Table 1.
Presentation and management of patients with cystic pheochromocytoma
| N=21 | |
|---|---|
| Clinical presentation | |
|
| |
| Age at diagnosis, years, median (IQR) | 57 (51–67) |
|
| |
| Women, n (%) | 12 (57) |
|
| |
| Race, n (%) | |
| White | 18 (86) |
| Native American | 1 (5) |
| Unknown | 2 (10) |
|
| |
| Mode of discovery, n (%) | |
| Incidental | 13 (57) |
| Hormone excess symptoms | 5 (24) |
| Mass effect symptoms | 2 (10) |
| Genetic surveillance | 1 (5) |
| Post-adrenalectomy | 1 (5) |
|
| |
| Symptoms and signs of catecholamine excess, n (%) | 13 (62) |
|
| |
| Symptoms and signs, n (%) | |
| Palpitations | 8 (62) |
| Spells | 8 (62) |
| Paroxysmal hypertension | 7 (54) |
| Sustained hypertension | 2 (15) |
|
| |
| Genetic testing, n (%) | |
| Not tested | 15 (72) |
| Tested, negative | 3 (14) |
| Tested, positive | 3 (14) |
|
| |
| Type of pathogenic variant, n (%) | |
| VHL | 1 (33) |
| NF1 | 1 (33) |
| MEN 2A | 1 (33) |
|
| |
| Extra-adrenal malignancy, n (%) | 7 (33) |
|
| |
| Imaging phenotype | |
|
| |
| Site, n (%) | |
| Right | 15 (71) |
| Left | 6 (29) |
|
| |
| Tumor size, cm, median (IQR) | 6.4 (5–11.1) |
|
| |
| Volume, cm3, median (IQR) | |
| Total | 115 (47.4–654.1) |
| Cystic component | 48.7 (15.6–236.3) |
| Solid component | 61.2 (27.3–186.2) |
|
| |
| Composition, %, median (IQR) | |
| Cystic component | 40 (24–60) |
| Solid component | 60 (40–76) |
|
| |
| Unenhanced CT attenuation, HU, median (IQR), available for n=16 | |
| Cystic component | 17.6 (14.6–20) |
| Solid component | 40 (32.1–44.3) |
|
| |
| Enhanced CT attenuation#, HU, median (IQR), available for n=14 | |
| Cystic component | 20.4 (14.9–26.8) |
| Solid component | 83 (83.3–121) |
|
| |
| Other imaging characteristics | |
| Round/oval shape, n (%) | 19 (90) |
| Well defined borders, n (%) | 21 (100) |
| Heterogeneous, n (%) | 18 (86) |
| Rim thickness, mm, median (IQR) | 14 (8–22) |
| Calcification, n (%) available for n=20 | 3 (15) |
|
| |
| Biochemical evaluation | |
|
| |
| Preoperative evaluation for catecholamine excess, n (%) | 20 (95) |
|
| |
| Plasma fractionated metanephrines, nmol/L, median (IQR), available for n=18 | |
| Metanephrine (normal <0.5 nmol/L) | 2.7 (0.9–8.7) |
| Normetanephrine (normal <0.9 nmol/L) | 13.2 (3.2–23.6) |
|
| |
| Urinary fractionated metanephrine, nmol/day, median (IQR), available for n=16 | |
| Metanephrine (normal < 2028 nmol/day) | 15828 (5663–33731) |
| Normetanephrine (normal < 4913 nmol/day) | 22957 (9206–84472) |
|
| |
| Urinary fractionated catecholamines, nmol/day, median (IQR), available for n=15 | |
| Epinephrine (normal < 115 nmol/day) | 470 (104–1715) |
| Norepinephrine (normal <473 nmol/day) | 1093 (609–6461) |
| Dopamine (normal <2611 nmol/day) | 1222 (975–2126) |
|
| |
| Tumor type, n (%) | |
| Adrenergic | 16 (80) |
| Noradrenergic | 4 (20) |
|
| |
| Perioperative details | |
|
| |
| Preoperative medical therapy, n (%) | 20 (95) |
|
| |
| Alpha-adrenergic blockade | |
| Number of patients treated, n (%) | 20 (100) |
| Duration, days, median (IQR) | 15.5 (10.3–20) |
|
| |
| Metyrosine | |
| Number of patients treated, n (%) | 4 (20) |
| Duration, days, median (IQR) | 5 (2.8–8) |
|
| |
| Beta-adrenergic blockade, n (%) | 17 (85) |
|
| |
| Adrenalectomy, n (%) | |
| Laparoscopic | 10 (48) |
| Open | 11 (52) |
|
| |
| Duration of hospital stay, days, median (IQR) | 4 (2–6.5) |
|
| |
| Postoperative complications, n (%) | |
| Hypotension requiring vasopressors | 3 (14%) |
| Pulmonary edema | 1 (5%) |
| Retroperitoneal hemorrhage | 1 (5%) |
| Postoperative ileus | 1 (5%) |
Abbreviations – CT: Computed Tomography, HU: Hounsfield Unit, IQR: Interquartile range, MEN 2A: Multiple endocrine neoplasia type 2A, NF1: Neurofibromatosis type 1, VHL: Von Hippel Lindau
#
Measured during portal venous phase