Abstract：
OBJECTIVE: Basal duct-like recess (DR) sign serves as a specific marker of papillary craniopharyngiomas (PCPs) of the strictly third-ventricular (3 V) topography. Origins of this sign are poorly understood with limited validation in external cohorts.
METHODS: In this retrospective study, MRIs of pathologically proven PCPs were reviewed and evaluated for tumor topography, DR sign prevalence, and morphological subtypes.
RESULTS: Twenty-three cases with 24 MRIs satisfied our inclusion criteria. Median age was 44.5 years with a predominant male distribution (M/F ratio 4.7:1). Overall, strictly 3 V was the commonest tumor topography (8/24, 33.3%), and tumors were most commonly solid-cystic (10/24, 41.7%). The prevalence of DR sign was 21.7% (5/23 cases), all with strictly 3 V topography and with a predominantly solid consistency. The sensitivity, specificity and positive and negative predictive value of the DR sign for strict 3 V topography was 62.5%, 100%, 100% and 84.2% respectively. New pertinent findings associated with the DR sign were observed in our cohort. This included development of the cleft-like variant of DR sign after a 9-year follow-up initially absent at baseline imaging. Additionally, cystic dilatation of the basal tumor cleft at the pituitary stalk-tumor junction and presence of a vascular structure overlapping the DR sign were noted. Relevant mechanisms, hypotheses, and implications were explored.
CONCLUSIONS: We confirm the DR sign as a highly specific marker of the strictly 3 V topography in PCPs. While embryological and molecular factors remain pertinent in understanding origins of the DR sign, non-embryological mechanisms may play a role in development of the cleft-like variant.
METHODS: In this retrospective study, MRIs of pathologically proven PCPs were reviewed and evaluated for tumor topography, DR sign prevalence, and morphological subtypes.
RESULTS: Twenty-three cases with 24 MRIs satisfied our inclusion criteria. Median age was 44.5 years with a predominant male distribution (M/F ratio 4.7:1). Overall, strictly 3 V was the commonest tumor topography (8/24, 33.3%), and tumors were most commonly solid-cystic (10/24, 41.7%). The prevalence of DR sign was 21.7% (5/23 cases), all with strictly 3 V topography and with a predominantly solid consistency. The sensitivity, specificity and positive and negative predictive value of the DR sign for strict 3 V topography was 62.5%, 100%, 100% and 84.2% respectively. New pertinent findings associated with the DR sign were observed in our cohort. This included development of the cleft-like variant of DR sign after a 9-year follow-up initially absent at baseline imaging. Additionally, cystic dilatation of the basal tumor cleft at the pituitary stalk-tumor junction and presence of a vascular structure overlapping the DR sign were noted. Relevant mechanisms, hypotheses, and implications were explored.
CONCLUSIONS: We confirm the DR sign as a highly specific marker of the strictly 3 V topography in PCPs. While embryological and molecular factors remain pertinent in understanding origins of the DR sign, non-embryological mechanisms may play a role in development of the cleft-like variant.
摘要：
目的：基底导管样隐窝（DR）征是严格的第三心室（3V）地形图的乳头状颅咽管瘤（PCPs）的特异性标记。由于外部队列的验证有限，因此对该标志的起源知之甚少。
方法：在这项回顾性研究中，对病理证实的PCP进行了MRI检查，并评估了肿瘤形貌，DR体征患病率,和形态亚型。
结果：23例24个MRI符合纳入标准。中位年龄为44.5岁，男性占主导地位（M/F比4.7：1）。总的来说,严格地说，3V是最常见的肿瘤地形图(8/24，33.3%)，肿瘤最常见的是实性囊性（10/24，41.7%）。DR征象的患病率为21.7%(5/23例),所有这些都具有严格的3V地形，并且具有主要的固体稠度。敏感性,DR征对严格的3V地形的特异性和阳性和阴性预测值为62.5%，100%,分别为100%和84.2%。在我们的队列中观察到与DR征象相关的新发现。这包括在基线成像时最初没有进行9年随访后，出现了DR征的裂隙样变体。此外,注意到垂体柄-肿瘤交界处基底肿瘤裂隙的囊性扩张，以及与DR征重叠的血管结构的存在。相关机制,假设，并探讨了其含义。
结论：我们确认DR征象是PCP中严格的3V形貌的高度特异性标记。虽然胚胎学和分子因素在理解DR体征的起源方面仍然相关，非胚胎机制可能在裂隙样变异体的发育中起作用。
方法：在这项回顾性研究中，对病理证实的PCP进行了MRI检查，并评估了肿瘤形貌，DR体征患病率,和形态亚型。
结果：23例24个MRI符合纳入标准。中位年龄为44.5岁，男性占主导地位（M/F比4.7：1）。总的来说,严格地说，3V是最常见的肿瘤地形图(8/24，33.3%)，肿瘤最常见的是实性囊性（10/24，41.7%）。DR征象的患病率为21.7%(5/23例),所有这些都具有严格的3V地形，并且具有主要的固体稠度。敏感性,DR征对严格的3V地形的特异性和阳性和阴性预测值为62.5%，100%,分别为100%和84.2%。在我们的队列中观察到与DR征象相关的新发现。这包括在基线成像时最初没有进行9年随访后，出现了DR征的裂隙样变体。此外,注意到垂体柄-肿瘤交界处基底肿瘤裂隙的囊性扩张，以及与DR征重叠的血管结构的存在。相关机制,假设，并探讨了其含义。
结论：我们确认DR征象是PCP中严格的3V形貌的高度特异性标记。虽然胚胎学和分子因素在理解DR体征的起源方面仍然相关，非胚胎机制可能在裂隙样变异体的发育中起作用。
