Pituitary gigantism: a retrospective case series
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Ana L. Creo
und
Aida N. Lteif
Abstract
Background: 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.
Methods: 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.
Results: 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.
Conclusions: 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 control GH secretion. Treatment with octreotide or pegvisomant was successful in less than half the patients failing surgery. Radiosurgery was curative, but is not an optimal treatment for pediatric patients. Despite the small sample, our study suggests that the treatment outcome of pediatric PG may be different than adults.
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Artikel in diesem Heft
- Frontmatter
- Review
- Resistance to thyroid hormone α, revelation of basic study to clinical consequences
- Original Articles
- Improved molecular diagnosis of patients with neonatal diabetes using a combined next-generation sequencing and MS-MLPA approach
- Safety and metabolic impact of Ramadan fasting in children and adolescents with type 1 diabetes
- Waist-to-height ratio as a marker of low-grade inflammation in obese children and adolescents
- Classification and clinical characterization of metabolically “healthy” obese children and adolescents
- Long-term BH4 (sapropterin) treatment of children with hyperphenylalaninemia – effect on median Phe/Tyr ratios
- Compound heterozygous mutations (p.T561M and c.2422delT) in the TPO gene associated with congenital hypothyroidism
- Prevalence and clinical features of polycystic ovarian syndrome in adolescents with previous childhood growth hormone deficiency
- Urate crystals deposition in the feet of overweight juveniles and those with symptomatic hyperuricemia: a dual-energy CT study
- A novel ALMS1 homozygous mutation in two Turkish brothers with Alström syndrome
- Novel AVPR2 mutation causing partial nephrogenic diabetes insipidus in a Japanese family
- Pituitary gigantism: a retrospective case series
- Case Reports
- A novel OTX2 gene frameshift mutation in a child with microphthalmia, ectopic pituitary and growth hormone deficiency
- A novel nonsense mutation in the WFS1 gene causes the Wolfram syndrome
- A 33-year-old male patient with paternal derived duplication of 14q11.2–14q22.1~22.3: clinical course, phenotypic and genotypic findings
- Familial Turner syndrome: the importance of information
- De novo mutation of PHEX in a type 1 diabetes patient
- Congenital hypothyroidism and thyroid dyshormonogenesis: a case report of siblings with a newly identified mutation in thyroperoxidase
