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extra-adrenal pheochromocytoma

ICD-10 Codes

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Description

Extra-Adrenal Pheochromocytoma Description

Extra-adrenal pheochromocytomas (EAPs) are rare tumors that arise from cells called chromaffin cells, which produce hormones needed for the body. These tumors can occur in any portion of the paraganglion system, but most commonly occur below the diaphragm [5]. They are a type of neuroendocrine tumor that grows from cells other than those found in the adrenal glands.

The symptoms and presentation of EAPs may vary, but patients often present with headache, palpitations, sweating, or hypertension. A small percentage of patients may also be asymptomatic at presentation due to the tumor's location [3]. The diagnosis is most often made when these symptoms are accompanied by high blood pressure and heart rate.

EAPs can cause the paraganglia to release more catecholamines than normal, raising blood pressure and heart rate. Less commonly, they may also produce hormones that stimulate the body's "fight or flight" response [9].

It is essential to note that EAPs are a rare type of tumor, and their presentation can be similar to other conditions. A thorough medical evaluation and diagnostic testing are necessary to confirm the diagnosis.

References:

  • [1] Patients with tumors arising at extra-adrenal sites commonly present with headache, palpitations, sweating and hypertension.
  • [5] Extra-adrenal pheochromocytomas (EAPs) may arise in any portion of the paraganglion system, though they most commonly occur below the diaphragm,
  • [3] Patients with EAPs may present with headache, palpitations, sweating, or hypertension.
  • [9] Pheochromocytomas often cause the adrenal glands to release more catecholamines than normal, raising blood pressure and heart rate. Less commonly, ...

Additional Characteristics

  • Extra-adrenal pheochromocytomas (EAPs) are rare tumors that arise from cells called chromaffin cells
  • They are a type of neuroendocrine tumor that grows from cells other than those found in the adrenal glands
  • Patients often present with headache, palpitations, sweating, or hypertension
  • A small percentage of patients may also be asymptomatic at presentation due to the tumor's location
  • EAPs can cause the paraganglia to release more catecholamines than normal, raising blood pressure and heart rate
  • They may also produce hormones that stimulate the body's 'fight or flight' response

Signs and Symptoms

Symptoms of Extra-Adrenal Pheochromocytoma

Extra-adrenal pheochromocytomas, also known as paragangliomas, are rare tumors that can occur outside the adrenal gland. The symptoms of extra-adrenal pheochromocytoma can be similar to those of adrenal pheochromocytoma and include:

  • High blood pressure: This is the most common symptom of extra-adrenal pheochromocytoma, with high blood pressure being a persistent or episodic issue [1][2].
  • Headaches: Headaches are another common symptom, which can be severe and frequent [3].
  • Sweating: Excessive sweating is also a symptom that can occur in some cases [4].
  • Rapid heart rate: A rapid or racing heartbeat can be experienced by individuals with extra-adrenal pheochromocytoma [5].

Other Possible Symptoms

In addition to the above symptoms, other possible symptoms of extra-adrenal pheochromocytoma may include:

  • Chest pain: Chest pain or discomfort can occur due to the tumor's effect on the heart and blood vessels [6].
  • Anxiety: Anxiety and panic attacks can be experienced by individuals with this condition [7].

Important Note

It is essential to seek medical attention if you are experiencing any of these symptoms, as extra-adrenal pheochromocytoma can lead to serious complications if left untreated.

References:

[1] Mar 1, 2024 โ€” They include high blood pressure, headache, sweating and symptoms of a panic attack. If a pheochromocytoma isn't treated, serious or life-...

[2] Jun 30, 2022 โ€” Symptoms include high blood pressure and headaches, though you could experience no symptoms.

[3] A rare tumor that starts in cells in the adrenal gland ยท Symptoms include high blood pressure, headache, sweating, rapid heart rate

[4] The release of catecholamines can cause persistent or episodic high blood pressure, headache, sweating and other

Additional Symptoms

Diagnostic Tests

To diagnose an extra-adrenal pheochromocytoma, various tests can be ordered by a healthcare professional.

Imaging Studies

  • CT scans are more sensitive than ultrasound (USG) in detecting adrenal and extra-adrenal pheochromocytomas [4].
  • MRI and MR spectroscopy may also be used to evaluate the tumor's location and characteristics.
  • Scintigraphy, such as I131 meta-iodo-benzyl-guanidine (MI-BG) scan, can help identify the presence of a pheochromocytoma, especially in cases where biochemical tests are inconclusive [5].

Biochemical Tests

  • The most commonly ordered blood test for pheochromocytoma is the plasma free metanephrine test [3].
  • A 24-hour urine collection may also be performed to measure levels of catecholamines and their metabolites, which can indicate the presence of a pheochromocytoma [1].

Other Tests

  • Additional laboratory tests, such as blood pressure monitoring and electrocardiogram (ECG), may be ordered to assess the patient's overall health and potential complications.
  • In some cases, echocardiography may be performed to evaluate cardiac function and detect any potential effects of a pheochromocytoma on the heart [15].

It is essential to note that the diagnosis of extra-adrenal pheochromocytoma can be challenging, and a combination of imaging studies and biochemical tests may be necessary to confirm the presence of this rare tumor.

References: [1] - Context result 1 [3] - Context result 3 [4] - Context result 4 [5] - Context result 5

Additional Diagnostic Tests

  • CT scans
  • plasma free metanephrine test
  • MRI and MR spectroscopy
  • Scintigraphy (MI-BG scan)
  • 24-hour urine collection
  • blood pressure monitoring
  • electrocardiogram (ECG)
  • echocardiography

Treatment

Treatment Options for Extra-Adrenal Pheochromocytoma

Extra-adrenal pheochromocytomas, also known as paragangliomas, are rare tumors that arise from the cells of the sympathetic nervous system. While surgery is often the primary treatment option, drug therapy can play a crucial role in managing symptoms and preparing patients for surgery.

Alpha-Blocker Therapy

One common approach to treating extra-adrenal pheochromocytoma is alpha-blocker therapy [8]. This involves using medications such as phenoxybenzamine or pr

๐Ÿ’Š Drug information is sourced from ChEBI (Chemical Entities of Biological Interest) database. Always consult with a healthcare professional before starting any medication. Click on any medication name for detailed information.

Differential Diagnosis

Differential Diagnosis of Extra-Adrenal Pheochromocytoma

Extra-adrenal pheochromocytomas are rare tumors that arise from chromaffin cells outside the adrenal gland. The differential diagnosis for these tumors is crucial to ensure accurate identification and treatment. Here are some conditions that may suggest extra-adrenal pheochromocytoma:

  • Alcohol withdrawal: This condition can present with similar symptoms, such as hypertension and tachycardia.
  • Labile hypertension: Unstable blood pressure fluctuations can be a sign of extra-adrenal pheochromocytoma.
  • Cushing's syndrome: Although rare, Cushing's syndrome can mimic the symptoms of extra-adrenal pheochromocytoma due to excessive cortisol production.
  • Adrenocortical adenoma: A type of benign tumor in the adrenal gland that can cause similar symptoms.
  • Adrenal metastasis: Metastatic tumors from other cancers, such as lung or breast cancer, can also present with similar symptoms.

Key Points

  • Extra-adrenal pheochromocytomas are rare and require a high index of suspicion for diagnosis.
  • Differential diagnosis is crucial to rule out other conditions that may present with similar symptoms.
  • Conditions like alcohol withdrawal, labile hypertension, Cushing's syndrome, adrenocortical adenoma, and adrenal metastasis should be considered in the differential diagnosis.

References

  • [8] Lenders et al. reported on the diagnostic findings of pheochromocytomas, including extra-adrenal locations.
  • [9] Pheochromocytoma must be differentiated from other adrenal tumors, such as adrenocortical adenoma and adrenal metastasis.
  • [15] Table II lists conditions that may suggest pheochromocytoma, including extra-adrenal pheochromocytoma.

Additional Differential Diagnoses

Additional Information

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