颅咽管瘤分型

doi:10.3969/j.issn.1672-6731.2023.10.008

摘要/Abstract

摘要：

颅咽管瘤与周围神经血管粘连紧密，手术难度较大，围手术期并发症较多，患者多预后不良。基于肿瘤组织学或解剖学分型用于指导手术原则及手术入路，以及药物治疗或联合治疗方案研究较多，但尚未形成共识。本文综述颅咽管瘤的组织学、解剖学和分子分型，为今后实现颅咽管瘤的融合分型指导精准治疗提供新的思路。

关键词: 颅咽管瘤, 解剖学, 外科手术, 综述

Abstract:

The tumor tissue of craniopharyngioma is closely adherent to peripheral neurovascular tissue, so the operation to remove lesion is more difficult, the perioperative complications are very serious, and the prognosis of patients is poor. There are many studies on the use of tumor histology or anatomical classification to guide surgical principles and surgical approaches, as well as drug therapy or combination therapy, but no consensus has been reached. This paper reviews the histological, anatomical and molecular typing of craniopharyngioma, providing new ideas for the future realization of the fusion typing of craniopharyngioma to guide the precise treatment.

Key words: Craniopharyngioma, Anatomy, Surgical procedures, operative, Review

斯良楠, 陈金桃, 林志雄. 颅咽管瘤分型[J]. 中国现代神经疾病杂志, 2023, 23(10): 914-923.

Liang-nan SI, Jin-tao CHEN, Zhi-xiong LIN. The research of craniopharyngioma classification[J]. Chinese Journal of Contemporary Neurology and Neurosurgery, 2023, 23(10): 914-923.

http://journal11.magtechjournal.com/cjcnn/CN/Y2023/V23/I10/914

图/表 7

图 1 颅咽管瘤解剖学分型示意图（根据肿瘤与周围组织结构贴附位置共分为6型^[24]；橙色区域代表肿瘤组织，绿色区域代表下丘脑及垂体柄，粉色区域为腺垂体） 1a 蝶鞍垂体腺型：贴附于鞍膈以下，包括垂体腺与垂体柄的连接处 1b 垂体柄-漏斗外侧型：贴附于垂体柄或漏斗-灰结节外侧面 1c 第三脑室底型：所粘连结构包括漏斗-灰结节全层 1d 第三脑室底和第三脑室壁型：粘连于第三脑室底神经层，以及一处或多处第三脑室前壁或部分侧壁 1e 第三脑室内型：仅粘连于第三脑室壁室管膜层 1f 全面贴附型：肿瘤贴附于以上所有结构

Figure 1 CP classification according to anatomical structures attached to the tumor ^[24]. The orange area represents the tumor tissue, the green area represents the hypothalamus and the pituitary stalk, and the pink area is the adenohypophysis Sella-gland: tumor attachment occurs within the sella turcica, below the diaphragma sellae, including the junction of the sella-pituitary gland (PG) with the pituitary stalk (PS, Panel 1a). PS - infundibulum (outer aspect): attachment to the solid portion of the PS and the outer aspect of the infundibulum-tuber cinereum (Panel 1b). Third ventricle floor (TVF): attachment to the entire thickness of the infundibulum - tuber cineveum (Panel 1c). TVF and walls: attachment to the TVF and third ventricle walls (Panel 1d). Third ventricle lining: attachment to the ependymal lining of the third ventricle (Panel 1e). Global: attachment to all above structures (Panel 1f).

图 2 颅咽管瘤形态学分型示意图（根据肿瘤组织贴附形态共分为6型^[24]；橙色区域代表肿瘤组织，绿色区域代表下丘脑及垂体柄，粉色区域为腺垂体） 2a 带蒂型：贴附点由一根狭窄的纤维血管干构成 2b 无柄型：肿瘤贴附面如一个宽大的补丁，附着于周围组织结构 2c 帽状型：肿瘤贴附面宽广，上部（上1/3 ~ 1/2部）向上推挤第三脑室底壁，后者如同“戴帽子” 2d 环状型：周围组织结构与肿瘤接触面如带子般环绕肿瘤中心表面 2e 碗状型：肿瘤贴附面宽广，下部（下1/3 ~ 1/2部）向下挤压接触面组织结构，如“碗”状 2f 环包型：周围组织结构与肿瘤接触面（通常是一种很薄的神经胶质层）如“包装纸”样包裹肿瘤表面大部分

Figure 2 Morphological patterns of CP adherence^[24]. The orange area represents the tumor tissue, the green area represents the hypothalamus and the pituitary stalk, and the pink area is the adenohypophysis Pedicle pattern: the attachment consists of a narrow fibrovascular stem (Panel 2a). Sessile pattern: a wide patch of the tumor surface is attached to the anatomical structure (Panel 2b). Cap-like pattern: wide attachment between the upper third to upper half portion of a tumor pushing the TVF upward, which looks like wearing a hat (Panel 2c). Ring-like pattern: the attachment encircles the center of the tumor surface as a band (Panel 2d). Bowl- like pattern: wide attachment between the lower third to half portion of a tumor pushing the contiguous structures downward, like bowl (Panel 2e). Circumferential pattern: the attachment involves most of the tumor surface, usually as a very thin layer of nervous-gliotic tissue, like wrapping paper (Panel 2f).

图 3 颅咽管瘤粘连程度分型示意图（根据肿瘤与周围组织结构粘连程度共分为4型^[24]；橙色区域代表肿瘤组织，绿色区域代表下丘脑及垂体柄，粉色区域为腺垂体） 3a 疏松型：贴附疏松，术中通过轻柔牵拉或钝性分离即可剥离肿瘤与周围组织，同时完整保留正常组织结构 3b 紧密型：肿瘤与周围组织贴附紧密，需锐性分离方可剥离肿瘤与周围组织并保留正常组织结构 3c 融合型：肿瘤囊壁与周围组织结构沿贴附面相互融合，术中难以分离，即使谨慎锐性分离仍可造成正常组织结构损伤 3d 替换型：由于肿瘤细胞的侵袭，贴附于肿瘤的组织结构已丧失其完整性，无法辨认

Figure 3 Adhesion strength of CP^[24]. The orange area represents the tumor tissue, the green area represents the hypothalamus and the pituitary stalk, and the pink area is the adenohypophysis Loose adherence: the adherence is loose. The tumor can be easily separated from the attached structure by gentle tugging or by blunt dissection, preserving the integrity of the anatomical structure (Panel 3a). Tight adherence: the tumor adheres closely to the involved structure. Separation of the tumor requires the use of sharp dissection to preserve the integrity of the anatomical structure involved (Panel 3b). Fusion adherence: the CP capsule and the adjacent anatomical structure are fused together along the plane of attachment. No safe cleavage plane for tumor dissection can be identified, and even careful sharp dissection may cause damage to the normal tissue (Panel 3c). Replacement adherence: the anatomical structure adhered to the tumor has lost its integrity and is no longer recognizable, because it has been replaced or invaded by the CP (Panel 3d).

图 4 Morisako（2016年版）颅咽管瘤解剖学分型示意图（根据肿瘤所在位置共分为4型^[28]；橙色区域代表肿瘤组织，粉色区域代表腺垂体，黑点代表肿瘤起始点，红点代表前交通动脉） 4a，4b 鞍内型：肿瘤起源于垂体柄鞍内部分 4c，4d 视交叉前型：肿瘤起源于鞍上垂体柄前部，视交叉和前交通动脉均上抬 4e，4f 视交叉后型：肿瘤起源于鞍上垂体柄后部并主要向后生长，视交叉和前交通动脉位于正常解剖位置 4g，4h 第三脑室内型：肿瘤起源于第三脑室底

Figure 4 Morisako 2016 anatomical classification of CP ^[28]. The orange areas represent the tumor tissue, the pink areas represent the adenohypophysis, the black dots indicate the tumor origin, the red dots indicate the anterior communicating artery Intrasellar type: the tumor originates from the intrasellar part of the pituitary stalk (Panel 4a, 4b). Prechiasmatic type: the tumor originates from the anterior part of the pituitary stalk and grows upward. Both of the optic chiasm and the anterior communicating artery are elevated upward in this lesion type (Panel 4c, 4d). Retrochiasmatic type: the tumor originates from the posterior part of the pituitary stalk and grows up toward the posterior fossa. Both of the optic chiasm and the anterior communicating artery remain in their normal positions (Panel 4e, 4f). Intra-third ventricle type: the tumor originates from the floor of the third ventricle (Panel 4g, 4h).

图 5 神经内镜辅助手术的颅咽管瘤Tang分型（2018年版）示意图（根据肿瘤与垂体柄关系分型^[29]；橙色区域代表肿瘤组织，黄点代表视神经，粉色区域代表腺垂体，红点代表颈内动脉，蓝色区域代表海绵窦） 5a，5b 中央型：肿瘤于下丘脑-垂体柄轴内居中生长，无法观察到明确的起源点 5c，5d 偏侧型：肿瘤沿下丘脑-垂体柄轴偏外侧生长，垂体被推向肿瘤一侧（前、后、左、右均可），于下丘脑-垂体柄轴可见明确的肿瘤起源点

Figure 5 The novel endoscopic classification of CP (Tang 2018)^[29]. The orange areas represent tumor tissue, the yellow dots indicate the optic nerve, the pink areas represent adenohypophysis, the red dots indicate the internal carotid artery, the blue areas represent the cavernous sinus Central type CP grows within and along the hypothalamic - pituitary stalk axis and no pedicle or definite origin site can be identified (Panel 5a, 5b). Peripheral type CP arises from the hypothalamic - pituitary stalk axis but expands and grows laterally in an exophytic pattern, the pituitary is usually displaced to circumferential surface of the tumor (Panel 5c, 5d).

图 6 神经内镜辅助手术的颅咽管瘤Tang分型（2018年版）示意图（根据肿瘤在下丘脑-垂体柄轴上的起源点分为3型^[29]；红点代表前交通动脉，黑点代表肿瘤起源点，粉色区域代表腺垂体） 6a 下丘脑垂体柄型：肿瘤起源于漏斗-灰结节，可向上扩展生长侵入第三脑室和（或）向下扩展进入鞍上池 6b 鞍上垂体柄型：肿瘤起源于垂体柄鞍上池部分，以鞍上池下段更为常见，于鞍上池第三脑室外生长 6c 鞍内垂体柄型：肿瘤起源于垂体柄鞍内部分，可向上生长或向蝶窦、海绵窦方向生长

Figure 6 The novel endoscopic classification in different origin sites of 3 subtypes of peripheral CP (Tang 2018) along the hypothalamic-pituitary stalk axis^[29]. The red dots indicate the anterior communicating artery, the black spots indicate the tumor origin, the pink areas represent adenohypophysis Hypothalamic stalk CP originates from the infundibulum-tuber cinereum, which usually extends up to the third ventricle and/or down to the suprasellar (Panel 6a). Suprasellar stalk CP derives from pituitary stalk suprasellar segment, usually low portion of the suprasellar, and commonly locates extraventriclely (Panel 6b). Intrasellar stalk CP originates from the part of pituitary stalk under diaphragma, which may grow upward, or toward the sphenoid sinus and cavernous sinus (Panel 6c).

图 7 颅咽管瘤QST分型^[31]（橙色区域代表肿瘤组织，粉色区域代表腺垂体） 7a Q型：肿瘤起源于鞍膈下区域 7b S型：肿瘤起源于鞍上垂体柄区域 7c T型：肿瘤起源于漏斗-灰结节

Figure 7 The CP of QST classification ^[31]. The orange areas represent the tumor tissue, and the pink areas indicate the adenohypophysis Type Q of CP originates below the diaphragmatic area (Panel 7a). Type S of CP originates from the pituitary talk area (Panel 7b). Type T of CP originates from the infundibulum-tuber cinereum (Panel 7c).

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