Pheochromocytoma - Symptoms, Causes, Images, and Treatment Options

Diseases

Highlights & Basics

Key Highlights

Pheochromocytoma is a rare neuroendocrine tumor of the adrenal glands. It can present with palpitations, diaphoresis, pallor, and paroxysmal hypertension.
Risk factors include multiple endocrine neoplasia type 2, Von Hippel-Lindau syndrome, and neurofibromatosis type 1.
Diagnosed by increased levels of metanephrines and normetanephrines.
Treatment includes medical therapy for hypertension and surgical excision of tumor (open or laparoscopic adrenalectomy). For unresectable tumors, alternative approaches include chemotherapy or radiopharmaceuticals.
Complications include hypertensive crisis, myocardial infarction, and hypotension.

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History & Exam
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Definition

Epidemiology

Etiology

Pathophysiology

Images

Abdominal CT scan with mass in the left adrenal gland, compatible with a pheochromocytoma
Metaiodobenzylguanidine (MIBG) scintigraphy identified hyperfixation in the left adrenal gland compatible with pheochromocytoma
Left laparoscopic adrenalectomy: (A) macroscopic examination, 6 cm tumor; (B) microscopic examination: neoplasm of the adrenal medulla with eosinophilic cytoplasm of large cells with positive fine granular chromogranin A; (C) round and oval nucleus and sustentacular cells S100+; (D) pheochromocytoma

Citations

Key Articles

Neumann HPH, Young WF Jr, Eng C. Pheochromocytoma and paraganglioma. N Engl J Med. 2019 Aug 8;381(6):552-65.[Abstract]
Lenders JW, Duh QY, Eisenhofer G, et al; Endocrine Society. Pheochromocytoma and paraganglioma: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2014 Jun;99(6):1915-42.[Abstract][Full Text]
Martucci VL, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014 Jan-Feb;38(1):7-41.[Abstract][Full Text]
Plouin PF, Amar L, Dekkers OM, et al; Guideline Working Group. European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma. Eur J Endocrinol. 2016 May;174(5):G1-10.[Abstract][Full Text]
Kunz PL, Reidy-Lagunes D, Anthony LB, et al; North American Neuroendocrine Tumor Society. Consensus guidelines for the management and treatment of neuroendocrine tumors. Pancreas. 2013 May;42(4):557-77.[Abstract][Full Text]
Pang Y, Liu Y, Pacak K, et al. Pheochromocytomas and paragangliomas: from genetic diversity to targeted therapies. Cancers (Basel). 2019 Mar 28;11(4).[Abstract]

Referenced Articles

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