Pathology definition - Pheochromocytoma
Pheochromocytoma
Pheochromocytoma may present with the rule of 10s. In this rules 10% are familial, 10 % are malignant, 10% are bilateral, 10% in children and 10% extra adrenal ( which is known as paragangliomas.)
Pheochromocytoma may present with changes in the adrenal medulla. There will be small,large or hemorrhagic circumscribed lesions and lobular pattern. Besides the lesion/tumor may composed of nests of polygonal chromaffin cells. These contains pleomorphic cells and catecholamine rich granules.
Pheochromocytoma may present with intermittent attacks of headache, palpitations , hypertension and diaphoresis due to the release of the epinephrine and norepinephrine.
The treatment may focus on alpha adrenergic blocker ( phenoxybenzamine). Pheochromocytoma may associate with MEN 11a, MEN 11b von Hippel - Lindau syndrome and neurofibromatosis.
There will be increased in the urinary catecholamine and plasma catecholamine levels. There will also be an increased in the metanephrine level and 24 hour urinary VMA.
Pheochromocytoma may present with the rule of 10s. In this rules 10% are familial, 10 % are malignant, 10% are bilateral, 10% in children and 10% extra adrenal ( which is known as paragangliomas.)
Pheochromocytoma may present with changes in the adrenal medulla. There will be small,large or hemorrhagic circumscribed lesions and lobular pattern. Besides the lesion/tumor may composed of nests of polygonal chromaffin cells. These contains pleomorphic cells and catecholamine rich granules.
Pheochromocytoma may present with intermittent attacks of headache, palpitations , hypertension and diaphoresis due to the release of the epinephrine and norepinephrine.
The treatment may focus on alpha adrenergic blocker ( phenoxybenzamine). Pheochromocytoma may associate with MEN 11a, MEN 11b von Hippel - Lindau syndrome and neurofibromatosis.
There will be increased in the urinary catecholamine and plasma catecholamine levels. There will also be an increased in the metanephrine level and 24 hour urinary VMA.
