Limited data is available on pituitary gigantism, as it is a rare disorder.
A retrospective analysis of 14 patients with pituitary gigantism presented to a single tertiary care institute, from 1990 to 2014, was conducted.
Thirteen patients were male, while one was female. Mean age at diagnosis was 21.9 ± 6.1 years, with a mean lag period of 6.5 ± 5.6 years. Mean height SD score at the time of diagnosis was 3.2 ± 0.6. Symptoms of tumor mass effect were the chief presenting complaint in majority (50 %) of the patients, while two patients were asymptomatic. Six patients had hyperprolactinemia. At presentation, nadir PGGH (Post-glucose GH) and IGF1-ULN (x upper limit of normal) were 63.2 ± 94.9 ng/ml and 1.98 ± 0.5 respectively. All (except one patient with mild pituitary hyperplasia) had pituitary macroadenoma. Six patients had invasive pituitary adenoma. Transsphenoidal approach (TSS) remained primary modality of treatment in 13/14 patients and it achieved remission in 4/13 patients (30.76%) without recurrence over a median follow up of 7 years. Radiotherapy (post-TSS) achieved remission in 3/5 patients (60 %) over a median follow up of 3.5 years. None of the patients received medical management at any point of time. Conclusion: Gigantism is more common in males and remission can be achieved in majority of the patients with the help of multimodality treatment (TSS and radiotherapy) 1).
Marques et al. report a five-generation kindred with two brothers with pituitary gigantism due to AIP mutation-positive GH secreting pituitary adenomas and their first-cousin coincidently also having gigantism due to Marfan syndrome 2).