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Pheochromocytoma
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Pheochromocytoma is a tumor of the middle portion of the adrenal gland. The adrenal glands are located atop the kidneys in the back of the abdominal cavity. The adrenal glands make hormones that control heart rate and blood pressure. The pheochromocytoma also makes hormones and this causes symptoms related to heart rate and blood pressure. A pheochromocytoma can be life threatening if not treated properly. Pheochromocytomas are not cancerous and a person can completely recover once the pheochromocytoma is removed.
The particular signs and symptoms that are seen in this disease include rapid heart rate, high blood pressure, forceful heart rate, extreme sweating, pain in the abdomen, severe headaches of sudden onset, paleness, anxiety, and weight loss. The symptoms can be intermittent or relatively chronic in nature before it is discovered. The hormones released are generally epinephrine and norepinephrine, which affect the heart and blood pressure.
Doctors do not understand exactly what causes a pheochromocytoma to occur. They begin in chromaffin cells of the adrenal gland, found in the central portion of the adrenal gland. These are the endocrine glands of the adrenal glands and release hormones that affect the body.
A pheochromocytoma can involve multiple small tumors rather than just one large tumor. The tumor or tumors release catecholamines, either as a steady low dose or as peaks of catecholamine release that can come on as a sudden onset of symptoms. Release of catecholamines can be triggered by stress, anxiety, surgical anesthesia or physical activity that can put pressure on the tumor and trigger release of the catecholamines. Changes in body positioning can trigger catecholamine release, pregnancy can do it, exercise can do it as can having a bowel movement or doing heavy lifting.
Certain things can also trigger hypertension and other symptoms, including using cocaine, decongestants or amphetamines, eating foods that contain tyramine like red wine or aged cheeses, or taking an MAOI inhibitor for depression or other mental illness. Pheochromocytomas can be a hereditary trait in some families. These include diseases like multiple endocrine neoplasia (MEN II), Von Hipple-Lindau disease and neurofibromatosis type I.
While the vast majority of pheochromocytomas are benign and don't spread to other body areas, there does exist a condition of cancerous pheochromocytomas that can travel to the lungs, the brain or the bone.
If you have high blood pressure from a pheochromocytoma, there is damage to end organs that are responsive to hypertension. Heart failure, kidney failure and stroke are all possible. You can get acute respiratory distress syndrome, psychosis, confusion, visual impairment, seizures and early death. You can get a heart arrhythmia and death if the high blood pressure is very high. There is a risk of the following complications:
- Congestive heart failure
- Heart damage
- An increased diabetes risk
Tests for pheochromocytoma include blood tests or urine tests for the amount of epinephrine or norepinephrine noted in these places. A 24 hour urine sample can show an elevated amount of these hormones. A CT scan can show the presence of a tumor in the adrenal gland. An MRI exam can also show the presence of the tumor. It is often found incidentally when scanning for other diseases.
Medications can be used to treat the high blood pressure of a pheochromocytoma. These include alpha blockers, which are blood pressure medications, beta blockers, which block the effects of the hormone on the heart and lower blood pressure.
Ultimately, surgery is used to remove the affected adrenal gland. This almost always completely resolves the symptoms. In some cases, a laparoscopic surgery can be done to remove the adrenal gland and the recovery time is considered much less than with an open procedure. In rare cases, surgery is not possible because it is a metastasized malignant pheochromocytoma.