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Pheochromocytoma

Pheochromocytoma is a rare neoplasm of chromaffin cells that synthesizes and releases catecholamines, leading to surgically correctable hypertension. It is characterized by the 'Rule of 10s,' indicating that 10% are extra-adrenal, bilateral, malignant, or normotensive, with familial cases being more common than previously thought. Diagnosis involves measuring urinary and plasma catecholamines, and treatment typically includes surgical excision along with preoperative medications to manage hypertension.

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10 views4 pages

Pheochromocytoma

Pheochromocytoma is a rare neoplasm of chromaffin cells that synthesizes and releases catecholamines, leading to surgically correctable hypertension. It is characterized by the 'Rule of 10s,' indicating that 10% are extra-adrenal, bilateral, malignant, or normotensive, with familial cases being more common than previously thought. Diagnosis involves measuring urinary and plasma catecholamines, and treatment typically includes surgical excision along with preoperative medications to manage hypertension.

Uploaded by

antoedathinal
Copyright
© © All Rights Reserved
We take content rights seriously. If you suspect this is your content, claim it here.
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Download as PDF, TXT or read online on Scribd
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Pheochromocytoma

Neoplasm of chromaffin cells  Synthesizes & releases catecholamines


(epinephrine, norepinephrine).

Rare but important cause of surgically correctable hypertension.

Rule of 10s
10% extra-adrenal  Occur in organs of Zuckerkandl, carotid body
Paragangliomas).

10% bilateral  Higher in familial cases (up to 50%.

10% malignant  Defined by presence of metastases (higher in extra-


adrenal cases).

10% normotensive  No hypertension despite catecholamine secretion.

Familial cases: Previously thought to be 10%, but now recognized as 25%.

Genetic Associations
Familial cases involve oncogenic germline mutations 25%.

Two major genetic pathways:


1. Enhanced growth factor signaling  RET MEN2, NF1.
2. Pseudohypoxia pathway (↑ HIF1α & HIF2α activity)  VHL, EPAS1,
SDHB, SDHC, SDHD.

VHL syndrome  Mutation in VHL gene → ↑ HIF1α → Tumors like


pheochromocytomas, paragangliomas.

Polycythemia-paraganglioma syndrome  EPAS1 HIF2α) mutation 


Stabilized HIF2α → Increased tumor risk.

Succinate dehydrogenase mutations SDHB, SDHC, SDHD  Disrupt


mitochondrial function & oxygen sensing → ↑ HIF expression
(pseudohypoxia).

Pheochromocytoma 1
Morphology
Size: Ranges from small nodules to large hemorrhagic masses 100g avg,
up to 4kg).

Gross Features:

Smaller tumors  Yellow-tan cut surface.

Larger tumors  Hemorrhagic, necrotic, cystic, effacing adrenal gland.

Potassium dichromate test: Tumor turns dark brown due to


catecholamine oxidation (chromaffin reaction).

Microscopic Features:

Zellballen pattern  Clusters of polygonal/spindle-shaped chromaffin


cells surrounded by sustentacular cells.

Salt-and-pepper chromatin  Characteristic of neuroendocrine tumors.

Catecholamine granules  Visualized by silver stains.

Immunohistochemistry:

Chromogranin & Synaptophysin  Stain chief cells.

S100  Stains sustentacular cells.

Malignancy determination is difficult.

Histologic features (mitoses, necrosis, pleomorphism) are unreliable.

Definitive malignancy diagnosis  Presence of metastases (lymph nodes,


liver, lung, bone).

Clinical Features
Hypertension 90% cases)

Sustained hypertension (majority) with paroxysms in 2/3 cases.

Paroxysmal hypertensive episodes  Sudden BP spikes + tachycardia,


palpitations, headache, sweating, tremor, apprehension.

Triggers for paroxysms:

Emotional stress, exercise, posture changes, tumor palpation.

Urinary bladder paragangliomas  Trigger during micturition.

Pheochromocytoma 2
Cardiac & Systemic Complications

Catecholamine cardiomyopathy  Myocardial damage from excessive


catecholamines.

Severe complications:

Pulmonary edema, myocardial infarction, ventricular fibrillation, stroke.

Possible hormone secretion:

ACTH  Leads to Cushingʼs syndrome.

Somatostatin  Can cause diabetes, gallbladder dysfunction.

Diagnosis

Urinary & Plasma Markers:

↑ Free catecholamines.

↑ Metabolites (vanillylmandelic acid, metanephrines).

Imaging:

CT/MRI  Locates adrenal/extra-adrenal tumors.

MIBG scintigraphy  Detects metastatic or extra-adrenal


pheochromocytomas.

Treatment

Isolated benign tumors  Surgical excision.

Preoperative α-blockers (phenoxybenzamine) & β-blockers to prevent


hypertensive crisis.

Malignant/metastatic cases  Long-term medical management for


hypertension + chemotherapy/radiotherapy.

Summary Table

Feature Pheochromocytoma

Cell of Origin Chromaffin cells (adrenal medulla)

Catecholamines Epi, NE;  Peptide


Hormones Secreted
hormones ACTH, Somatostatin)

Present in 90% 2/3 with paroxysmal


Hypertension
episodes)

Pheochromocytoma 3
Feature Pheochromocytoma

10% extra-adrenal, 10% bilateral, 10%


Rule of 10s
malignant, 10% normotensive

RET, NF1, VHL, EPAS1, SDHB/SDHC/SDHD


Genetics
mutations

Yellow-tan cut surface; Large tumors →


Gross Features
Hemorrhagic, necrotic, cystic

Zellballen pattern, Salt-and-pepper


Microscopic Features
chromatin, Catecholamine granules

Catecholamine cardiomyopathy, MI,


Complications
Pulmonary edema, Stroke

↑ Urinary metanephrines, vanillylmandelic


Diagnosis
acid; CT/MRI, MIBG scan

Treatment Surgical excision ± α-blockers & β-blockers

Pheochromocytoma 4

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