Narayanaswamy Gopinath, Sarma Debanga, Saikia Uma Kaimal, Baro Abhamoni, Bhuyan Ashok Krishna
Department of Endocrinology, Gauhati Medical College and Hospital, Guwahati, India.
J ASEAN Fed Endocr Soc. 2024;39(2):48-53. doi: 10.15605/jafes.039.02.07. Epub 2024 Aug 5.
Pheochromocytomas are catecholamine-secreting tumors arising from chromaffin cells of the adrenal gland. Surgery is the only curative treatment with a high biochemical cure rate, low mortality and high risk of perioperative complications.
To study the demographic characteristics of patients with pheochromocytoma and to identify the risk factors for perioperative complications, treatment outcomes, and aggressive behavior of the tumor.
We retrospectively studied the data of pheochromocytoma patients registered from 2012 to 2022.
In our study, a total of 30 patients with pheochromocytoma were included. The mean age of presentation was 35 ± 12.8 years. Fifty-six percent were females, and the sex ratio was 1.3:1. Pheochromocytoma spells (60%) was the most common complaint, followed by abdominal pain (53%), orthostatic complaints (10%) and incidentalomas (6%). The baseline mean 24-hour urinary total metanephrines was 2963.7 ± 2658 mcg/24 hours, and the mean tumor size was 7.3 ± 0.53 cm. Forty-three percent of patients underwent laparoscopic adrenalectomy, while the rest underwent open surgery. The mean Pheochromocytoma of Adrenal gland Scaled Score(PASS) was 3.41 ± 0.28, and 23% had a high risk for malignancy. Among perioperative complications, hypertensive crisis (17%) was the most common, followed by postoperative hypotension (13%), hypoglycemia (3%) and right-sided pneumothorax (3%). These patients with complications had higher metanephrine levels (5490 vs. 1880 mcg/24 hours, = 0.001). Blood pressure normalized in 50%, and this was associated with male sex, younger age (29.5 vs. 40 years, = 0.03), higher metanephrines (4619 vs. 1855 mcg/24 hours, = 0.001) and smaller tumors (5.91 vs. 8.61 cm, = 0.046). PASS score greater than or equal to 4 was associated with higher metanephrine levels (5104 vs. 2312 mcg/24 hours, = 0.021) and larger tumors (9.28 vs. 6.68 cm, = 0.024). Biochemical cure rate was achieved in 76% of patients after surgery and was associated with older age (37.7 years vs. 27.7 years, = 0.047) and absence of pheochromocytoma spells (100% vs. 61%, = 0.014).
Young age, smaller tumor size and higher metanephrine concentrations were associated with normalization of blood pressure post-surgery. On the other hand, older patients and those without pheochromocytoma spells had better biochemical cure rates. Patients with higher baseline metanephrine levels had increased perioperative complications. More aggressive tumor behavior was associated with higher metanephrine levels and larger tumors. Sex, baseline blood pressure and mode of surgery did not have any influence on treatment outcomes.
嗜铬细胞瘤是起源于肾上腺嗜铬细胞的分泌儿茶酚胺的肿瘤。手术是唯一的根治性治疗方法，生化治愈率高、死亡率低，但围手术期并发症风险高。
研究嗜铬细胞瘤患者的人口统计学特征，确定围手术期并发症的危险因素、治疗结果以及肿瘤的侵袭性。
我们回顾性研究了2012年至2022年登记的嗜铬细胞瘤患者的数据。
在我们的研究中，共纳入30例嗜铬细胞瘤患者。就诊时的平均年龄为35±12.8岁。56%为女性，性别比为1.3:1。嗜铬细胞瘤发作（60%）是最常见的主诉，其次是腹痛（53%）、体位性不适（10%）和偶发瘤（6%）。基线24小时尿总甲氧基肾上腺素平均为2963.7±2658微克/24小时，平均肿瘤大小为7.3±0.53厘米。43%的患者接受了腹腔镜肾上腺切除术，其余患者接受了开放手术。肾上腺嗜铬细胞瘤量表评分（PASS）平均为3.41±0.28，23%有恶性高风险。在围手术期并发症中，高血压危象（17%）最常见，其次是术后低血压（13%）、低血糖（3%）和右侧气胸（3%）。这些有并发症的患者甲氧基肾上腺素水平较高（5490对1880微克/24小时，P = 0.001）。50%的患者血压恢复正常，这与男性、年龄较小（29.5对40岁，P = 0.03）、较高的甲氧基肾上腺素水平（4619对1855微克/24小时，P = 0.001）和较小的肿瘤（5.91对8.61厘米，P = 0.046）有关。PASS评分大于或等于4与较高的甲氧基肾上腺素水平（5104对2312微克/24小时，P = 0.021）和较大的肿瘤（9.28对6.68厘米，P = 0.024）有关。术后76%的患者实现了生化治愈，这与年龄较大（37.7岁对27.7岁，P = 0.047）和无嗜铬细胞瘤发作（100%对61%，P = 0.014）有关。
年龄较小、肿瘤较小和甲氧基肾上腺素浓度较高与术后血压恢复正常有关。另一方面，年龄较大的患者和无嗜铬细胞瘤发作的患者生化治愈率更高。基线甲氧基肾上腺素水平较高的患者围手术期并发症增加。更具侵袭性的肿瘤行为与较高的甲氧基肾上腺素水平和较大的肿瘤有关。性别、基线血压和手术方式对治疗结果没有任何影响。
