Bose Sayantan, Balogun James, du Plessis Daniel, Bailey Matthew, Lalloo Fiona, Pathmanaban Omar
Department of Neurosurgery, Salford Royal NHS Foundation Trust, Salford, United Kingdom.
Department of Cellular Pathology, Salford Royal NHS Foundation Trust, Salford, United Kingdom.
Surg Neurol Int. 2024 Sep 20;15:340. doi: 10.25259/SNI_315_2024. eCollection 2024.
Craniopharyngiomas are benign tumors arising in the sellar and suprasellar regions. Although ectopic tumors do occur, it is usually due to local spread or recurrent tumors. Purely ectopic cerebellopontine angle (CPA) or 4 ventricle tumors are extremely rare and have been found to be significantly associated with familial adenomatous polyposis (FAP), a genetic disorder.
Only four cases of ectopic CPA craniopharyngioma associated with FAP have been reported to date. Here, we present the 5 case of ectopic CPA craniopharyngioma on a background of FAP. The previously described cases have been elaborated as well.
CPA tumor with a background of FAP should raise a differential diagnosis of craniopharyngioma, and similarly, a CPA primary ectopic craniopharyngioma may raise suspicion of underlying APC gene mutation.
颅咽管瘤是起源于鞍区和鞍上区域的良性肿瘤。虽然异位肿瘤确实会发生，但通常是由于局部扩散或复发性肿瘤。单纯的异位小脑脑桥角（CPA）或第四脑室肿瘤极为罕见，并且已发现与遗传性疾病家族性腺瘤性息肉病（FAP）显著相关。
迄今为止，仅报道了4例与FAP相关的异位CPA颅咽管瘤病例。在此，我们报告第5例以FAP为背景的异位CPA颅咽管瘤病例。同时也对先前描述的病例进行了阐述。
以FAP为背景的CPA肿瘤应考虑颅咽管瘤的鉴别诊断，同样，原发性异位CPA颅咽管瘤可能提示潜在的APC基因突变。
