Description

Sumo is a traditional Japanese full-contact wrestling sport. Among sumo wrestlers, Ikezuki Geitazaemon (1827–1850) was one of the most famous wrestlers due to his large body size. Here, we hypothesize that the legendary Japanese sumo wrestler could have had gigantism or acromegaly, which disorders are caused by the hyper secretion of growth hormone (GH). GH-secreting pituitary adenoma leads to the hyper secretion of GH and insulin-like growth factor-1 (IGF-1). If GH-secreting pituitary adenoma develops during childhood/puberty, it can cause gigantism. Adenomas also occur in over 95% of patients with acromegaly.

Based on his substantial height (227 cm), Geitazaemon may have had gigantism, or acromegaly considering his characteristics of mandibular prognathism, enlarged fingertips, and heel pad thickness, as shown in woodblock prints (ukiyo-e). He would thus be the first recorded patient with gigantism or acromegaly in premodern Japan. Our objective was to describe the clinical course and treatment challenges in a very young patient with a pituitary adenoma due to a novel aryl hydrocarbon receptor–interacting protein (AIP) gene mutation, highlighting the limitations of somatostatin receptor immunohistochemistry to predict clinical responses to somatostatin analogs in acromegaly. Acromegaly and pituitary gigantism are two related clinical manifestations of chronic growth hormone (GH) and insulin-like growth factor 1 (IGF-1) hypersecretion, usually from a pituitary adenoma. Management of acromegaly is guided by consensus reports based on accumulated clinical trials, in which medical therapies including somatostatin analogs, the GH receptor antagonist pegvisomant, and dopamine agonists play a key role alongside neurosurgery. Pituitary gigantism represents a severe subtype of acromegaly, as it affects children/adolescents whose growth plates have not fused. Apart from the risk of increased final adult height, patients with pituitary gigantism also have relatively aggressive and treatment resistant disease. This phenotype is driven by the presence of genetic mutations in nearly 50% of pituitary gigantism cases, including AIP mutations, X-linked acrogigantism syndrome, and McCune–Albright syndrome. This chapter assesses the consensus medical therapy of acromegaly and highlights specific aims and challenges for the effective management of patients with pituitary gigantism.

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With Regards
praneeth varma
Journal Coordinator
Journal of Reproductive Endocrinology & Infertility