Pheochromocytoma

WHAT IS IT? 

Pheochromocytoma are adrenal tumors of the medulla that arise from chromaffin cells in origin (originally these cells developed form he neural crest).

Gross pathology showing a pheochromocytoma in the adrenal medulla (source)
Gross pathology showing a pheochromocytoma in the adrenal medulla (source)
WHY IS IT A PROBLEM?

Most of these tumor types secrete epinephrine, norepinephrine, and dopamine. This pattern of hormone secretion can cause episodic hypertension.

WHAT MAKES US SUSPECT IT?

Risk factors: neurofibromatosis type 1, von Hippel-Lindau disease, MEN2A and MEN2B

*Symptoms occur in “episodes” marked by spells of relapse and remission:

  • Hypertension
  • Headache
  • Perspiration
  • Palpitations (tachycardia)
  • Pallor (from alpha vasoconstriction)
HOW DO WE CONFIRM A DIAGNOSIS?

Urine analysis will reveal increased catecholamines and metanephrines. Vanillylmandelic acid (VMA) can also be present in the urine.

Serum analysis will also reveal increased catecholamines and metanephrines. Chromogranin A 

Imaging: can help localize the tumor.

Source
Source

Histology will demonstrate enlarged pleomorphic nuclei seen in chromaffin cells.

Pleimorphic chroffafin cell nuclei seen on histological analysis of pheochromocytoma (source)
Pleimorphic chroffafin cell nuclei seen on histological analysis of pheochromocytoma (source)
HOW DO WE TREAT IT? 

Irreversible α-antagonists (phenoxybenzamine) followed by β-blockers: alpha blockade must be achieved first in order to avoid hyerptensive crisis during surgery. These medications are used before surgery in the event that catecholamines leak into blood stream upon manipulating the tumor.

Surgical excision of the tumor is the ultimate treatment.

HOW WELL DO THE PATIENTS DO?

The tumors are usually benign and are not metastatic. Excision of the tumor is curative.

WAS THERE A WAY TO PREVENT IT? 

This condition does not have a clear means of prevention.

WHAT ELSE ARE WE WORRIED ABOUT?

Neurofibromatosis type 1 can cause pheochromocytomas

Von Hippel-Lindau disease can also cause pheochromocytomas

OTHER HY FACTS?

These are the most common tumors of the adrenal medulla in adults.

Metanephrines are metabolites of catecolimines.

Erythropoietin can also be secreted by pheochromocytomas

Rule of 10’s:

  • 10% are malignant
  • 10% bilateral
  • 10% extra-adreanl
  • 10% calcify
  • 10% of patients are children
ARCHIVE OF STANDARDIZED EXAM QUESTIONS 

This archive compiles standardized exam questions that relate to this topic.

FURTHER READING

DynaMed

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Page Updated: 02.20.2016