Papillary craniopharyngioma: A clinicopathologic study of a rare entity from a major tertiary care center in Pakistan

Papillary craniopharyngioma (PCP) are uncommon variants of craniopharyngiomas (CP), which are benign epithelial neoplasms of the sellar and suprasellar region. Histologically, PCPs are typically composed of well-differentiated stratified squamous epithelium; however, focal variations are not uncommon. A distinction from other lesions of the region, despite being difficult to achieve due to the overlapping radiological and clinical features, is important for adequate treatment to be administered. Our aim was to study the clinical and histological features of PCP with emphasis on features that are helpful in its distinction from other lesions that are similar in appearance. We reviewed 13 cases of PCP diagnosed in our institution between January 2010 and December 2015. The mean age at presentation was 30.76 years. Two of the patients belonged to the pediatric age group. Male-to-female ratio was 2.25:1. Suprasellar location (either alone or in combination with sellar region) was the most common tumor site. Microscopically, all of the cases showed stratified squamous epithelium with frequent pseudopapillae formation. Focal adamantinomatous epithelium and columnar epithelium with variable cilia and goblet cells were seen in 4 (30.7%) cases. Brain invasion was observed in 3 (23%) cases. Four patients died of their disease; 2 of the 7 patients with an available follow up, developed recurrences; and, 5 experienced severe postoperative morbidity. Majority of the PCPs exhibited typical features with minor variations. Knowledge of the variations in histologic features helps in reaching the correct diagnosis. These tumors can behave aggressively with a high recurrence rate and decreased overall survival.