Pheochromocytoma pathophysiology: Difference between revisions
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{{CMG}} {{AE}} {{AAM}} | {{CMG}} {{AE}} {{AAM}} | ||
==Overview== | ==Overview== | ||
On gross pathology pheochromocytoma appears as multinodular | On gross pathology, pheochromocytoma appears as a multinodular and multicentric pattern of growth. On microscopic histopathological analysis, nesting (Zellballen) pattern composed of well-defined clusters of tumor cells separated by fibrovascular stroma is a characteristic finding. | ||
==Pathophysiology== | ==Pathophysiology== | ||
Mutations of the genes ''[[VHL]]'', ''RET'', ''NF1'', ''[[SDHB]]'', and ''[[SDHD]]'' are all known to cause familial pheochromocytoma. | Mutations of the genes ''[[VHL]]'', ''RET'', ''NF1'', ''[[SDHB]]'', and ''[[SDHD]]'' are all known to cause familial pheochromocytoma. | ||
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 most common extradrenal locations are the abdomen, thorax and urinary bladder. | |||
Traditionally pheochromocytoma known as the "10% tumor": | Traditionally pheochromocytoma known as the "10% tumor": | ||
* Bilateral disease is present in approximately 10% of patients | * Bilateral disease is present in approximately 10% of patients | ||
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Pheochromocytoma can occur in patients with [[multiple endocrine neoplasia]] (MEN 2 and MEN 3), also patients with Von Hippel Lindau ([[VHL]] ) may develop pheocromocytoma. | Pheochromocytoma can occur in patients with [[multiple endocrine neoplasia]] (MEN 2 and MEN 3), also patients with Von Hippel Lindau ([[VHL]] ) may develop pheocromocytoma. | ||
==Gross Pathology== | ==Gross Pathology== | ||
Multinodular | Multinodular and multicentric pattern of growth of pheochromocytoma may be seen. | ||
<gallery> | <gallery> | ||
Image:Bilateral pheo MEN2.jpg|Bilateral pheochromocytoma in [[Multiple_endocrine_neoplasia_type_2|MEN2]]. Gross image. | Image:Bilateral pheo MEN2.jpg|Bilateral pheochromocytoma in [[Multiple_endocrine_neoplasia_type_2|MEN2]]. Gross image. | ||
Revision as of 19:36, 11 September 2015
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]
Overview
On gross pathology, pheochromocytoma appears as a multinodular and multicentric pattern of growth. On microscopic histopathological analysis, nesting (Zellballen) pattern composed of well-defined clusters of tumor cells separated by fibrovascular stroma is a characteristic finding.
Pathophysiology
Mutations of the genes VHL, RET, NF1, SDHB, and SDHD are all known to cause familial pheochromocytoma. 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 most common extradrenal locations are the abdomen, thorax and urinary bladder.
Traditionally pheochromocytoma known as the "10% tumor":
- Bilateral disease is present in approximately 10% of patients
- Approximately 10% of tumors are malignant
- Approximately 10% are located in chromaffin tissue outside of the adrenal gland
- Approximately 10% occur in childhood
- Approximately 10% are familial
- Approximately 10% recur after being resected
- Approximately 10% patients do not have hypertension
Pheochromocytoma can occur in patients with multiple endocrine neoplasia (MEN 2 and MEN 3), also patients with Von Hippel Lindau (VHL ) may develop pheocromocytoma.
Gross Pathology
Multinodular and multicentric pattern of growth of pheochromocytoma may be seen.
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Bilateral pheochromocytoma in MEN2. Gross image.
Microscopic Pathology
Pheochromocytoma typically demonstrate a nesting (Zellballen) pattern on microscopy. This pattern is composed of well-defined clusters of tumor cells containing eosinophilic cytoplasm separated by fibrovascular stroma.
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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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