Higher surgical cure of pediatric gigantism with endoscopic endonasal surgery: case series and review of the literature.

OBJECTIVES

Pediatric gigantism associated with somatotropinomas is exceedingly rare. Although transsphenoidal surgery (TSS) constitutes first-line management, tumor recurrence is common. However, it remains unclear how surgical cure rates differ between transsphenoidal microsurgery (TMS) and the more contemporary endoscopic endonasal approach (EEA).

METHODS

Three children with somatotropinoma-induced gigantism who underwent EEA between December 2010 and October 2021 were identified via retrospective chart review. Primary outcomes collected included complications, length of stay (LOS), and rates of postoperative biochemical remission according to the 2010 Acromegaly Consensus Group criteria. A literature review compiled surgical cure rates for pediatric gigantism, stratified by TSS technique (TMS vs EEA).

RESULTS

Three children (2F, 1 M) presented with clinical signs of gigantism at an average age of 12 years [range: 10-14y]. Two patients failed prior somatostatin analogue therapy and one underwent attempted but incomplete EEA at an outside institution. Mean tumor diameter was 0.9 ± 0.2 cm. EEA was performed without complications and a mean LOS of 4.6 days [range: 3-7d]. Biochemical remission was achieved in all patients without adjuvant therapy during follow-up [range: 1-11y]. Nineteen historical studies report surgical outcomes for 325 total pediatric patients with gigantism. Overall surgical cure rates are estimated at 24.7% (73/296). Of these, 17 studies distinguished between transsphenoidal approaches, reporting surgical cure rates of 20.5% (54/264) with TMS and 66.7% (6/9) for EEA. Rates of hypopituitarism/diabetes insipidus were notably higher in TMS compared with EEA patients (31.1% vs 6.8%).

CONCLUSION

Pediatric gigantism is a rare clinical entity associated with historically low surgical cure rates. Literature review indicates that EEA is associated with higher cure rates, which is further supported by our small series in which all patients were cured. The surgical cure rate for gigantism may be higher in the contemporary endoscopic era and merits further study with larger populations of patients.