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Spectrum of pheochromocytoma in the131I-MIBG era

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Abstract

131I-metaiodobenzylguanidine (131I-MIBG) scintigraphy allows for both functional diagnosis and anatomical localization of pheochromocytoma. The spectrum of pheochromocytoma since the routine use of preoperative131I-MIBG scan was studied.

From 1980 to 1986, a total of 34 patients were primarily diagnosed and treated at the University of Michigan Medical Center, Ann Arbor, Michigan, U.S.A. There were 16 males and 18 females. The mean age was 38 years and 4 patients (11.8%) were under 18 years of age. Six patients (17.6%) had family history of pheochromocytoma or multiple endocrine neoplasia (MEN) II syndrome. The presenting symptoms were hypertension in 29 patients (85.3%); attacks of headache, palpitation, sweating, and flushing in 4 (11.8%), and 1 patient presented with a neck mass.

Plasma catecholamines were elevated in 97% of patients while urinary catecholamines and metabolites were elevated in 93.5%.131I-MIBG was accurate in 82.3%, partly positive in 11.8%, and false-negative in 5.9% of patients. CT scan was accurate in 80%, partly positive in 10%, but failed to show the tumor in another 10% of patients.

At operation, extraadrenal lesions were found in 38.2% of the patients and among these, one-third were extraabdominal. Multiple tumors occurred in 5 (14.7%), and bilateral adrenal lesions occurred in 4 patients (11.8%). Malignancy was diagnosed in 3 patients (8.8%) after an average follow-up period of 2 years.

We conclude that the use of routine preoperative131I-MIBG scanning improves localization of pheochromocytoma and earlier diagnosis is possible in patients with MEN II syndrome. Multiple tumors, extraadrenal and extraabdominal lesions occur more often than commonly believed. The low rate of confirmed malignancy is probably related to the short period of follow-up.

Résumé

La scintigraphie à l'131I-MIBG permet de diagnostiquer et de localiser les phéochromocytomes. Toute la gamme de types de phéochromocytomes reconnu depuis l'utilisation préopératoire systématique de la scintigraphie à l'131I-MIBG est présentée.

Entre 1980 et 1986, 34 patients ont été explorés et traités au Centre Médical de l'Université de Michigan, Ann Arbor, Michigan. Il y avait 16 hommes et 18 femmes. L'âge moyen était de 38 ans, et 4 patients (11.8%) avaient moins de 18 ans. Six patients (17.6%) avaient des antécédents familiaux de phéochromocytome ou de néoplasmes endocrines multiples (MEN) du type II. Les symptômes amenant à consulter étaient l'hypertension chez 29 patients (85.3%), des crises de céphalées, des palpitations et un flush chez 4 patients (11.8%); un patient présentait une masse cervicale.

Les catécholamines plasmatiques étaient élevées chez 97% des patients alors que les catécholamines et leurs métabolites étaient en quantité élevée chez 93.5% des patients. La scintigraphie à l'131I-MIBG était positive chez 82.3%, partiellement positive chez 11.8%, et faussement négative chez 5.9% des patients. La tomodensitométrie était positive chez 80%, partiellement positive chez 10%, et faussement négative chez 10% des patients.

A l'intervention, des lésions extra-surrénales étaient présentes dans 38.2% des cas, et parmi celles-ci, un tiers étaient extra-abdominales. Les tumeurs étaient multiples dans 5 cas (14.7%), et bilatérales surrénales dans 4 cas (11.8%). Une tumeur maligne était diagnostiquée chez 3 patients (8.8%), après une période de suivi de 2 ans en moyenne.

Nous concluons que l'utilisation systématique de la scintigraphie à l'131I-MIBG améliore la localisation des phéochromocytomes, permettant un diagnostic plus précoce chez les patients présentant un syndrome MEN II. La multiplicité tumorale et les localisations extra-médullosurrénales ou extra-abdominales se voient beaucoup plus fréquemment qu'on le pensait auparavant. Le taux de malignité peu élevé était probablement en rapport avec la courte période de suivi.

Resumen

La escintigrafía con131I-MIBG permite tanto la localización anatómica como el diagnóstico funcional del feocromocitoma. Las características o espectro del feocromocitoma a partir del uso rutinario de131I-MIBG han sido estudiadas.

Entre 1980 y 1986, un total de 34 pacientes fueron diagnosticados y tratados en el Centro Médico de la Universidad de Michigan, Ann Arbor, Michigan, 16 hombres y 18 mujeres. La edad promedio fue 38 años, con 4 pacientes (11.8%) menores de 18 años. Seis pacientes (17.6%) tenían historia familiar de feocromocitoma o de síndrome de neoplasia endocrina múltiple tipo II. Los síntomas de presentación fueron: hipertensión en 29 casos (85.3%), ataques de cefalea, palpitación, sudoración, y enrojecimiento (“flushing”) en 4 (11.8%), y un paciente presentaba masa cervical.

Las catecolaminas plasmáticas aparecieron elevadas en 97% de los pacientes, mientras las catecolaminas urinarias y sus metabolitos aparecieron elevadas en 93.5%. La escintigrafía con131I-MIBG resultó certera en 82.3%, parcialmente positiva en 11.8%, y falsa-negativa en 5.9% de los casos. La tomografía computadorizada resultó certera en 80%, parcialmente positiva en 10%, pero falló en demostrar el tumor en otro 10% de los pacientes.

En la operación se hallaron lesiones por fuera de las suprarrenales en 38.2% de los pacientes, y entre estos un tercio estaban por fuera del abdomen. Tumores múltiples ocurrieron en 5 pacientes (14.7%), lesiones suprarrenales bilaterales en 4 (11.8%). Se diagnosticó malignidad en 3 casos (8.8%) después de un período promedio de seguimiento de 2 años.

Nuestra conclusión es que el uso preoperatorio rutinario de la Escintigrafía con131I-MIGB representa un avance en la localización preoperatoria del feocromocitoma, con lo cual se puede hacer un diagnóstico más temprano en pacientes con el síndrome de neoplasia endocrina múltiple tipo II. Hay una mayor incidencia, de la que comunmente se conoce, de tumores múltiples, extraadrenales, y extraabdominales. La baja tasa de malignidad confirmada probablemente se relaciona con el corto período de seguimiento.

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Authors and Affiliations

  1. Department of Surgery, University of Hong Kong, Queen Mary Hospital, Hong Kong

    Polly S. Y. Cheung M.B.B.S., F.R.C.S., Norman W. Thompson M.D., Carl F. Dmuchowski Ph.D. & James C. Sisson M.D.

  2. Department of Surgery, Division of Endocrine Surgery, and Department of Internal Medicine, Division of Nuclear Medicine, University of Michigan Medical Center, Ann Arbor, Michigan, USA

    Polly S. Y. Cheung M.B.B.S., F.R.C.S., Norman W. Thompson M.D., Carl F. Dmuchowski Ph.D. & James C. Sisson M.D.

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  1. Polly S. Y. Cheung M.B.B.S., F.R.C.S.
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  2. Norman W. Thompson M.D.
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  3. Carl F. Dmuchowski Ph.D.
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  4. James C. Sisson M.D.
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Cheung, P.S.Y., Thompson, N.W., Dmuchowski, C.F. et al. Spectrum of pheochromocytoma in the131I-MIBG era. World J. Surg. 12, 546–550 (1988). https://doi.org/10.1007/BF01655447

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