bubble_chart Overview

Pheochromocytoma originates from chromaffin cells. This disease can occur in any location where chromaffin cells are distributed. The most common site is the adrenal gland, but the incidence of extra-adrenal tumors can reach 30%. Tumors may be multiple. Most tumors have a membrane, are well-differentiated, and are considered malignant when metastasis or recurrence occurs in non-chromaffin tissues. The typical age of onset is 6 to 15 years, but it can also be seen in newborns and infants. 9% of cases have a family history.

bubble_chart Diagnosis

(1) Clinical Manifestations

1. Hypertension is the characteristic manifestation of this disease, caused by excessive Black Catechu phenols in the blood circulation. 790% of cases present as persistent hypertension, while a minority are paroxysmal or exhibit persistent hypertension with paroxysmal exacerbations. During episodes, symptoms such as headache, palpitation, profuse sweating, pallor, cold limbs, nausea, vomiting, and abdominal pain are often present. A few cases may show cerebral symptoms, cardiovascular symptoms, and fundus changes due to hypertension.
2. Extra-adrenal tumors may present with corresponding symptoms. For example, when a pheochromocytoma occurs in the bladder, blood pressure may suddenly rise whenever the bladder is full or during urination, accompanied by other symptoms of increased Black Catechu phenols. (2) Laboratory and Auxiliary Examinations 1

. Measurement of 24-hour urinary Black Catechu phenols and their metabolites Elevated levels of 24-hour urinary Black Catechu phenols or VMA (vanillylmandelic acid) are significant for confirming the diagnosis. 1. Tumor localization diagnosis Intravenous pyelography, B-ultrasound, and CT scans can reveal the tumor's location and its relationship with adjacent organs, which is particularly valuable for locating extra-adrenal pheochromocytomas. Chest and skull X-rays are useful for diagnosing mediastinal and intracranial tumors.

bubble_chart Treatment Measures

﹝Treatment﹞

(1) After diagnosis, the tumor should be surgically removed. To reduce surgical mortality, the key points of preoperative and postoperative treatment are as follows:

1. Appropriate use of α and β receptor blockers before surgery to normalize blood pressure and heart rate and restore cardiac function. Start with the α receptor blocker phenoxybenzamine at 1mg/(kg·d), divided into three oral doses. Adjust the dose based on blood pressure to find the maintenance dose that keeps blood pressure near normal, maintain for 4–10 days before surgery, and add β receptor blockers if necessary.
2. During and after surgery, adequate blood transfusion is required to reduce blood pressure drop caused by vascular bed expansion after tumor removal. If blood pressure remains low after transfusion, norepinephrine can be administered. (2) Postoperative follow-up of VMA to detect residual or recurrent tumors early. (3) Symptomatic treatment: Effective antihypertensive drugs can be used during hypertensive episodes, such as slow intravenous infusion of phentolamine, and dehydration agents may be used if necessary.

bubble_chart Differentiation

This disease needs to be differentiated from kidney diseases, aortic stenosis, adrenal medullary hyperplasia, and other conditions with hypertension symptoms.