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Gigantism

Pituitary Gigantism

Gigantism is characterized by abnormal growth in children due to excessive production of insulinlike growth factor I (IGF-I). Basically there is excess production of growth hormone which contributes to the accelerated linear growth.


Presentation

Children with gigantism are taller than other age matched children. In many instances, certain parts are proportionately bigger than other parts. Affected children can also have long hands and feet, with signs of thickening of fingers and toes along with a prominent forehead and jaw. Children with gigantism may also have coarse facial features which include a larger than normal head, flat nose, and large lips and tongue.

Symptoms of gigantism largely depend on the size of the tumor on the pituitary gland. When the tumor gradually increases in size, it may exert pressure on the neighboring nerves in the brain. This may further cause the children to suffer from headaches or loss of vision. In addition, children would also experience weakness, fertility problems, delay in onset of puberty and excessive sweating. In severe cases, deafness can also set in [7].

Tall Stature
  • This boy appears likely to have neurofibromatosis type 1 (NF1) which raises the question of subtle GH excess in NF1 patients with tall stature.[ncbi.nlm.nih.gov]
  • A large pituitary adenoma with suprasellar extension was diagnosed in a 12-year-old girl with progressive tall stature (178 cm), GH hypersecretion without suppression during oral glucose loading (nadir serum GH, 90 mU/l), high serum IGF-I and serum prolactin[ncbi.nlm.nih.gov]
  • 2018 2019 Billable/Specific Code Applicable To Constitutional gigantism E34.4 ) constitutional tall stature ( ICD-10-CM Diagnosis Code E34.4 Constitutional tall stature 2016 2017 2018 2019 Billable/Specific Code Applicable To Constitutional gigantism[icd10data.com]
  • Gigantism develops when a child experiences accelerated growth due to excessive GH production, which eventually leads to their tall stature.[theconversation.com]
Accelerated Growth
  • A 15-yr-old female patient presented with accelerated growth due to a large pituitary tumor that was surgically resected to relieve pressure effects.[ncbi.nlm.nih.gov]
  • growth continues for the first 4 or 5 years, the rate being normal thereafter.[medical-dictionary.thefreedictionary.com]
  • The consequence is an accelerated growth rate and increased height as well as a number of additional soft tissue changes.[yourhormones.info]
  • Partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections.[link.springer.com]
Thrombosis
  • The patient's past medical history included renal transplantation on the same side well before the AVG was inserted and right leg deep vein thrombosis.[ncbi.nlm.nih.gov]
Prognathism
  • It appears in childhood and many morphological signs lead us to suspect it: enlarged hands and feet, thick skin, a prominent chin (called prognathism), and an abnormal tendency to sweat ... The organs are also increased in size.[health.ccm.net]
  • What you'll see in someone with acromegaly is they get what's called frontal bossing [an enlarged forehead], prognathism--their jaw juts out--they get spreading of the teeth, they get enlarged hands and feet.[scientificamerican.com]
  • Overgrowth of the jawbone (mandible) can cause the jaw to protrude (prognathism). Cartilage in the voice box (larynx) may thicken, making the voice deep and husky. The ribs may thicken, creating a barrel chest. Joint pain is common.[merckmanuals.com]
  • Overgrowth of the mandible leads to protrusion of the jaw (prognathism) and malocclusion of teeth. Cartilaginous proliferation of the larynx leads to a deep, husky voice. The tongue is frequently enlarged and furrowed.[merckmanuals.com]
Foot Ulcer
  • Systemic sepsis from an infected foot ulcer necessitated treatment by an above-knee amputation. Postoperatively, the stump increased in size by 19 kg. A trial of rapamycin to reverse the growth of the stump has shown promise.[ncbi.nlm.nih.gov]
Macrocephaly
  • We report on a neonate presenting with polyhydramnios; macrosomia; macrocephaly; visceromegaly including bilateral nephromegaly, hepatomegaly, cardiomegaly; thymus hyperplasia; cryptorchidism; generalized muscle hypotonia; and a distinctive facial appearance[ncbi.nlm.nih.gov]
  • Partial gigantism of the hands and/or feet, nevi, hemihypertrophy, subcutaneous tumors, macrocephaly or other skull anomalies and possible accelerated growth and visceral affections.[link.springer.com]
  • His growth-hormone excess manifested as tall stature, coarse facial features, and macrocephaly. More than 95% of acromegaly cases are caused by a pituitary adenoma that secretes excess amounts of GH.[emedicine.medscape.com]
Advanced Bone Age
  • Her precocious manifestations were public hair, acne vulgaris, hirsutism, and advanced bone age. Endocrinological examination revealed markedly increased serum growth hormone (GH) and prolactin (PRL), which responded paradoxically to a TRH test.[ncbi.nlm.nih.gov]
Short Hands
  • Voice is hoarse and affected individuals have a plump, stocky body with pectus excavatum, thoracic scoliosis, hepatosplenomegaly, umbilical and/or inguinal hernias, broad short hands and feet, and in some cases preauricular dimples, abnormal ears, postaxial[ncbi.nlm.nih.gov]
Frontal Bossing
  • Other symptoms may include delayed puberty ; double vision or difficulty with side (peripheral) vision; prominent forehead ( frontal bossing ) and a prominent jaw; headache; increased sweating ( hyperhidrosis ); irregular periods; large hands and feet[rarediseases.info.nih.gov]
  • Other symptoms include: Delayed puberty Double vision or difficulty with side (peripheral) vision Very prominent forehead ( frontal bossing ) and a prominent jaw Gaps between the teeth Headache Increased sweating Irregular periods (menstruation) Joint[medlineplus.gov]
  • What you'll see in someone with acromegaly is they get what's called frontal bossing [an enlarged forehead], prognathism--their jaw juts out--they get spreading of the teeth, they get enlarged hands and feet.[scientificamerican.com]
Cerebral Calcification
  • ., cutaneous hypomelanotic macules, poliosis, cyst-like lesions of the right upper limb and cerebral calcifications.[ncbi.nlm.nih.gov]

Workup

Measuring the blood levels of growth hormone and insulinlike growth factors forms the preliminary basis of diagnostic procedure. Another method of measuring the insulinlike growth factor is to determine the levels about 1 hour after administration of a glucose load. Under normal circumstances, the hormone levels decrease after a glucose load. In children with gigantism, the values remain normal even after consuming glucose. This indicates that the body produces excessive growth hormones, which causes the levels to remain normal [8]. Imaging studies such as CT scan and MRI are indicated to detect the location as well as size of the tumor in the pituitary gland. 

Treatment

Treatment of gigantism is aimed at slowing down the progression of production of growth hormones as well as effective management of symptoms. The following methods are employed in order to fulfill the objectives:

  • Surgery is the first line of treatment if tumors in the pituitary gland are the major factor for gigantism. 
  • Medicines are employed when surgery cannot be performed. In such cases, medications are administered for management of the symptoms. Medicines such as lanreotide or octreotide are given monthly intravenously to reduce the excessive production of growth hormones. Another new drug known as pegvisomant has been developed to lower the production of growth hormones. The drug has to be injected daily and is used when other options or medications fail to yield positive results [9].
  • Gamma knife radiosurgery: This type of method is given when surgery is not an option. In such cases, high beam radiation is given to the tumor cells in order to destroy it. This method takes several years for bringing the levels of growth hormone back to normal and is also associated with several side effects; hence it is used very rarely [10].

Prognosis

Research has shown that, about 80% of cases can be successfully treated with surgery to remove the tumor. In situations, when surgery cannot be successfully accomplished then medications are employed for effective management of symptoms. In either of the cases, individuals can live a long and healthy life [6].

Etiology

Development of a tumor in the pituitary gland is the major cause of gigantism. Excessive release of growth hormone due to a benign tumor in the pituitary gland favors abnormal growth development in children [2].

In addition to excess production of growth hormones, gigantisms can also occur as a result of other conditions, which include neurofibromatosis, Carney complex, multiple endocrine neoplasia type I, tuberous sclerosis and McCune-Albright syndrome. It has been estimated that, about 20% patients suffering from gigantisms also suffer from McCune-Albright syndrome [1].

Epidemiology

Gigantism is a rare phenomenon, and only about 100 cases are reported till date. The condition of acromegaly is more common than gigantism, with a reported incidence of 3 – 4 cases per million each year; having a prevalence rate of 40 – 70 cases per million population. Gigantism can strike at any age before the epiphyseal fusion takes place. Agromegaly occurs in the third decade of life; with mean age of diagnosis being 40 years and 54 years in females and males respectively [4]. No sex predilection is known. 

Sex distribution
Age distribution

Pathophysiology

The excess production of insulinlike growth factor can be divided into the following factors:

  • Production and release of excessive growth hormone from the pituitary gland
  • Increase in the secretion of growth hormone releasing hormone 
  • Excess production of insulinlike growth hormone-binding protein, which in turn is responsible for prolonging the half-life of insulinlike growth hormone
  • The malfunctioning of the pituitary gland due to development of benign tumor is the major cause, and this promotes excessive production of growth hormones. This gland is located at base of the brain and is important for production of hormones that control the various functions of the body [5].

Prevention

Gigantism cannot be prevented. However, with early initiation of treatment, the onset of complications can certainly be kept at bay.

Summary

Gigantism occurs during childhood, much before the epiphyseal growth plates have closed. Early diagnosis along with prompt initiation of treatment is important for arresting the accelerated growth pattern. However, many parents fail to recognize that their child is suffering from gigantism as they consider the sudden growth as normal childhood growth spurts. Individuals with gigantism measure between 2.13 m to 2.74 m [1].

Patient Information

  • Definition: Gigantism is a condition wherein there is excessive production of growth hormones that causes abnormal growth in affected children. Prompt diagnosis and early initiation of treatment is required to arrest complications to set in.
  • Cause: Excessive production of growth hormones due to development of tumor in the pituitary gland is the major cause for gigantism. Other less common causes include various conditions such as neurofibromatosis, Carney complex, multiple endocrine neoplasia type I and McCune-Albright syndrome.
  • Symptoms: Symptoms of gigantism include larger than normal height compared to other children of same age. Affected children also suffer from delayed onset of puberty, difficulty in vision, sleep problems, weakness, excessive sweating, prominent jaws, headache, irregular menstruation, larger than normal hands and feet which have thickened fingers and toes.
  • Diagnosis: Determining the blood levels of growth hormone helps in arriving at a definitive diagnosis. The levels of the hormone can also be checked an hour later after administration of glucose load. MRI and CT scan would be required for locating the site and determining the shape of the tumor.
  • Treatment: Surgery forms the first line of treatment. If surgery isn’t an option then, medications are given to control the excessive production of growth hormones. A new drug that has been introduced to control the growth hormone release is pegvisomant. Gamma knife radiosurgery is the last resort and employed only when surgery and medications have not been successful in bringing about the desired results.

References

Article

  1. Tanner JM, Davies PS. Clinical longitudinal standards for height and height velocity for North American children. J Pediatr 1985; 107:317.
  2. Nainggolan L. Gene Discovery in Giants Could Shed Light on Human Growth. Medscape Medical News.
  3. Sotos JF. Overgrowth.Hormonal Causes.ClinPediatr (Phila) 1996; 35:579.
  4. Giustina A, Chanson P, Bronstein MD, Klibanski A, Lamberts S, Casanueva FF, et al. A consensus on criteria for cure of acromegaly. J ClinEndocrinolMetab. Jul 2010;95(7):3141-8.
  5. Khandelwal D, Khadgawat R, Mukund A, Suri A. Acromegaly with no pituitary adenoma and no evidence of ectopic source. Indian J EndocrinolMetab. Sep 2011;15Suppl 3:S250-2.
  6. Thomsett MJ. Referrals for tall stature in children: a 25-year personal experience. J Paediatr Child Health 2009; 45:58.
  7. Abe T, Tara LA, Lüdecke DK. Growth hormone-secreting pituitary adenomas in childhood and adolescence: features and results of transnasal surgery. Neurosurgery. Jul 1999;45(1):1-10
  8. Sippell WG, Partsch CJ, Wiedemann HR. Growth, bone maturation and pubertal development in children with the EMG-syndrome. Clin Genet 1989; 35:20.
  9. Rix M, Laurberg P, Hoejberg AS, Brock-Jacobsen B. Pegvisomant therapy in pituitary gigantism: successful treatment in a 12-year-old girl. Eur J Endocrinol. Aug 2005;153(2):195-201
  10. Jagannathan J, Yen CP, Pouratian N, Laws ER, Sheehan JP. Stereotactic radiosurgery for pituitary adenomas: a comprehensive review of indications, techniques and long-term results using the Gamma Knife. J Neurooncol. May 2009;92(3):345-56.

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Last updated: 2017-08-09 17:57