嗜铬细胞瘤患者的特点及预后

Nadeema Rafiq, Tauseef Nabi, S. Dar, Shahnawaz Rasool
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摘要

目的:嗜铬细胞瘤是一种罕见的神经内分泌肿瘤,临床表现多变,包括高血压,通常起源于肾上腺髓质。本研究的目的是评估嗜铬细胞瘤患者的临床特征和治疗结果。方法:这是一项单中心回顾性观察性研究,旨在研究14例诊断为嗜铬细胞瘤的患者的临床、实验室、放射学和手术结果。结果:14例患者中,女性10例,男性4例,确诊时平均年龄38±16岁。头痛(96.6%)、心悸(64.3%)、腹痛(64.3%)和出汗(57.1%)是最常见的症状,而三联征(42.8%)存在。高血压是主要的临床表现(92.8%),其次是站立不稳(35.7%)和高血糖(35.7%)。嗜铬细胞瘤多为散发性(85.7%)、肾上腺肿瘤(78.6%)和良性(92.8%);2例为家族性(均为神经纤维瘤型和von Hippel-Lindau)。58.3%的患者有肾上腺素和去肾上腺素分泌模式,41.7%的患者有肾上腺素分泌模式。超过一半的肿瘤(54.5%)位于左侧,36.4%位于右侧,1例患者双侧表现为肿瘤。35.7%的高血压患者术后高血压缓解。生化治愈率为85.7%,手术治愈率为78.5%。患者生存时间从2个月到9年不等。结论:本研究证实嗜铬细胞瘤的临床表现是可变的和非特异性的。虽然嗜铬细胞瘤是一种罕见的肿瘤,但正确的评估、术前准备和完全的手术切除对其治疗至关重要。
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Characteristics and outcome of patients with pheochromocytoma
Aim: Pheochromocytomas are rare neuroendocrine tumors with variable clinical presentation, including hypertension, and usually arise from the adrenal medulla. The aim of the current study was to evaluate the clinical characteristics and therapeutic outcomes of patients with pheochromocytoma. Methods: This was a single-center retrospective observational study designed to study the clinical, laboratory, radiological, and surgical outcomes of 14 patients diagnosed with pheochromocytoma. Results: Of 14 patients, 10 were females and 4 males, with a mean age of 38 ± 16 years at diagnosis. Headache (96.6%), palpitation (64.3%), abdominal pain (64.3%), and sweating (57.1%) were the most common presenting symptoms, while triad was present in 42.8%. Hypertension was the predominant clinical finding (92.8%) followed by orthostasis in 35.7% and hyperglycemia in 35.7%. Most pheochromocytomas were sporadic (85.7%), adrenal gland tumors (78.6%), and benign (92.8%); two were familial (each due to neurofibromatosis type and von Hippel–Lindau). Metanephrine and nor-metanephrine secretory pattern was seen in 58.3% of the patients, while metanephrine only was seen in 41.7% of the patients. More than half of the tumors (54.5%) were located on the left side, with 36.4% on the right side, and one patient had bilateral presentation of a tumor. Postsurgical remission of hypertension was found in 35.7% of the hypertensive patients. Biochemical cure was obtained in 85.7% and surgical cure rate in 78.5% of the patients. Patient survival ranged from 2 months to 9 years. Conclusions: The present study confirms that the clinical presentation of pheochromocytoma is variable and nonspecific. Although pheochromocytoma is a rare tumor, proper evaluation, preoperative preparation, and complete surgical excision are important for its management.
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