Gigantism

Gigantism is a serious condition that is nearly always caused by an adenoma, a tumor of the
pituitary gland. Gigantism occurs in patients who had excessive growth hormone in childhood.
The pituitary tumor cells secrete too much growth hormone (GH), leading to many changes
in the body.

Gigantism: Expert Care in Southern California Advanced

minimally invasive pituitary surgery

The physicians at the UCLA Pituitary Tumor Program offer comprehensive management of
gigantism. Our years of experience in diagnosing, treating and managing pituitary conditions,
as well as the high volume of patients we see every year, make us one of the best pituitary
programs in the United States.

Learn more about gigantism:

Physiology

Symptoms

Diagnosis

Treatment
Gigantism: Physiology
Physiology of Gigantism
Gigantism usually presents in childhood or young
adulthood. If a pituitary tumor that secretes growth
hormone develops after the bone growth plates
fuse, the result is acromegaly.

Gigantism is a form of familial pituitary adenomas,

and may run in some families due to a genetic
mutation.

Gigantism can also be associated with other conditions, including:

Carney complex

McCune-Albright syndrome (MAS)

Multiple endocrine neoplasia type 1 (MEN-1)

Neurofibromatosis

Normal Growth Hormone Physiology

Growth hormone is secreted by the pituitary gland in a pulsatile fashion, meaning that its level
goes up and down significantly throughout the day.

For this reason, growth hormone measured at a random time of the day may be meaningless
for diagnosis

Growth hormone release normally falls dramatically after eating a large quantity of sugar, a
phenomenon that is useful in making the diagnosis in acromegaly because tumor cells do not
exhibit this response (see oral glucose tolerance test)

Growth hormone has direct affects on the body, but like most pituitary hormones, acts on
other specialized gland cells in the body to stimulate the release of another hormone
Growth hormone acts on special cells in the liver, leading to the release into the blood stream
a hormone called Insulin-like Growth Factor (IGF-1).

IGF-1 is also called Somatomedin-C

Because IGF-1 is released in a more steady fashion, its levels do not go up and down quickly
and therefore it is a better hormone to measure to screen for acromegaly

Normal Control of Growth Hormone Production

A part of the brain called the hypothalamus controls the amount of growth hormone
production by sending chemicals ("neuropeptides") down the pituitary stalk to the pituitary
gland.

The main neuropeptide that stimulates growth hormone release is called growth hormone
releasing hormone (GHRH).

The main neuropeptide that inhibits growth hormone release is called somatostatin; drugs
that mimic the effects of somatostatin (somatostatin analogs, or SSA's) are used to treat
acromegaly.

Gigantism: Symptoms
The main symptom associated with gigantism is large body stature with increased height
compared to peers. Muscles and organs may be enlarged as well.

Other symptoms include:

Delay in puberty

Enlargement of Hands and Feet - Symptom of

Gigantism Physical changes similar to patients with
acromegaly, including:

Abnormal enlargement of the hands and feet

Widening of distal fingers and toes, called "paddle"

toes

Hands feel like soft bread dough

 Changes in facial features, which can be quite prominent:

Enlarged forehead and jaw

Pronounced underbite

Spreading teeth

Enlarged tongue, nose and lips

Deepening of the voice in males

Skin changes:

Increased number of skin tags

Oily skin and excessive sweating

Increased tissue in the wrists can lead to nerve compression, which may cause carpal
tunnel syndrome

Arthritis

Headache

Serious conditions related to long-standing, untreated gigantism/acromegaly include:

High blood pressure

Diabetes mellitus (adult-onset or Type 2)

Heart disease, including heart failure due to heart enlargement

Giants are at increased risk of developing other tumors or lesions of the body:

Thyroid cancer occurs at a higher frequency; talk to your doctor about screening

You should also undergo regular colonoscopies to screen for colon polyps
Gigantism: Macroadenomas
Macroadenomas are large pituitary tumors.
Large tumors can compress surrounding
structures, primarily the normal pituitary
gland and optic (visual) pathways, causing
symptoms. The symptoms that result from
the compression are independent of the
effects of excess growth hormone secretion.
Macroadenomas can cause:
Bitemporal Hemianopsia due to Macroadenomas Vision loss. This occurs when
macroadenomas grow upward into the brain cavity, compressing the optic chiasm.

A loss of the outer peripheral vision, called a bitemporal hemianopsia

When severe, a patient can only see what is directly in front of them

Many patients do not become aware of their visual loss until it is quite severe

Other visual problems can include:

Loss of visual acuity (blurry vision), especially if the macroadenoma grows forward and
compresses an optic nerve.

Colors not perceived as bright as usual

Pituitary Failure or Hypopituitarism

Increased compression of the normal gland can cause hormone insufficiency, called
hypopituitarism. The symptoms depend upon which hormone is involved.

Reduction of sex hormones, luteinizing hormone (LH) and follicle-stimulating hormone (FSH).

In men, this can lead to a low testosterone level, causing decreased sexual drive and
impotence.

In some cases, there can be loss of body and facial hair.

In women, this can lead to infertility.

Large pituitary tumors can slightly elevate blood prolactin levels. Doctors think this occurs
because of compression of the pituitary stalk, the connection between the brain and the
pituitary gland. It is called the "stalk effect."

In premenopausal women, this can lead to reduction or loss of menstrual periods and/or
breast milk production (galactorrhea).

Prolactin levels are only slightly elevated, as opposed to prolactinomas in which the prolactin
level is usually very high.

More severe hypopituitarism can lead to hypothyroidism or abnormally low cortisol levels, which may be life
threatening. Symptoms of severe hypopituitarism include:

Loss of appetite

Weight loss or weight gain

Fatigue

Decreased energy

Decreased mental function

Dizziness

Changes in hormonal function can cause electrolyte imbalance in the blood, typically low sodium levels
(hyponatremia). Symptoms could include: *Fatigue *Seizure

Gigantism: Diagnosis
Your doctor will conduct a physical examination
and ask you about your medical history and
symptoms. Pituitary Function Testing for
patients with pituitary tumor.

Other diagnostic procedures include:

Hormone testing

Imaging scans

Hormone Testing for Gigantism

Doctors are looking for an elevated blood IGF-1 level to diagnose gigantism.
Your doctor may order an oral glucose tolerance test (OGTT) to confirm the diagnosis. MRI
Scan Equipment

Drinking 75 grams of glucose solution lowers blood GH levels to less than one nanogram per
milliliter (ng/ml) in healthy people.

In acromegalics, this suppression does not occur. In fact, sometimes GH levels increase.

Surgery for Gigantism

Surgery for Gigantism Surgically removing the selected
tumors offers the best chance for cure. You should
seek the care of an experienced neurosurgeon that
performs a high volume of pituitary surgeries every
year. Our neurosurgeons practice the latest surgical
techniques, such as the expanded, endoscopic
endonasal approach, allowing us to cure pituitary
tumors that were previously deemed unresectable
(unable to be removed surgically).

Gigantism Treatment:
Radiation Therapy
Stereotactic radiosurgery is a technique in which
doctors aim a highly focused dose of radiation to the
tumor. It takes an average of 18 months to lower the
GH and IGF-1 levels.

The radiation beam is designed to target only the tumor. The surrounding brain structures
receive only a fraction of the radiation dose and are typically unharmed, with the exception
of the normal pituitary gland. In children, though, radiation therapy may cause learning
disabilities, obesity and emotional changes, so doctors may limit radiation use to situations
when all other treatment options have failed.
A consequence of radiation treatment is that it can cause delayed pituitary failure. This
typically occurs several years after treatment, and therefore continued long-term follow-up
with an endocrinologist is important. Hormone replacement may be required.

Organizations Supporting this Disease

Organizations Supporting this Disease

Pituitary Network Association

P.O. Box 1958

Thousand Oaks, CA 91358 USA
Phone: (805) 499-9973
Email: info@pituitary.org
Website: http://www.pituitary.org

Hormone Health Network Endocrine Society

2055 L Street NW
Suite 600
Washington, DC 20036
Phone: (202) 971-3636
Toll-free: (888) 363-6274
Email: hormone@endocrine.org
Website: http://www.hormone.org/
References/Weblinks
Science 10 (Learner’s Manual) pp. 165-190 Unit 3: Module 1 – Coordinated Functions of the Nervous
System, Endocrine, and Reproductive System
https://www.msdmanuals.com/home/hormonal-and-metabolic-disorders/pituitary-gland-
disorders/gigantism-and-acromegaly
http://pituitary.ucla.edu/resources
https://rarediseases.info.nih.gov/diseases/6506/gigantism
https://rarediseases.org/rare-diseases/acromegaly/
PREPARED BY: SANDONG,
BONIFACIO S.