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Pheochromocytoma

Maya Campara, PharmD, BCPS and Anna C. Porter, MD Reviewed 06/2018
 


BASICS

DESCRIPTION

  • A pheochromocytoma is a rare neuroendocrine tumor arising from the adrenal or extra-adrenal chromaffin tissue and, less commonly, the sympathetic ganglia.

  • These tumors are catecholami...

DIAGNOSIS

HISTORY

  • Classic “triad” of symptoms includes headache, palpitations, and diaphoresis.

  • 5 Ps mnemonic

    • Paroxysmal spells

    • Pressure: sudden increase in BP

    • Pain: headache, chest, abdominal pain

    • Perspira...

TREATMENT

MEDICATION

First Line

Surgical resection of the tumor is the treatment of choice. Medical management of BP is essential prior to surgery. 
  • Preoperative control of BP (target <120/80 mm Hg) a...

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

  • Monitor BP daily before surgery.

  • Invasive hemodynamic monitoring intraoperatively

  • Monitor urine metanephrine and catecholamine 2 weeks postoperati...

REFERENCES

1
Karagiannis A, Mikhailidis DP, Athyros VG, et al. Pheochromocytoma: an update on genetics and management. Endocr Relat Cancer.  2007;14(4):935–956. [View Abstract on OvidMedline]
2
Bruy...

SEE ALSO

Hypertension, Essential; Multiple Endocrine Neoplasia (MEN) Syndromes 

CODES

ICD10

  • D35.00 Benign neoplasm of unspecified adrenal gland

  • D35.02 Benign neoplasm of left adrenal gland

  • D35.01 Benign neoplasm of right adrenal gland

  • C74.90 Malignant neoplasm of unsp part of unspeci...

CLINICAL PEARLS

  • Rare catecholamine-secreting tumor with serious and potentially lethal cardiovascular complications

  • Clinical presentation is variable, but classic triad consists of episodic headaches, ...

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