Craniopharyngioma: a clinicopathological analysis of 71 cases
10.3760/cma.j.cn112151-20250208-00084
- VernacularTitle:颅咽管瘤71例临床病理学分析
- Author:
Xiaoyu YANG
1
;
Yujie LI
1
;
Chong GE
1
;
Yuan LI
1
;
Haibo WU
1
Author Information
1. 中国科学技术大学附属第一医院(安徽省立医院)临床病理中心 中国科学技术大学智慧病理学研究所,合肥230036
- Publication Type:Journal Article
- Keywords:
Craniopharyngioma;
Proto-oncogene proteins B-raf;
beta Catenin;
Immunohistochemistry;
Diagnosis, differential
- From:
Chinese Journal of Pathology
2025;54(8):798-804
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To investigate the clinicopathological characteristics of adamantinomatous craniopharyngioma (ACP) and papillary craniopharyngioma (PCP).Methods:A total of 71 cases craniopharyngioma, included 52 cases of ACP and 19 cases of PCP, diagnosed at the First Affiliated Hospital of University of Science and Technology of China (Anhui Provincial Hospital), Hefei, China from September 2019 to November 2023 were collected. Clinical pathological data were analyzed, immunohistochemical staining was performed, and mutations in the CTNNB1 and BRAF V600E genes were examined to identify differences between ACP and PCP.Results:The ACP cohort comprised 27 male and 25 female patients, with an age at onset ranging from 6 to 70 years, mean age (42.0±18.3) years. In contrast, the PCP group included 15 males and 4 females, with an age at onset spanning 28 to 74 years, mean age (51.0±13.3) years. The ACP group more commonly showed calcifications on imaging than the PCP group [92.3% (48/52) versus 11/19]. Partial tumor resection and the maximum diameter of the tumor were important factors affecting the recurrence of ACP. Whorled cell clusters, wet keratinization, stellate reticulum, cysts, and calcification were more often seen in ACP than PCP ( P<0.05). Immunohistochemically, all (100%,52/52) of the ACP showed nuclear β-catenin expression, with varying degrees of expression in the nodular whorls, and scattered cytoplasmic β-catenin expression. The BRAF V600E expression was found in the cytoplasm of all (19/19) PCP cases, but only non-specific BRAF V600E nuclear positivity was observed in ACP cases. Molecular testing showed that the mutation rate of the CTNNB1 gene in ACP was 22.7% (5/22), and the mutation rate of the BRAF V600E in PCP was 19/19. Conclusions:ACP and PCP have different age at onset, radiological features, histopathological morphology, and genetic alterations. Proper use and interpretation of immunohistochemical results can help distinguish between ACP and PCP, while molecular testing can be used as an auxiliary diagnostic modality.