Acromegaly in preadolescence: A case report of a 9-year-old boy with acromegaly

Abstract

Background: Acromegaly has been reported in adolescents and young adults, but it is unusual in preadolescence. Diagnosis and management pose different challenges in this age group. Here, we present a rare case of acromegaly in preadolescence.

Case description: A 9-year-old boy presented with acromegalic features and MRI revealed a pituitary tumor. He was diagnosed as having growth hormone secreting pituitary adenoma based on the multidisciplinary assessment, and underwent gross total tumor resection through an endoscopic endonasal approach (EEA) with subsequent normalization of the hormonal parameters.

Conclusions: Advances in EEA have made safe removal and cure possible even in children. However, long- term follow-up is needed in such younger patients with multidisciplinary management by neurosurgeons, endocrinologists, pediatricians, and ENT surgeons.

Keywords: Acromegaly; Endoscopic endonasal approach; Growth hormone secreting pituitary adenoma; Preadolescence.

Figure 1:

Preoperative MRI showing a sellar mass lesion with less enhancement with gadolinium (a). Postoperative T1- weighted postcontrast image showing complete tumor removal with preservation of the pituitary gland and stalk (b). Skull X-ray showing prominence of the jaw and mild frontal bossing (c). Hand X-ray revealing cauliflower appearance at the distal phalanx of the hands (d).

Figure 2:

Intraoperative photographs. The stretched anterior pituitary lobe had covered the tumor (a). The tumor was resected in a double-suction technique intracapsularly (b). The pseudocapsule was resected following internal debulking of the tumor (c). A fat graft was packed into the cavity with a dural stitch following tumor resection (d).

Figure 3:

Histopathological photographs. The tumor was composed of monomorphic neoplastic cells with eosinophilic cytoplasm (H and E, ×100) (a). Most of the neoplastic cells showed immunoreactivity for GH in their cytoplasm (immunohistochemistry, ×100) (b). The Ki-67 proliferation index was 3% (22/725) (immunohistochemistry, ×100) (c).