Abushamat Layla A, Kerr Janice M, Lopes M Beatriz S, Kleinschmidt-DeMasters Bette K
Department of Endocrinology, University of Colorado Health Sciences Center, University of Colorado Denver, Anschutz Medical Campus, Aurora, Colorado.
Department of Pathology (Neuropathology) and Neurological Surgery, University of Virginia, Charlottesville, Virginia.
J Neuropathol Exp Neurol. 2019 Aug 1;78(8):673-684. doi: 10.1093/jnen/nlz044.
The cause of sellar region masses in large retrospective series is overwhelmingly pituitary adenomas (84.6%), followed by craniopharyngiomas (3.2%), cystic nonneoplastic lesions (2.8%), inflammatory lesions (1.1%), meningiomas (0.94%), metastases (0.6%), and chordomas (0.5%) (1). While other rare lesions were also identified (collectively 6.0%), single unusual entities in the above-cited series numbered <1-2 examples each out of the 4122 cases, underscoring their rarity. We searched our joint files for rare, often singular, sellar/suprasellar masses that we had encountered over the past several decades in our own specialty, tertiary care specialty pituitary center practices. Cases for this review were subjectively selected for their challenging clinical and/or histological features as well as teaching value based on the senior authors' (MBSL, BKD) collective experience with over 7000 examples. We excluded entities deemed to be already well-appreciated by neuropathologists such as mixed adenoma-gangliocytoma, posterior pituitary tumors, metastases, and hypophysitis. We identified examples that, in our judgment, were sufficiently unusual enough to warrant further reporting. Herein, we present 3 diffuse large cell B cell pituitary lymphomas confined to the sellar region with first presentation at that site, 2 sarcomas primary to sella in nonirradiated patients, and 1 case each of granulomatosis with polyangiitis and neurosarcoidosis with first presentations as a sellar/suprasellar mass. Other cases included 1 of chronic lymphocytic leukemia within a gonadotroph adenoma and 1 of ectopic nerve fascicles embedded within a somatotroph adenoma, neither of which impacted patient care. Our objective was to share these examples and review the relevant literature.
在大型回顾性系列研究中，鞍区肿物的病因绝大多数是垂体腺瘤（84.6%），其次是颅咽管瘤（3.2%）、囊性非肿瘤性病变（2.8%）、炎性病变（1.1%）、脑膜瘤（0.94%）、转移瘤（0.6%）和弦瘤（0.5%）（1）。虽然也发现了其他罕见病变（总计6.0%），但在上述系列研究的4122例病例中，每种罕见病变的单一不寻常实体均不到1 - 2例，突出了它们的罕见性。我们在我们联合的档案中搜索过去几十年在我们自己的专科、三级医疗专科垂体中心实践中遇到的罕见的、通常为单一的鞍区/鞍上肿物。基于资深作者（MBSL、BKD）对7000多例病例的集体经验，本次综述的病例因其具有挑战性的临床和/或组织学特征以及教学价值而被主观选择。我们排除了神经病理学家认为已充分认识的实体，如混合性腺瘤 - 神经节细胞瘤、垂体后叶肿瘤、转移瘤和垂体炎。我们确定了一些我们认为足够不寻常、值得进一步报告的病例。在此，我们报告3例局限于鞍区且首次在该部位出现的弥漫性大B细胞垂体淋巴瘤，2例非放疗患者鞍区原发的肉瘤，以及各1例以鞍区/鞍上肿物首次出现的肉芽肿性多血管炎和神经结节病。其他病例包括1例促性腺激素腺瘤内的慢性淋巴细胞白血病和1例生长激素腺瘤内嵌入的异位神经束，这两例均未影响患者的治疗。我们的目的是分享这些病例并回顾相关文献。
