Pheochromocytoma pathophysiology: Difference between revisions
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* Approximately 10% are familial | * Approximately 10% are familial | ||
* Approximately 10% recur after being resected | * Approximately 10% recur after being resected | ||
* Approximately 10% patients do not have [[hypertension]] (Campbell's Urology) | * Approximately 10% patients do not have [[hypertension]] (Campbell's Urology)These tumors can form a pattern with other endocrine gland cancers which is labeled [[multiple endocrine neoplasia]] (MEN). Pheochromocytoma may occur in patients with MEN 2 and MEN 3. [[VHL]] (Von Hippel Lindau) patients may also develop these tumors. | ||
===Tumor Location=== | ===Tumor Location=== | ||
In adults, 90% tumors are located unilaterally and are solitary, and 10% are located outside the adrenal gland. In children 50% are adrenal, while 25% are bilateral and 25% are extraadrenal. The common extradrenal locations are the abdomen, thorax and urinary bladder. | In adults, 90% tumors are located unilaterally and are solitary, and 10% are located outside the adrenal gland. In children 50% are adrenal, while 25% are bilateral and 25% are extraadrenal. The common extradrenal locations are the abdomen, thorax and urinary bladder. |
Revision as of 14:27, 2 September 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Pathophysiology
Traditionally it is known as the "10% tumor":
- bilateral disease is present in approximately 10% of patients
- approximately 10% of tumours are malignant
- approximately 10% are located in chromaffin tissue outside of the adrenal gland
- Approximately 10% arise in childhood
- Approximately 10% are familial
- Approximately 10% recur after being resected
- Approximately 10% patients do not have hypertension (Campbell's Urology)These tumors can form a pattern with other endocrine gland cancers which is labeled multiple endocrine neoplasia (MEN). Pheochromocytoma may occur in patients with MEN 2 and MEN 3. VHL (Von Hippel Lindau) patients may also develop these tumors.
Tumor Location
In adults, 90% tumors are located unilaterally and are solitary, and 10% are located outside the adrenal gland. In children 50% are adrenal, while 25% are bilateral and 25% are extraadrenal. The common extradrenal locations are the abdomen, thorax and urinary bladder.
Images
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Micrograph of pheochromocytoma.
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Histopathology of adrenal pheochromocytoma. Adrenectomy specimen.
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Micrograph of pheochromocytoma.
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Micrograph of pheochromocytoma.
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Bilateral pheochromocytoma in MEN2. Gross image.