinées, 125 (40.3%) seulement avaient une description classique, 47% au moment de la présentation, et seulement 40.3% à la fin du suivi. La survenue de métastases ou le diagnostic de maladie familiale métachrone sont des arguments pour rendre la surveillance obligatoire la vie durant. Dans certains cas, des études génétiques sont souhaitables.

Resumen

Este estudio busca verificar la frecuencia del feocromocitoma, el cual ha sido definido históricamente como un “tumor unilateral benigno, intra-adrenal, hipertensivo y esporádico”; tres voluminosas series que comprenden 310 tumores cromafínicos subfrénicos operados durante 17, 23 y 41 años, respectivamente, han sido revisadas. Entre los 310 feocromocitomas, 48 (15.5%) eran malignos, o sea metastásicos, y 262 (84.5%) benignos; 42 (13.5%) fueron ectópicos (35% de los malignos vs. 9.5% de los benignos); 230 (74.2%) eran hipertensivos (74% de los benignos vs. 73% de los malignos); 29 (9.4%) fueron bilaterales, incluyendo 23 con historia familiar positiva; 41 (13.2%) tenían síndrome NEM II A o B y 20 (6.5%) se presentaron en un cuadro de facomatosis. Algunos presentaban una historia familiar de feocromocitoma no-NEM (5 casos) o hiperparatiroidismo aparentemente esporádico (4 casos) u otros tumores neuroendocrinos (9 casos). Algunas de estas caracteristicas aparecían posiblemente combinadas; finalmente 125 (40.3%) casos solamente se ajustaban a la descripción clásica del tumor, 47% en el momento de su presentación inicial y 40.3% sólo al final del período de seguimiento. El hecho de que se presenten metástasis o el diagnósitico metacrónico de enfermedad familiar hace obligatorio el seguimiento a largo plazo. Los estudios genéticos pueden estar indicados en pacientes con feocromocitoma.

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Authors and Affiliations

  1. Clinique Chirurgicale Adultes Est, Service de Chirurgie Générale et Endocrinienne, CHU de Lille, Hôpital Huriez, Place de Verdun, 59037, Lille Cédex, France

    F. R. C. S. Edbg & Michel Vix M.D.

  2. Department of Surgery, Sahlgrenska Sjukhuset, S-413 45, Göteborg, Sweden

    Svante Jansson M.D. & Lars-E. Tisell M.D.

  3. Klinik für Abdominal und Transplantationchirurgie, Medizinische Hochschule Hannover, D-3000, Hannover, Germany

    Hennig Dralle M.D. & Wolfgang Hiller M.D.

Authors
  1. Charles A. G. Proye M.D.
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  2. F. R. C. S. Edbg
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  3. Michel Vix M.D.
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  4. Svante Jansson M.D.
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  5. Lars-E. Tisell M.D.
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  6. Hennig Dralle M.D.
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  7. Wolfgang Hiller M.D.
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Proye, C.A.G., Edbg, F.R.C.S., Vix, M. et al. “The” pheochromocytoma: A benign, intra-adrenal, hypertensive, sporadic unilateral tumor. Does it exist?. World J. Surg. 18, 467–472 (1994). https://doi.org/10.1007/BF00353738

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