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Acromegaly & Gigantism

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Enormously huge fighters compete in the world of combat sports. A small percentage of people in the world are born with the genetic predisposition to become large-sized adults, while another small percentage have the genetic predisposition to become small-sized adults. Most people are within a “medium range”. The environment, such as malnutrition, can have dramatic effect on the final size of an individual adult.

How can we tell whether an enormous-sized person was simply born with the genetic predisposition to become an adult along the upper echelon in size, thus a “normal” person at the upper end of a bell-shaped curve versus someone who is “abnormal”, i.e. the individual’s large-size is secondary to pathology. Large fighters abound in the world of mixed martial arts (MMA): with some being “normal” huge and others being “abnormal” large. For example, I hear many MMA fans ask, “How did Hong Man Choi get so big?”

The answer is Hong Man Choi has acromegaly/gigantism, which is almost always due to a pituitary tumor, known as adenoma. Since the pituitary is a central endocrine gland, the people with gigantism also have disturbance of many endocrine functions, e.g. glucose intolerance. Antonio ‘Big Foot’ Silva is another MMA fighter who competed for quite a while before he was treated surgically for his pathology.

How do we know if someone has acromegaly/gigantism or if he is simply a large person along the bell curve, representing a “normal” population of people? One key attribute of acromegaly/gigantism is the afflicted individuals have similar facial features, regardless of race or ethnicity. For example, although Antonio Silva is Brazilian and Hong Man is Korean, their face looks alike. The bone & cartilage changes have very distinct affects on the face/skull, along with enlarged hands/feet.

The characteristic clinical manifestations are the consequence of chronic growth hormone (GH) hypersecretion, which in turn leads to excessive generation of the somatomedins, i.e. the mediators of most of the effects of GH. Overgrowth of bone is the classic feature, but GH excess produces a generalized systemic disorder with deleterious effects on numerous body systems and an increased mortality rate. However, the deaths are rarely due to the space-occupying or destructive effects of the pituitary adenoma per se.

In adults, GH excess leads to acromegaly or the syndrome characterized by local overgrowth of bone, especially the skull and mandible (jaw bone). In childhood and adolescence, the onset of chronic GH excess leads to gigantism. Most people with gigantism develop facial features of acromegaly if GH hypersecretion persists through their teenage years and into adulthood.

Acromegaly is a chronic disabling and disfiguring disorder with increased late morbidity and mortality if left untreated. Spontaneous remissions have been reported, but the usual course is slow progressive development of symptoms and signs. Early manifestations include enlarged hands and feet along with coarsening of facial features. By the time a physician makes the diagnosis, virtually all patients have the classic manifestations, which include thickening of the calvarium, increased size of frontal sinuses, prominence of the supraorbital ridges, enlargement of the nose, and downward and forward protrusion of the jaw bone, which leads to widely spaced teeth. Predominantly soft tissue growth results in enlarged hands/feet. A bulky, sweaty handshake is notorious for the condition.

A wide variety of symptoms and signs may be present: excessive sweating (hyperhidrosis), lethargy/fatigue, numbness/tingling in hand/fingers (due to carpal tunnel compression), painful joints, sensitivity to bright light (photophobia), thyroid goiter, enlarged salivary glands, hypertension, enlarged heart (cardiomegaly), kidney stones, degenerative knee and hip joints, and degenerative spine.

People with acromegaly ought to undergo therapy to halt progression of the disorder and prevent late complications. Therapy is designed to remove or destroy the pituitary tumor, reverse the GH hypersecretion, while maintaining normal anterior and posterior pituitary function. Many patients with acromegaly undergo transsphenoidal selective surgical adenoma removal, especially those with small or medium-sized tumors.  

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