ArticleAACE clinical case reports
Two Cases of Composite Pheochromocytoma-Ganglioneuromas With Plasma Metanephrine Levels in the Subclinical Range Pheochromocytoma-Ganglioneuroma.
Article in AACE clinical case reports. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.
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Who cites it
2 citing papers in PubMed.
- Clinical and biochemical variability in composite pheochromocytomas: a report of 3 cases.JCEM case reports · 2026Article
- Editorial for January/February Issue of AACE Clinical Case Reports.AACE clinical case reportsArticle
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6 authors.
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No grant is acknowledged in the PubMed record.
Abstract
Background/Objective: In <10% of cases, pheochromocytomas coexist with other tumors, most commonly ganglioneuromas, and are termed composite pheochromocytoma-ganglioneuromas. We present 2 cases of composite pheochromocytoma-ganglioneuromas and review the diagnosis and management of these rare tumors. Case Report: Patient 1 and patient 2 were 35-year-old and 45-year-old woman, respectively. Patient 1 presented with a history of controlled hypertension and symptoms of anxiety along with palpitations, diaphoresis, and flushing. Patient 2 complained of abdominal pain and underwent abdominal computed tomography (CT) imaging. Patient 1 and patient 2 had metanephrine levels of 76 pg/mL and 61 pg/mL (normal <57 pg/mL), respectively, and normetanephrine levels of 161 pg/mL and 116 pg/mL (normal < 148 pg/mL), respectively. CT scans depicted right adrenal masses in both cases: patient 1 had a 2.3 × 2.6 cm mass measuring 36 Hounsfield units on noncontrast CT imaging and patient 2 had a 4.5 × 3.5 cm right adrenal mass measuring 73 Hounsfield units on contrast CT imaging. Both patients underwent laparoscopic robotic adrenalectomies without complications. The pathologic analyses of both cases revealed composite pheochromocytoma-ganglioneuroma tumors. Surveillance at 1 year in both patients demonstrated no evidence of recurrence. Discussion: The clinical and radiological presentation of composite pheochromocytoma-ganglioneuromas mirrors pheochromocytomas. The diagnosis relies on histopathological analysis. Treatment of pheochromocytoma-ganglioneuromas is complete surgical excision in a high-volume center with adrenal expertise and is associated with an overall excellent prognosis. The probability of recurrence is low, and distant metastases have rarely been reported. Conclusion: Pheochromocytoma-ganglioneuromas may present with plasma metanephrine levels in the subclinical range. As with isolated pheochromocytomas, lifetime surveillance is critical for composite pheochromocytoma-ganglioneuromas.
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