Coexistence of Craniopharyngioma and Cranial Fibrous Dysplasia: A Clinicopathological Study of 5 Cases and Literature Review

Abstract

Background: Craniopharyngioma (CP) and cranial fibrous dysplasia (CFD) are both rare embryonic benign cranial diseases that most commonly present during childhood or adolescence. Co-existence of CP and CFD is extremely rare, which has never been reported before.Methods: We retrospectively reviewed the data of 5 patients coexisted with CP and CFD in Beijing Tiantan Hospital from January 2003 to January 2021. Their clinicopathological features, treatment modalities, and outcomes were summarized. Moreover, a comprehensive literature review was conducted, and in order to explore the potential connection leading to this coexistence, the CFD characteristic GNAS gene and corresponding Gsα protein were tested in the CPResults: There were 4 males and 1 female (median age, 39 years) in the present series. The symptoms mainly included headache, dizziness, fatigue, polyuria/polydipsia, hypogonadism and blurred vision. Sphenoidal bone is the most common involved bone by CFD (n =4). Four patients had undergone surgery to remove the CP (1 transsphnoid and 3 transcranial). Complete and subtotal resection were achieved in 2 cases respectively. The tumor subtypes were 3 adamantinomatous, 1 unknown subtype. The common postoperative complications are pan-hypopituitarism, diabetes insipidus, and hypothyroidism. The mean follow-up time was 57.2 months. Postoperative hormone replacement was required in 2 patients. 3 patients underwent a genetic study of tumor specimens. GNAS mutations were not detected, but they were positive for Gsα protein. Conclusions: Though the definite causative relationship has not been proved, the coexistence of CP and CFD should not completely be excluded potential interplay or atypical FD course for the uncommon manifestations of CPs. Prompt diagnosis and appropriate treatment are more challenging than for solitary CPs for the deformations of skull base, as of now, management strategies are aimed at surgical treating the CP and regularly monitoring the CFD.