Yu Xin, Liu Rui, Wang Yaming, Wang Hongwei, Zhao Hulei, Wu Zhaohun
Department of Neurosurgery, Navy General Hospital, Beijing, China.
Clin Neurol Neurosurg. 2012 Feb;114(2):112-9. doi: 10.1016/j.clineuro.2011.09.010. Epub 2011 Oct 21.
Infrasellar craniopharyngioma (IC) is a rare tumor. This study aimed to investigate the clinical manifestations, treatment methods, and prognosis of IC, which invades the cranial base, nasal sinuses, nasopharynx and clivus.
Eleven consecutive cases of IC who received treatment in People's Liberation Army Navy General Hospital from 1988 to 2007 were retrospectively analyzed, and the clinical manifestations of IC were summarized. At the same time, literature pertinent to IC was reviewed. These patients consisted of six males and five females with an average age of 28.5 years (range 7-52 years old). Among them, nine cases were identified as simple IC and two cases as suprasellar and IC. Clinical manifestations included headache (seven cases), nasal obstruction (four cases), polydipsia and polyuria (four cases), visual disorder (five cases), delayed sex organ development (three cases), menstrual disorder (one case) and no symptoms (one case). Tumor invasion regions included sellar bottom, ethmoidal sinus, maxillary sinus, sphenoidal sinus, infrasellar region, clivus, nasopharynx and nasal cavity. Solid craniopharyngioma was observed in three cases, cystic craniopharyngioma in seven cases, and mixed cystic and solid type in one case. Four cases underwent craniotomy for tumor resection (three cases also received adjuvant external beam radiation therapy), two cases underwent transnasal approach tumor resection under endoscope guidance (one case simultaneously received adjuvant interstitial brachytherapy) and four cases underwent stereotactic interstitial radiation (radioisotope (32)P brachytherapy).
All cases were followed up for an average of 22.5 years (range 9-98 months). Imaging results showed that tumors disappeared in one case, were clearly reduced in eight cases and were stable in two cases. Clinical symptoms disappeared in three cases, and improved in seven cases. No symptoms appeared in the case presenting with no symptoms. All patients were able to resume work, study and daily tasks.
IC is rare (the present cases account for 0.23% of all retrieved cases). Its chief clinical manifestations include headache, nasal obstruction, polydipsia and polyuria, and visual disorder. Lesions include solid, cystic, and mixed cystic and solid types. It is very difficult to resect the whole diseased region because this disease invades the cranial base, nasal sinuses and nasopharynx. Individualized treatments should be used according to lesion characteristics and invasion range, for example, stereotaxic interstitial brachytherapy. Radical resection or partial resection plus external beam radiation therapy produces better prognosis in IC than intracranial craniopharyngioma.
鞍下颅咽管瘤（IC）是一种罕见肿瘤。本研究旨在探讨侵犯颅底、鼻窦、鼻咽和斜坡的IC的临床表现、治疗方法及预后。
回顾性分析1988年至2007年在解放军海军总医院接受治疗的11例连续性IC病例，总结IC的临床表现。同时，复习与IC相关的文献。这些患者包括6例男性和5例女性，平均年龄28.5岁（范围7 - 52岁）。其中，9例被诊断为单纯IC，2例为鞍上及IC。临床表现包括头痛（7例）、鼻塞（4例）、多饮多尿（4例）、视力障碍（5例）、性器官发育延迟（3例）、月经紊乱（1例）及无症状（1例）。肿瘤侵犯区域包括鞍底、筛窦、上颌窦、蝶窦、鞍下区域、斜坡、鼻咽和鼻腔。观察到3例为实性颅咽管瘤，7例为囊性颅咽管瘤，1例为囊实性混合型。4例行开颅肿瘤切除术（3例还接受辅助外照射放疗），2例在内镜引导下行经鼻入路肿瘤切除术（1例同时接受辅助组织间近距离放疗），4例行立体定向组织间放疗（放射性核素（32）P近距离放疗）。
所有病例平均随访22.5年（范围9 - 98个月）。影像学结果显示，1例肿瘤消失，8例明显缩小，2例稳定。3例临床症状消失，7例改善。无症状病例未出现症状。所有患者均能恢复工作、学习和日常活动。
IC罕见（本病例占所有检索病例的0.23%）。其主要临床表现包括头痛、鼻塞、多饮多尿及视力障碍。病变包括实性、囊性及囊实性混合型。由于该疾病侵犯颅底、鼻窦和鼻咽，很难切除整个病变区域。应根据病变特点和侵犯范围采用个体化治疗，如立体定向组织间近距离放疗。与颅内颅咽管瘤相比，根治性切除或部分切除加外照射放疗在IC中预后较好。
