ArticleJournal of endocrinological investigation2005
Unique association of non-functioning pheochromocytoma, ganglioneuroma, adrenal cortical adenoma, hepatic and vertebral hemangiomas in a patient with a new intronic variant in the VHL gene.
Article in Journal of endocrinological investigation, 2005. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers, 1 of them a synthesis that pooled it.
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Who cites it
9 citing papers in PubMed, 1 synthesis or guideline pooled it, 30 citations in OpenAlex.
- Adrenal mixed corticomedullary tumors: report of a case with molecular characterization and systematic review.Virchows Archiv : an international journal of pathology · 2026Pooled it
- The clinical spectrum of adrenal ganglioneuromas extends from severe hypertension to an asymptomatic incidentaloma; two cases and mini review of literature.Hormones (Athens, Greece) · 2025Review
- Left adrenal aldosteronism coexisting with left paraaortic paraganglioma presenting as bilateral adrenal and left paraaortic tumors- comprehensive adrenal evaluation aiding perfect management: a case report.BMC endocrine disorders · 2022Article
- Case Report: Pheochromocytoma and Synchronous Neuroblastoma in a Family With Hereditary Pheochromocytoma Associated With a MAX Deleterious Variant.Frontiers in endocrinology · 2021Article
- A Novel MAX Gene Mutation Variant in a Patient With Multiple and "Composite" Neuroendocrine-Neuroblastic Tumors.Frontiers in endocrinology · 2020Article
- Pheochromocytoma with Synchronous Ipsilateral Adrenal Cortical Adenoma.World journal of surgery · 2017Article
- Composite Pheochromocytoma/Paraganglioma-Ganglioneuroma: A Clinicopathologic Study of Eight Cases with Analysis of Succinate Dehydrogenase.Endocrine pathology · 2017Article
- Mature ganglioneuroma of the adrenal gland as a new rare cause of visible haematuria: A case report & literature review.International journal of surgery case reports · 2015Article
- Composite pheochromocytoma-ganglioneuroma of the adrenal gland: A case report with immunohistochemical study.Urology annals · 2013Article
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Authors and funding
8 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
We analyzed the clinical, hormonal, immunohistochemical and genetic features in a 69-yr-old Caucasian woman with a very rare "composite and mixed pheochromocytoma". This was characterized by right adrenal pheochromocytoma associated with homolateral ganglioneuroma and controlateral adrenal cortical adenoma. The three tumors, incidentally discovered, proved to be non-functioning (normal secretion of catecholamines and of other neuroendocrine peptides, glucocorticoids, mineralcorticoids and androgens). Accordingly, the patient showed no sign or symptom of endocrine disease. Computed tomography (CT) and magnetic resonance (MR) demonstrated a typical adenomatous lesion on the left adrenal gland with precocious uptake of the radiotracer on radioidine (131I)-norcholesterol adrenal scintigraphy, while the controlateral gland showed hyperdensity on CT, hyperintensity on MR and no uptake at adrenal scintigraphy. In addition, CT and MR revealed a vertebral and two hepatic hemangiomas. The right adrenal gland was surgically removed and, microscopically, pheochromocytoma and ganglioneuroma areas appeared intermixed without a predominant component. The former showed strong immunoreactivity for chromogranin, synaptophysin, vascular endothelial growth factor (VEGF) and CD34, while the latter appeared positive for neuron-specific enolase (NSE) and S-100. Peripheral blood genomic DNA analysis revealed a new intronic variant (5557A > G) in the von Hippel-Lindau gene (VHL) not observed in our control population.
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