Carney Triad (Pentad) and Catecholamine-Secreting Paragangliomas

Carney triad (described in 1977) is a rare, nonfamilial, multitumoral syndrome, with three tumors in the initial description: gastrointestinal stromal tumor (GIST), pulmonary chondroma, and extraadrenal paraganglioma (PGL). , Subsequently, two other tumors, adrenal cortical adenoma and esophageal leiomyoma, were added as components—thus actually Carney triad is a “pentad.” Although it is rare, it is important for endocrinologists to be aware of this disorder because of the links to PGL and adrenocortical tumors.

Case Report

The patient was a 26-year-old woman referred for further management of Carney triad-related neoplasms. The first sign of the triad was at age 17 when she presented with microcytic anemia (hemoglobin 7.0 g/dL) that was treated with iron sulfate. The anemia proved refractory, and esophagogastroduodenoscopy (EGD) performed 1 year before consultation at Mayo Clinic found a large gastrointestinal stromal tumor (GIST), and she underwent an 80% gastrectomy for the removal of a 17-cm GIST. More recently it was noted that she had a mass in her left lingula, which was resected and proved to be a 3.3-cm chondroma. She was also noted to have a 4-cm mass near the aortic arch. She had labile hypertension controlled with a calcium channel blocker (amlodipine 10 mg daily). She had no signs or symptoms of adrenocortical or adrenomedullary hormone excess. On physical examination her body mass index was 28.7 kg/m 2 , blood pressure 138/84 mmHg, and heart rate 96 beats per minute.


Chest magnetic resonance imaging (MRI) showed a 4.1 × 3.3–cm precarinal soft tissue mass ( Fig. 49.1 ). The plasma fractionated metanephrines and 24-hour urine for fractionated metanephrines and catecholamines, while not markedly elevated, were consistent with a catecholamine-secreting tumor ( Table 49.1 ). I-123 metaiodobenzylguanidine (MIBG) scintigraphy colocalized with the precarinal mass and two sites of abnormal uptake were seen in the upper abdomen just to the right and left of midline ( Fig. 49.2 ). MRI of the abdomen demonstrated two retroperitoneal lesions that colocalized with the findings on I-123 MIBG scintigraphy ( Fig. 49.3 ). In addition, the abdominal MRI detected a 2.5-cm right adrenal mass with imaging characteristics consistent with a cortical adenoma ( Fig. 49.4 ). The 1-mg overnight dexamethasone suppression test was normal (see Table 49.1 ). Finally, EGD showed no sign of residual GIST; however, two asymptomatic 1.5–2.0 cm esophageal leiomyomas were found.

Fig. 49.1

Coronal (A) and axial (B) magnetic resonance imaging of the chest demonstrated a 4.1 × 3.3–cm precarinal soft tissue mass (arrows) .

TABLE 49.1

Laboratory Tests

Biochemical Test Result Reference Range
Sodium, mmol/L 137 135–145

Potassium, mmol/L



Creatinine, mg/dL



Plasma metanephrine, nmol/L



Plasma normetanephrine, nmol/L



1-mg overnight DST cortisol, mcg/dL



24-hr urine:

Metanephrine, mcg



Normetanephrine, mcg



Norepinephrine, mcg



Epinephrine, mcg



Dopamine, mcg



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Aug 8, 2022 | Posted by in ENDOCRINOLOGY | Comments Off on Carney Triad (Pentad) and Catecholamine-Secreting Paragangliomas

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