Cited by 124Adrenal and Paraganglionic TumorsNeuroendocrine Tumor Research AdvancesEar and Head Tumors

The DOI matches its Crossref record; 27 of the 27 references it cites were found in a registry or an index.

Differences between sporadic and multiple endocrine neoplasia type 2A phaeochromocytoma

Pomares, F. J., Canas, R., Rodriguez, J. M., Hernandez, A. M., Parrilla, P., Tebar, F. J.·1998·Clinical Endocrinology

Abstract

OBJECTIVES: Phaeochromocytoma may be either sporadic or part of a familial cancer syndrome. We have investigated whether there are differences between sporadic and MEN 2A phaeochromocytomas. DESIGN: A retrospective study. We analysed age at presentation, sex, mode of presentation, clinical data, laboratory and imaging techniques, treatment, pathology and follow-up. PATIENTS: Forty-six patients diagnosed with phaeochromocytoma between 1979 and 1995 (23 sporadic and 23 familial) at the Hospital Universitario Virgen de la Arrixaca (Murcia, Spain), a tertiary referral centre. RESULTS: Mean age at presentation was 47 +/- 16 years for sporadic and 38 +/- 11 years for familial phaeochromocytoma (P < 0.05). The most common clinical feature and the presenting feature in cases of isolated phaeochromocytoma was hypertension. Of the MEN 2A patients, 52% were asymptomatic at diagnosis and only 35% presented with hypertension. Levels of adrenaline, noradrenaline, dopamine and metanephrines in a 24-hour urine connection confirmed the diagnosis in 95% of the isolated and 91% of the familial phaeochromocytomas. Computed tomography located 100% of the sporadic and 76% of the familial phaeochromocytomas (P < 0.02). All the MEN 2A phaeochromocytomas were bilateral compared with none of the sporadic phaeochromocytomas (P < 0.001). All the patients with familial phaeochromocytoma underwent bilateral adrenalectomy. The patients with sporadic phaeochromocytoma had the affected gland or the tumour, if extraadrenal, removed. CONCLUSIONS: In comparing sporadic and MEN 2A phaeochromocytoma we found differences in age at presentation, mode of presentation, clinical data, duration of clinical features, imaging findings bilaterality and type of surgical treatment required.

Overview

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Crossref · 10.1046/j.1365-2265.1998.3751208.x

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27 of 27 references verified0 citation errors · 0 not checkable

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  • Patients with familial pheochromocytoma may be asymptomatic in 50% of cases, with only a third presenting with hypertension.

    “Patients with familial pheochromocytoma may be asymptomatic in 50% of cases, with only a third presenting with hypertension, possibly representing earlier diagnosis and smaller lesions as a result of regular screening in these patients (195,196).”

    From Adrenal incidentaloma (2020) · cites this paper as [195]

Citations

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Research works printed but not linked: 6

  1. Bravo E.L., 1984, New England Journal of Medicine, 30, 1682
  2. Bravo E.L., 1993, Endocrinology and Metabolism Clinics of North America, 22, 329
  3. Howe J.R., 1993, Surgery, 114, 1070
  4. Orchard T., 1993, Surgery, 114, 1153
  5. Raue F., 1994, Endocrinology and Metabolism Clinics of North America, 23, 137
  6. Shapiro B., 1991, Critical Care Clinics, 7, 1

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Metadata

Authors
Pomares, F. J., Canas, R., Rodriguez, J. M., Hernandez, A. M., Parrilla, P., & Tebar, F. J.
Affiliations
Hospital Universitario Virgen de la Arrixaca (ES)
Venue
Clinical Endocrinology
Publisher
Wiley
Published
1998-02
Volume
48
Issue
2
Pages
195-200
ISSN
  • 0300-0664 (ISSN-L)
  • 1365-2265
OpenAlex
W2095363025
Cited by
124
References
27
Citations by year
2025: 2 · 2024: 6 · 2023: 2 · 2022: 4 · 2021: 3 · 2020: 4 · 2019: 1 · 2018: 2 · 2017: 4 · 2016: 7 · 2015: 3 · 2014: 6 · 2013: 4 · 2012: 6
FWCI
4.44
Type
journal article
Language
en
Open access
closed
Locations
  • Clinical Endocrinology (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Oncology › Neuroendocrine Tumor Research Advances
  • Health Sciences › Medicine › Oncology › Ear and Head Tumors
Keywords
pheochromocytoma, multiple endocrine neoplasia type 2A, hereditary cancer, age at presentation, hypertension, adrenalectomy, surgical treatment, tumor imaging
MeSH terms
Adolescent, Adrenal Gland Neoplasms, Adrenalectomy, Adult, Aged, Child, Diagnosis, Differential, Dopamine, Epinephrine, Female, Humans, Hypertension, Male, Metanephrine, Middle Aged, Norepinephrine, Pheochromocytoma, Retrospective Studies, Tomography, X-Ray Computed, Age of Onset, Multiple Endocrine Neoplasia Type 2a

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