Pituitary gigantism: a retrospective case series

Journal of Pediatric Endocrinology & Metabolism : JPEM
Ana L Creo, Aida N Lteif

Abstract

Pituitary gigantism (PG) is a rare pediatric disease with poorly defined long-term outcomes. Our aim is to describe the longitudinal clinical course in PG patients using a single-center, retrospective cohort study. Patients younger than 19 years diagnosed with PG were identified. Thirteen cases were confirmed based on histopathology of a GH secreting adenoma or hyperplasia and a height >2 SD for age and gender. Laboratory studies, initial pathology, and imaging were abstracted. Average age at diagnosis was 13 years with an average initial tumor size of 7.4×3.8 mm. Initial transsphenoidal surgery was curative in 3/12 patients. Four of the nine patients who failed the initial surgery required a repeat procedure. Octreotide successfully normalized GH levels in 1/6 patients with disease refractory to surgery (1/6). Two out of five patients received pegvisomant after failing octreotide but only one patient responded to treatment. Five patients were ultimately treated with radiosurgery or radiation patients were followed for an average of 10 years. PG is difficult to treat. In most patients, the initial transsphenoidal surgery failed to normalize GH levels. If the initial surgery was unsuccessful, repeat surgery was unlikely to contr...Continue Reading

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Citations

Sep 16, 2016·Pituitary·Nicholas A Tritos, Beverly M K Biller
Aug 1, 2019·Archives of Endocrinology and Metabolism·William Rojas GarcíaAndrés Florez Romero
Jan 2, 2022·BMJ Case Reports·Clara CunhaJoão Sequeira Duarte

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