Recurrent adamantinomatous craniopharyngioma: Case report of an uncommon tumor, and a brief review of the literature

Adamantinomatous craniopharyngioma (ACP) is a rare, WHO type I, benign but aggressive tumor, associated with a known challenging diagnosis. It overwhelmingly occurs in the pediatric population. ACP is known for being locally aggressive, with a high recurrence rate and long-term morbidity. We present a case of a 12-year-old boy who was diagnosed with ACP after a long journey with symptoms lasting three years. On physical examination, there was evidence of raised intracranial pressure, delayed puberty, and signs of central diabetes insipidus. MRI and CT scans revealed a huge multicystic, focally calcified suprasellar mass leading to hydrocephalus. The imaging features, in addition to the clinical and laboratory data, indicated a craniopharyngioma.

The patient was subjected to endoscopic transsphenoidal resection that was partially successful since the tumor was firmly adherent to the surrounding structures. Histopathologically, the tumor features were consistent with the diagnosis of ACP. Immunohistochemistry (IHC) and molecular analysis showing the WNT signaling pathway activation due to the mutation of CTNNB1 confirmed the definitive diagnosis. The postoperative treatment involved external beam radiotherapy and a multidisciplinary follow-up of endocrine and visual sequelae. The patient developed recurrence of the tumor after three years of treatment, with a subtotal resection being done once again. He was still under active follow-up at the four-year follow-up, with the emphasis on long-term disease control and quality of life.

This case highlights the significance of early detection of silent neuroendocrine symptoms, precision in imaging analysis, and the multidisciplinary management strategy in the treatment of pediatric ACP, which has a high recurrence rate and long-term consequences.