Case Report: Neurosurgery, octreotide, cabergoline, radiotherapy, pasireotide and pegvisomant - a multimodal strategy for adolescent acrogigantism.
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Growth hormone (GH)-secreting pituitary neuroendocrine tumors (PitNETs) are rare in the pediatric population. They often present as large, invasive tumors and are frequently associated with an underlying genetic predisposition, making their management particularly challenging. Optimal treatment requires an individualized multidisciplinary approach, which often involves a combination of surgery and adjuvant medical therapy or radiotherapy when necessary. We present a challenging case of an adolescent female with acrogigantism. The patient was diagnosed at the age of 13 years and 10 months during the evaluation of chronic headaches. Brain magnetic resonance imaging revealed an invasive PitNET measuring 38 × 23 × 32 mm, while biochemical assessment confirmed marked GH hypersecretion. The disease followed an aggressive course, and biochemical control could not be achieved despite optimal multimodal therapy. Sequential treatment included neurosurgical resection, octreotide LAR, cabergoline, pasireotide LAR, proton beam radiotherapy, and pegvisomant. Combination therapy with pegvisomant and octreotide LAR ultimately normalized insulin-like growth factor 1 concentrations while maintaining long-term radiological tumor stability. This case underscores the complexity of managing aggressive GH-secreting PitNETs in adolescents and emphasizes the need for individualized, multidisciplinary care and long-term follow-up. Combined medical therapy with pegvisomant and octreotide LAR may provide effective biochemical control when surgery, radiotherapy, and standard medical treatment are insufficient.