Huge Catecholamine-Secreting Tumor





The average size of a symptomatic catecholamine-secreting tumor is 4.5 cm. Some of the very large pheochromocytomas or paragangliomas (PPGLs) (e.g., >10 cm in diameter) are biochemically nonfunctioning. However, there is a unique subset of patients with PPGL who are asymptomatic despite large tumors and massive catecholamine hypersecretion. We describe one such case here.


Case Report


The patient was a 47-year-old man who was incidentally discovered to have a large right adrenal pheochromocytoma during an elective partial sigmoidectomy to remove large benign colon polyps. There was no problem with blood pressure lability intraoperatively. After his partial sigmoidectomy, abdominal magnetic resonance imaging showed a massive right retroperitoneal tumor (20 × 10 × 12 cm) ( Fig. 37.1 ). He always had normal blood pressure and denied paroxysms, palpitations, headaches, or sweating. There is no family history of pheochromocytoma. On physical examination his body mass index was 25.9 kg/m 2 , blood pressure 108/65 mmHg, and heart rate 87 beats per minute.




Fig. 37.1


Axial (A) and sagittal (B) images from abdominal magnetic resonance imaging revealed huge pheochromocytoma measuring 20 × 10 × 12 cm (longitudinal, transverse, and AP diameters, respectively). The tumor had numerous cystic and necrotic areas throughout and displaced the right kidney inferiorly and anteriorly.


INVESTIGATIONS


Blood and 24-hour urine studies provided unequivocal proof that this was a catecholamine-secreting tumor ( Table 37.1 ). The 24-hour urinary excretion of dopamine, normetanephrine, and metanephrine was increased 124-fold, 7-fold, and 5-fold above the upper limits of the respective reference ranges.



TABLE 37.1

Laboratory Tests



















































Biochemical Test Result Reference Range
Sodium, mmol/L 143 135–145

Potassium, mmol/L

4.4

3.6–5.2

Creatinine, mg/dL

1.0

0.6–1.1

Plasma metanephrine, nmol/L

1.9

<0.5

Plasma normetanephrine, nmol/L

15.0

<0.9

24-Hour urine:

Metanephrine, mcg

2155

<400

Normetanephrine, mcg

6361

<900

Norepinephrine, mcg

374

<80

Epinephrine, mcg

1.7

<20

Dopamine, mcg

49,662

<400

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Aug 8, 2022 | Posted by in ENDOCRINOLOGY | Comments Off on Huge Catecholamine-Secreting Tumor

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