ADRENAL TUMORS [131 durga T]

ADRENAL GLAND TUMORS

Adrenal tumors are cancerous or noncancerous growths on the adrenal glands



 The adrenal glands are located above the kidneys and have two parts, the adrenal cortex and the adrenal medulla. The adrenal cortex secretes hormones including cortisol and aldosterone. The adrenal medulla produces dopamine, epinephrine and norepinephrine. There are different types of adrenal tumors, and they can be either cancerous or noncancerous (benign).

Aside from categorizing them as noncancerous (benign) or cancerous (malignant), they are categorized as functioning or nonfunctioning. Functioning tumors lead to overproduction of one or more of the normal adrenal hormones.

CAUSES:

The cause of most adrenal tumors is unknown. Some hereditary conditions have been linked to an increased risk of developing an adrenal tumor. The hereditary conditions include:

MAJOR PROBLEMS:

Adrenal tumors can cause several problems. These problems can include:

  • Cushing’s Syndrome: This condition occurs when the tumor leads to excessive secretion of cortisol. While most cases of Cushing’s Syndrome are caused by tumors in the pituitary gland in the brain, some happen because of adrenal tumors. Symptoms of this disorder include diabetes, high blood pressure, obesity and sexual dysfunction.
  • Conn’s Disease: This condition involves excessive secretion of aldosterone. Symptoms include personality changes, excessive urination, high blood pressure, constipation and weakness.
  • Pheochromocytoma: This condition causes excessive secretion of adrenaline and noradrenaline. Symptoms include sweating, high blood pressure, headache, anxiety, weakness and weight loss.



WHO CLASSIFICATION OF ADRENAL TUMORS




SYMPTOMS:

Several signs and symptoms may be seen, including:

Clinical images

Images hosted on other servers:

Moon facies, acne, bitemporal excess hair

 

Moon facies and central obesity

 

Facial and chest hair, absence of breasts

 

Back acne

 

Penile enlargement

 

Clitoromegaly and pubic hair

Gross description
  • Weight usually < 50 grams (in pediatric patients may weight up to 500 grams) 
  • Size usually < 5 cm
  • Unilateral, solitary, golden yellow
  • May have focal dark areas corresponding with hemorrhage, lipid depletion, increased lipofuscin
  • Functional adenoma may result in atrophy of ipsilateral or contralateral adrenal cortex

adrenal cortical carcinoma

ADENOCORTICAL ADENOMA

PHEOCHROMOCYTOMA





  • Microscopic (histologic) description
  • In comparison to surrounding adrenal gland, adenoma cells are larger with different cytoplasm, increased variation in nuclear size
  • Distinct cell borders, cells have abundant foamy cytoplasm reminiscent of zona fasciculata
  • Balloon cells: clusters of cells with enlarged lipid-rich cytoplasm (seen in Cushing syndrome)
  • Histologic variants: oncocytic, myxoid

Electron microscopy description
  • Abundant intracytoplasmic lipid droplets of varying sizes
  • Prominent microvillous projections along cell borders
  • Abundant smooth endoplasmic reticulum
  • Prominent, round to oval mitochondria; cristae may have tubular to vesicular (zona fasciculata) or lamellar (zona reticularis) profile
Microscopic (histologic) images

                                         ADENOCORTICAL ADENOMA

ADENOCORTICAL CARCINOMA

PHEOCHROMOCYTOMA



Cytology description
  • Difficult to impossible to distinguish from unremarkable adrenal cortex 
  • Cellularity varies, generally loose clusters of large cells 
  • Foamy / vacuolated cytoplasm 
  • Round to oval nuclei with smooth contours, may have naked nuclei, variation in nuclear size / shape has little significance .
DIAGNOSIS:
Blood tests will be completed. A test of the cortisol levels may be done. Urine may need to be collected for 24 hours.                                   A biopsy of the tumor may be completed to determine whether or not the tumor is malignant.                                                                            Magnetic resonance imaging (MRI) or computed tomography (CT) scans may be used.                                                                                           Other tests may include adrenal vein sampling or a metaiodobenzylguanidine (MIBG) scan.                                                         If the tumor is cancerous, the tumor will be staged, which includes the size and location of the tumor; whether it has spread to the lymph nodes; and whether it has spread to other parts of the body. 
TREATMENT:                                                                                                                                                                                                                     The tumor may be removed via surgery. It is recommended that most functioning tumors, regardless of their size, be removed.                         Small, nonfunctioning tumors can be followed with repeat CT scans and if they remain stable in size they can be observed without treatment.               If a nonfunctioning tumor grows more rapidly than expected, or if it approaches 5 centimeters in maximum dimension, it is generally removed. If the tumor is small and appears to be noncancerous (benign), a laparoscopic strategy (surgery without large incisions) may be ordered.  For a larger tumor or one that may be cancerous, surgery with an incision in the back may be preferred.                                                                        In some cases, the entire adrenal gland may need to be removed. Hormone therapy may be required prior to or after other options, such as surgery, have been done.                                                                              If the tumor is cancerous, chemotherapy and radiation therapy may be needed. 






 

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