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Multiple neuroectodermal abnormalities in pheochromocytoma patients

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Abstract

A series of 80 consecutive pheochromocytoma patients operated on from 1956 to 1985 were investigated for other neuroectodermal abnormalities. Such abnormalities were found in 23 (29%) patients. Medullary thyroid carcinoma was the most common associated neuroectodermal abnormality followed by von Recklinghausen's neurofibromatosis. Other abnormalities were intracranial tumors, midgut carcinoid, and parathyroid hyperplasia. All 6 patients with multiple pheochromocytomas had other neuroectodermal abnormalities. In the total series, the 14 patients with hereditary disease had multiple neuroectodermal abnormalities (MNA). Hypertension occurred significantly less often in MNA patients (p<0.05). All the MNA patients had benign adrenal pheochromocytoma. In 7 of 9 normotensive patients, pheochromocytoma was suspected because of the presence of other neuroectodermal abnormalities. None of these 9 patients had any cardio- or cerebrovascular complications, but these occurred in 22% of the hypertensive patients. No patient in the series died from pheochromocytoma, but 4 of 5 deceased MNA patients died as a result of their associated neuroectodermal disease. During the last decade, 40% of the pheochromocytoma patients had MNA diagnosed compared to 20% during the previous period. An increased observation for other neuroectodermal abnormalities in pheochromocytoma patients and for pheochromocytoma in patients with neuroectodermal abnormalities should lead to earlier diagnosis and a better prognosis for the patients concerned.

Résumé

Chez 80 patients consécutifs porteurs de phéochromocytome, opérés de 1956 à 1985, on a recherché des signes d'autres anomalies neuroectodermiques. On en a trouvé chez 23 patients (29%). L'anomalie associée la plus fréquente était le cancer médullaire de thyroïde, puis venait la neurofibromatose de von Recklinghausen. Les autres anomalies retrouvées étaient les tumeurs intracrâniennes, les tumeurs carcinoïdes de l'intestin moyen et l'hyperplasie parathyroïdienne. Les 6 patients ayant des phéochromocytomes multiples avaient des anomalies neuroectodermiques associées. Quatorze patients avec une maladie héréditaire avaient des anomalies neuroectodermiques multiples associées (ANM). Il y avait significativement moins d'hypertension parmi les patients avec des ANM (p<0.05). Tous les patients présentant une ANM, avaient aussi un phéochromocytome surrénalien bénin. Chez 1 patient parmi les 9 patients normotensifs, on a soupçonné un phéochromocytome en raison de l'association d'anomalies neuroectodermiques. Parmi ces 9 patients, il n'y a pas eu de complications cardio- ou cérébrovasculaires, mais celles-ci se sont produites chez 22% des patients hypertendus. Aucun patient dans cette série n'est mort de son phéochromocytome, mais 4 sur 5 patients avec une ANM sont morts de leur maladie neuroectodermique associée. Pendant ces 10 dernières années, on a fait le diagnostic d'ANM chez 40% des patients avec un phéochromocytome, alors qu'au cours des 10 précédentes années, ce diagnostic n'a été fait que chez 20%. Une recherche approfondie des anomalies neuroectodermiques associées chez le patient avec phéochromocytome ainsi que la recherche de phéochromocytome chez le patient présentant des anomalies neuroectodermiques devraient aider à poser le diagnostic plus précocément et ainsi à améliorer le pronostic.

Resumen

Una serie de 80 pacientes con feocromocitoma operados entre 1956 y 1985 fueron investigados para establecer la presencia de otras anomalías neuroectodérmicas, las cuales fueron halladas en 23 pacientes (29%). La más frecuente fue el carcinoma medular de tiroides, seguida de neurofibromatosis de von Recklinghausen. Otras anomalías encontradas fueron tumores intracraneales, carcinomas del intestino medio, e hiperplasia paratiroidea. La totalidad de los 6 pacientes con feocromocitomas múltiples poseían otras anomalías neuroectodérmicas. Los 14 pacientes con enfermedad hereditaria presentaban también anomalías neuroectodérmicas múltiples (ANM). La hipertensión se presentó con incidencia significativamente menor en los pacientes con ANM (p<0.05). Todos los pacientes con ANM tenían feocromocitoma adrenal benigno. En 7 de los 9 pacientes normotensos se sospechó el feocromocitoma por la presencia de otras anomalías neuroectodérmicas. Ninguno de los 9 pacientes exhibió complicaciones cardiovasculares o neurovasculares, pero éstas ocurrieron en 22% de los pacientes hipertensos. Ningún paciente de la serie falleció por causa de su feocromocitoma, pero 4 de los 5 pacientes fallecidos con ANM murieron como resultado de la enfermedad neuroectodérmica asociada. En el curso del último decenio, 40% de los pacientes con feocromocitoma tuvieron diagnóstico de ANM, contra 20% en el período previo. La creciente conciencia sobre la posibilidad de otras anomalías neuroectodérmicas en pacientes con feocromocitoma y de feocromocitoma en pacientes con anomalías neuroectodérmicas debe resultar en diagnósticos más tempranos y mejor pronóstico en estos pacientes.

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Authors and Affiliations

  1. Departments of Surgery I, Pathology, and Medicine II, Sahlgren's Hospital, University of Göteborg, Göteborg, Sweden

    Svante Jansson M.D., Lars -Erik Tisell M.D., Ph.D., Göran Hansson M.D., Ph.D. & Gunnar Stenström M.D., Ph.D.

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  1. Svante Jansson M.D.
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  2. Lars -Erik Tisell M.D., Ph.D.
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  3. Göran Hansson M.D., Ph.D.
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  4. Gunnar Stenström M.D., Ph.D.
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Supported by grants from the University of Göteborg, the Göteborg Medical Society, and the Swedish Medical Research Council (B87-17X-06534-05A).

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Jansson, S., Tisell, L.E., Hansson, G. et al. Multiple neuroectodermal abnormalities in pheochromocytoma patients. World J. Surg. 12, 710–717 (1988). https://doi.org/10.1007/BF01655896

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