{"entity":{"id":"somatotroph-pitnet","kind":"cancer","name":"Somatotroph pituitary neuroendocrine tumour (acromegaly)","aka":["Somatotroph tumour","Somatotroph tumour (acromegaly)","Growth hormone-secreting pituitary adenoma","GH-secreting PitNET","Somatotroph adenoma","Gigantism (in children)"],"tldr":"A somatotroph tumour is a pituitary tumour that makes too much growth hormone, causing acromegaly in adults (enlarging hands, feet and face, diabetes, heart and joint disease) or gigantism in children. Surgery through the nose comes first; if hormone levels stay high, somatostatin analogue injections, the blocker pegvisomant or radiotherapy bring them down, restoring a normal life expectancy.","summary":"The 2022 WHO classification places the somatotroph tumour in the PIT1 lineage with densely and sparsely granulated subtypes (the sparsely granulated tumour being larger, more invasive and less responsive to first-generation somatostatin analogues) beside the mammosomatotroph and mixed tumours (Asa 2022). The 13th Acromegaly Consensus Conference (2019) set out multidisciplinary management at pituitary tumour centres of excellence: surgery, radiotherapy and medical therapy, their results and side effects, and how they are combined and personalised (Giustina 2020); the 2013 consensus on medical treatment defined biochemical, clinical and tumour-volume goals and the place of somatostatin receptor ligands, the growth hormone receptor antagonist and dopamine agonists (Giustina 2014).\n\nHow it differs from its parent: the harm comes from the hormone rather than the mass, so biochemical control (normal IGF-1 and growth hormone) is the treatment target and predicts survival; the granulation subtype predicts drug response; and the disease is often diagnosed a decade after onset because the changes are slow.\n\nHow common: no separate incidence figure in the sources read.\n\nTreatment: transsphenoidal surgery first; first-generation somatostatin analogues (octreotide, lanreotide) or pasireotide, pegvisomant, or cabergoline for persistent disease, alone or combined; radiotherapy including radiosurgery for residual tumour not controlled medically, as the consensus documents describe (Giustina 2020; Giustina 2014).","asOf":"2026-09-24","wikipedia":"https://en.wikipedia.org/wiki/Acromegaly","links":[{"label":"NCI PDQ: pituitary tumours treatment","url":"https://www.cancer.gov/types/pituitary/treatment"},{"label":"Asa 2022, Endocrine Pathology: overview of the 2022 WHO classification of pituitary tumours","url":"https://doi.org/10.1007/s12022-022-09703-7"},{"label":"Giustina 2020, Rev Endocr Metab Disord: multidisciplinary management of acromegaly, a consensus","url":"https://doi.org/10.1007/s11154-020-09588-z"},{"label":"Giustina 2014, Nature Reviews Endocrinology: consensus on the medical treatment of acromegaly","url":"https://doi.org/10.1038/nrendo.2014.21"}],"tags":["subtype-page","wave4","rare"],"related":["pituitary-tumours","lactotroph-pitnet","corticotroph-pitnet","gonadotroph-pitnet","thyrotroph-pitnet"],"cancers":[],"sections":[],"technologies":["radiosurgery-srs"],"targets":[],"drugs":["pasireotide","pegvisomant","cabergoline"],"companies":[],"institutions":[],"pathways":[],"terms":[],"trials":[],"people":[],"bottlenecks":[],"keyPapers":[],"journals":[],"dependsOn":[],"notes":[],"group":"endocrine","burden":"The pituitary tumour behind acromegaly; the acromegaly consensus conferences give its clinical figures and the parent page the pituitary tumour figures. GLOBOCAN does not count pituitary tumours.","subtypes":["Densely granulated somatotroph pituitary tumour (responds to first-generation somatostatin analogues)","Sparsely granulated somatotroph pituitary tumour (larger, invasive, less responsive)","Mammosomatotroph and mixed somatotroph-lactotroph tumours","Somatotroph tumour in gigantism (children; AIP or GPR101 germline in some)"],"biomarkers":["IGF-1 and growth hormone after glucose","Granulation pattern and cytokeratin staining; PIT1 lineage","Somatostatin receptor 2 and 5 expression (drug response)","AIP and GPR101 germline testing in young or familial cases"],"standardOfCare":[{"setting":"First line","approach":"Transsphenoidal surgery at a pituitary centre.","refs":["pituitary-tumours"]},{"setting":"Persistent disease","approach":"Somatostatin analogues or pasireotide, pegvisomant, cabergoline, alone or combined; radiotherapy for residual tumour not controlled medically.","refs":["pasireotide","pegvisomant","cabergoline","radiosurgery-srs"]}],"stateOfArt":[],"history":[],"pipeline":[],"openProblems":[],"parent":"pituitary-tumours"},"route":"/cancers/somatotroph-pitnet/","neighbours":{"cancer":[{"id":"corticotroph-pitnet","kind":"cancer","name":"Corticotroph pituitary neuroendocrine tumour (Cushing disease and silent corticotroph tumour)","route":"/cancers/corticotroph-pitnet/"},{"id":"gonadotroph-pitnet","kind":"cancer","name":"Gonadotroph pituitary neuroendocrine tumour (non-functioning adenoma)","route":"/cancers/gonadotroph-pitnet/"},{"id":"lactotroph-pitnet","kind":"cancer","name":"Lactotroph pituitary neuroendocrine tumour (prolactinoma)","route":"/cancers/lactotroph-pitnet/"},{"id":"pituitary-tumours","kind":"cancer","name":"Pituitary tumours (pituitary neuroendocrine tumours) and pituitary carcinoma","route":"/cancers/pituitary-tumours/"},{"id":"thyrotroph-pitnet","kind":"cancer","name":"Thyrotroph pituitary neuroendocrine tumour (TSH-secreting)","route":"/cancers/thyrotroph-pitnet/"}],"technology":[{"id":"radiosurgery-srs","kind":"technology","name":"Stereotactic radiosurgery (Gamma Knife, CyberKnife, linac SRS)","route":"/technologies/radiosurgery-srs/"}],"drug":[{"id":"cabergoline","kind":"drug","name":"Cabergoline","route":"/drugs/cabergoline/"},{"id":"pasireotide","kind":"drug","name":"Pasireotide","route":"/drugs/pasireotide/"},{"id":"pegvisomant","kind":"drug","name":"Pegvisomant","route":"/drugs/pegvisomant/"}]}}