Nama Maba : Dian Febiola Christian

Nama Amerta : Endocrinology

ACROMEGALY

Acromegaly is a hormonal disorder that results from too much growth

hormone (GH) in the body. The pituitary, a small gland in the brain, makes GH. In
acromegaly, the pituitary produces excessive amounts of GH. Usually the excess GH
comes from benign, or noncancerous, tumors on the pituitary. These benign tumors
are called adenomas. Acromegaly is most often diagnosed in middle-aged adults,
although symptoms can appear at any age. If not treated, acromegaly can result in
serious illness and premature death.i

More than 95% of acromegaly cases are caused by a pituitary adenoma that
secretes excess amounts of GH. Histopathologically, tumors include acidophil
adenomas, densely granulated GH adenomas, sparsely granulated GH adenomas,
somatomammotropic adenomas, and plurihormonal adenomas. Ectopic production of
GH and GHRH by malignant tumors accounts for other causes of IGF-I excess.
(Ectopic GHRH-producing tumors, usually seen in the lung or pancreas, may
occasionally be evident elsewhere, such as in the duodenum as a neuroendocrine
carcinoma.)ii

Of these tumors, up to 40% have a mutation involving the alpha subunit of

the stimulatory guanosine triphosphate (GTP)–binding protein. In the presence of a
mutation, persistent elevation of cyclic adenosine monophosphate (cAMP) in the
somatotrophs results in excessive GH secretion. The pathologic effects of GH excess
include acral overgrowth, insulin antagonism, nitrogen retention, increased risk of
colon polyps/tumors, and acral overgrowth (ie, macrognathia; enlargement of the
facial bone structure, as well as of the hands and feet; and visceral overgrowth,
including macroglossia and enlargement of the heart muscle, thyroid, liver, and
kidney).iii

Because IGF-I is a general growth factor, somatic hypertrophy in acromegaly

occurs across all organ systems.iv Associated complications include the following
Acromegalic heart, increased muscle and soft tissue mass, increased kidney size,
articular overgrowth of synovial tissue and hypertrophic arthropathy, joint symptoms,
back pain, and kyphosis, common presenting features, thick skin, hyperhidrosis (often
malodorous), carpal tunnel syndrome and other entrapment syndromes, macroglossia:
may result in sleep apnea, cerebral aneurysm and increased risk of cerebrovascular
accident: Less common.v

Daftar Pustaka

i
Anne Klibanski, M.D. and Shlomo Melmed, M.D. Agustus 2016. NIDKK.
Endocrine Disease. Acromegaly. https://www.niddk.nih.gov/health-
information/endocrine-diseases/acromegaly. Diakses tanggal 19 Agustus 2017. Jam
23.40.

ii
Khandelwal D, Khadgawat R, Mukund A, Suri A. Acromegaly with no pituitary
adenoma and no evidence of ectopic source. Indian J Endocrinol Metab. 2013 Sep.
15 Suppl 3:S250-2.
iii
Uchoa HB, Lima GA, Corrêa LL, Vidal AP, Cavallieri SA, Vaisman M, et al.
Prevalence of thyroid diseases in patients with acromegaly: experience of a Brazilian
center. Arq Bras Endocrinol Metabol. 2013 Dec. 57(9):685-90.
iv
Melmed S, Casanueva FF, Klibanski A, Bronstein MD, Chanson P, Lamberts SW,
et al. A consensus on the diagnosis and treatment of acromegaly
complications. Pituitary. 2013 Sep. 16(3):294-302.

v
Oshino S, Nishino A, Suzuki T, et al. Prevalence of cerebral aneurysm in patients
with acromegaly. Pituitary. 2013 Jun. 16(2):195-201.