A 24-Year-Old Male with Gigantism, Growth Hormone Deficiency, Suspected Clivus Chordoma, Primary Hypothyroidism, Hypogonadism and Pancytopenia

W.A. Arsana, M.I. Diah Pramudianti

Abstract


Pituitary gigantism is a condition caused by excessive secretion of Growth Hormone (GH). The GH is the most common
pituitary hormone-deficient in pituitary disease. Chordoma is a bone primary tumor that grows slowly and is rarely found.
Hypothyroidism is a pathological condition due to thyroid hormone deficiency. Symptoms of hypogonadism are
non-specific including libido disorders, erectile dysfunction, and decreased muscle mass and no hair growth in the head or
body. A 24-year-old male came with pain in the knee. Physical examination showed increased growth of natural and body
parts as well as the loss of body hair. Laboratory investigations revealed pancytopenia, increased prolactin; decreased GH,
Insulin-Like Growth Factor-1 (IGF-1) and testosterone; increased Thyroid-Stimulating Hormone (TSH), decreased Free
Triiodothyronine (FT3) and Free Thyroxine (FT4). Ahead MRI demonstrated the presence of a mass in the clivus. In this case,
the patient presented with clinical gigantism. However, laboratory examination showed decreased GH and IGF-1 which
might be due to the suppressive effect of mass on the clivus bone to the pituitary. Further examinations were needed to clear
the suspicion of hypothyroid. Hypogonadism can result from suppression in the pituitary. Pancytopenia can be caused by a
deficiency of GH or from hypothyroidism. Gigantism may occur with GH and IGF-1 deficiency due to suppressed pituitary
caused by chordoma.


Keywords


Gigantism, clivus chordoma, primary hypothyroidism, hypogonadism, pancytopenia

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References


Young WF. The Netter Collection of Medical Illustrations: The Endocrine System. 2nd ed. Philadelphia: Elsevier, p 21-22; 2011.

Holt IG, Hanley NA. Essential Endocrinology and Diabetes. 6th ed. West Sussex: Blackwell Publishing, p 65-98; 2012.

Melmed S. Endocrinology : Adult and pediatric. 7th ed. Philadelphia: Elsevier, p:209-226e7; 2016.

Hannah-Shmoni F, Trivellin G, Stratakis CA. Genetics of Gigantism and Acromegaly. Growth Horm IGF Res. 2016;30-31: 37–41. doi:10.1016/j.ghir.2016.08.002.

Rhee N, Jeong K, Yang EM, Kim CJ. Gigantism caused by growth hormone secreting pituitary adenoma. Ann Pediatr Endocrinol Metab. 2014;19:96-99. doi :10.6065/apem.2014.19.2.96.

Durmaz SA, Bahceci M, Tuzku A, Gokalp D, Guzel A, Ayag H. An acromegalic patient with low Insulin-Like Growth Factor-1 levels: it may not be found to be elevated during diagnosis of acromegaly each time. Turkısh Journal of Clinics and Laboratory. 2015;6:45-48

Ayuk J, Sheppard MC. Growth hormone and its disorders. Postgrad Med J. 2006;82:24–30. doi:10.1136/pgmj.2005.036087.

Stagi S, Scalini P, Farello G, Verrotti A. Possible effects of an early diagnosis and treatment in patients with growth hormone deficiency: the state of art. Italian Journal of Pediatrics . 2017;43:81. doi:10.1186/s13052-017-0402-8.

Devesa J, Almenglo C, Devesa P. Multiple Effects of Growth Hormone in the Body: Is it Really the Hormone for Growth?. Clinical Medicine Insights: Endocrinology and Diabetes. 2016;9:47–7. doi:10.4137/CMed.s38201.

Cordido F, Arnes JAG, Aspiroz MM, Vela ET. Practical guidelines for diagnosis and treatment of acromegaly. Endocrinol Nutr. 2013;60(8):457.e1-457.e15.

Labidi M, Watanabe K, Bouazza S, Bresson D, Bernat AL, George B, Froelich S. Clivus chordomas: a systematic review and meta-analysis of contemporary surgical management. J Neurosurg Sci. 2016;60(4):476-84.

Walcott BP, Nahed BV, Mohyeldin A, Coumans JV, Kahle KT, Ferreira MJ. Chordoma: current concepts, management, and future directions. Lancet Oncol. 2012;13: e69–76.

Hirosawa RM, Santos ABA, Franca MM, Vebris VE, Castro AVB, Zanini MA, Nunes VS. Intrasellar chondroid chordoma: A case report. ISRN Endocrinology. 2011. doi:10.5402/2011/259392.

Falconer MA, Bailey IC, Duchen LW. Surgical treatment of chordoma and chondroma of the skull base. J Neurosurg. 1968;29(3):261-75. doi:10.3171/jns.1968.29.3.0261.

Fernandez-Miranda J C, Gardner PA, Snyderman CH, Devaney KO, Mendenhall WM, Suarez C, Rinaldo A, Ferlito A. Clival chordomas: A pathological, surgical, and radiotherapeutic review. Wiley Online Library. 2013;p 892-906. doi:10.1002/hed.23415.

Virdi G, McGraw I, MacDuff E, Periasamy K, Sharma H. An Atypical Presentation of Chordoma: Case Report and Review. Orthop Muscular Syst. 2017; 6(4). doi: 10.4172/2161-0533.1000247.

What, Where is the Clivus [cited 2016 Sept 10}. Available on http://clivustumor.blogspot.co.id/2013/03/what-where-is-clivus.html

Chaker L, Bianco AC, Jonklaas J, Peeters RP. Hypothyroidism. Lancet. 2017;390:1550-62. doi:10.1016/S0140-6736(17)30703-1.

Luca P, Brabant G, Dattani M, Bonomi M, Feldt-Rasmussen U, Fliers E, Gruters A, et al. 2018 European Thyroid Association (ETA) Guidelines on the Diagnosis and Management of Central Hypothyroidism. Eur Thyroid J. 2018. doi: 10.1159/000491388.

Aw TC, Yap CY. Thyroid function tests: Proceedings of Singapore Healthcare. 2011; 20(2):132-137

Basaria S. Male hypogonadism. Lancet. 2014;383:1250-63. doi:10.1016/S0140-6736(13)61126-5.

Rosenquist KJ, Kaiser UB. Pituitary Disorders : Diagnosis and Management. 1st ed. West Sussex: Wiley Blackwell. 2013. Chapter 12, Hypopituitarism; p.120-129.

Dohle GR, Arver S, Bettocchi C, Jones TH, Kliesch S. EAU Guidelines on Male Hypogonadism. European Association of Urology. 2017.

Lang D, Mead JS, Sykes DB. Hormones and the bone marrow: panhypopituitarism and pancytopenia in a man with a pituitary adenoma. J Gen Intern Med. 2015;30(5):692–6. doi:10.1007/s11606-014-3161-x.

Koh JH, Lee YJ, Kang JH, Choi BK, Jeon YK, Kim SS, Kim BH, Kim IJ. Pancytopenia associated with hypopituitarism in an acromegaly patient: A case report and a review of the literature. Endocrinol Metab. 2012;27(4):308-313. doi:10.3803/EnM.2012.27.4.308.




DOI: http://dx.doi.org/10.24293/ijcpml.v26i2.1478

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