Pheochromocytoma, a challenging medical puzzle. This rare tumour originates in the adrenal medulla or the parachromaffin cells of the sympathetic and parasympathetic nerves outside the adrenal glands, and its abnormal growth can lead to an imbalance in hormone levels in the body, triggering high blood pressure, heart palpitations, and other serious symptoms. In the medical world, such cases are often noticeable, and they can be a difficult test for both doctors and patients. However, it is precisely these challenges that have inspired the medical community to make unremitting efforts in the research and development of rare diseases. Next, Luo Huarong, attending physician of the Department of Urology of Tongji Hospital affiliated to Tongji University, unveiled the mystery of the huge suprarenal pheochromocytoma.
Mr. Wang, 35 years old, was found to have an elevated blood pressure of up to 190 100 mmHg due to a headache a year ago, but he did not accept the regularity**, and was found to have a substantial mass in the right adrenal gland area at a local hospital. In the past year, Mr. Wang had repeated intermittent headaches, and by chance he went to Tongji Hospital affiliated to Tongji University for treatment. The results found that Mr. Wang's whole body was highly pigmented, with multiple subcutaneous nodules, the largest of which was about 4cm, with a tough texture and a good range of motion. Synchronous recording of ambulatory ECG blood pressure suggests sinus tachycardia, large changes in circadian rhythm of blood pressure, increased blood pressure at part of the time, and a small amount of tricuspid regurgitation on cardiac ultrasound.
Laboratory tests show elevated levels of epinephrine and norepinephrine. Abdominal ultrasound showed that Mr. Wang had a slightly hyperechoic area in the right adrenal gland area, which was about 103cm*7.7 cm, the boundary is still clear, the internal echo is still even, and no obvious blood flow signal is observed. CT spiral scan showed a round-like mass shadow in the right adrenal gland area, with clear borders, uneven density, and a size of about 108cm*6.7 cm, adjacent to the liver, right kidney and vena cava compression, small vessel shadows can be seen in the posterior arterial phase, and the portal phase and delayed phase are further strengthened.
MRI showed a large mass shadow in the right adrenal gland area, with clear borders, showing a slightly longer T1 and slightly longer T2 signal, and the DWI showed obvious hyperintensity.
Based on the results of these examinations, Mr. Wang was diagnosed with a giant pheochromocytoma of the right adrenal gland and multiple neurofibromatosis, and underwent a resection of the giant pheochromocytoma of the right adrenal gland after oral doxazosin controlled-release tablets and metoprolol to control his blood pressure and heart rate for 3 weeks.
What is a pheochromocytoma?
Pheochromocytoma originates from pheochromocytoma. During the embryonic stage, the distribution of chromaffin cells is related to the sympathetic ganglia of the body. As the embryo matures, the vast majority of chromaffin cells degenerate, and their remnants form the adrenal medulla. As a result, the vast majority of pheochromocytomas occur in the adrenal medulla. Extraadrenal pheochromocytomas can occur anywhere from the carotid body to the pelvis, but are predominantly found in the paraspinal sympathetic ganglia (predominantly postmediastinum) and the abdomen mainly in the para-aortic apparatus at the bifurcation. Pheochromocytomas account for about 0 percent of hypertension**5-1%。More than 90% of patients are operational**.
There is no clear and uniform standard for giant pheochromocytoma. In the past, giant pheochromocytoma was defined as a tumor mass of more than 500 g, but pheochromocytoma was large in size, and the tumor was often accompanied by hemorrhage, necrosis and liquefaction, so the size of the tumor was not necessarily proportional to its weight, so a giant pheochromocytoma was defined as a tumor diameter greater than 10 cm. Giant pheochromocytoma cardiovascular and cerebrovascular complications such as catecholamine heart disease, arrhythmia, stroke, TIA proportion is high, and there is a risk of causing emergencies and accidents during the attack, which is more harmful, and some of them are malignant pheochromocytomas, so they should be diagnosed and treated as soon as possible.
What are the clinical manifestations of pheochromocytoma?
1.Typical symptoms: including the triad of "headache, palpitations, and excessive sweating", which occurs in more than 50% of cases.
2.Hypertension is the most common clinical symptom, occurring in about 0% to 90% of patients. 50% to 60% are persistent and 40% to 50% are paroxysmal.
3.Orthostatic hypotension, which occurs in 10% to 50% of patients, is due to hypovolemia.
4.Cardiovascular complications, about 12% of patients present with cardiovascular complications for the first time, especially those with large tumors.
5.Other symptoms: Some patients may be accompanied by leukocytosis, polycythemia;Some patients may present with cardiomyopathy, hypercalcemia, hematuria, diabetes, Cushing's syndrome, intestinal obstruction, and even vision loss.
Pheochromocytoma is called "10% tumor", and 10% can be malignant: more common in women, more common in adrenal glands, with a 5-year survival rate of about 36%-60%;10% were bilateral polymorphic;More common in familial disorders;10% of cases occur outside the adrenal glands and are called PGLs, most commonly in paraaortic chromaffin. 10% are child-onset: more common in families.
Pheochromocytoma**
Pheochromocytoma is completely surgically removed. Depending on the ability and experience of surgeons and anesthesiologists, the survival rate is 98%-100%. Laparoscopic tumor resection has become the standard procedure for most adrenal tumors** and is also suitable for the management of pheochromocytoma. Surgery**Laparoscopic or open surgery to remove the tumor is associated with good results. When the tumor is large, laparoscopic surgery can be performed through the abdominal approach, and when the tumor is small, the peritoneal ** approach can also be used, and when the tumor is large, open surgery is safer.
Due to the increase in catecholamines in the blood of adrenal pheochromocytoma patients, the peripheral blood vessels are in a state of constriction for a long time, the blood volume is relatively low, the catecholamine content decreases after tumor resection, and the vasodilation leads to a sharp drop in blood pressure, and hypovolemic shock that is difficult to correct during and after surgery, and even life-threatening. To this end, perioperative management should be strengthened, including adequate preoperative preparation, meticulous intraoperative manipulation, and close postoperative monitoring.
With the improvement of the understanding of pheochromocytoma, the diagnosis and treatment of typical adrenal pheochromocytoma has gradually been mastered by the majority of clinicians. However, for giant pheochromocytoma, there is room for further improvement in preoperative diagnosis, preoperative preparation, surgical selection, and follow-up. Giant pheochromocytoma tumors are huge, often involving the liver and vena cava, difficult to expose, and at the same time, there is a certain proportion of malignant possibility, and it is difficult to operate, so open surgery is a safe and effective surgical method.
Author: Luo Huarong, Tongji Hospital, Tongji University.
Editor: Jiani Lu.
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