Endocrinology, Department of Translational Medicine, University of Piemonte Orientale, Novara, Italy.
Endocrinology, Diabetology and Metabolism, Department of Medical Sciences, University of Torino, Italy.
Endocr Pract. 2020 Nov;26(11):1337-1350. doi: 10.4158/EP-2020-0286.
Differential diagnosis of nonadenomatous sellar masses causing hypopituitarism is still a challenge. Among these masses, growing evidence has demonstrated that primary pituitary lymphoma is a specific and emerging entity. The aim of our study was to describe our experience with a case of primary pituitary lymphoma and to perform a review of the available literature.
We searched relevant databases up to March 2020, identifying 36 suitable articles basing on inclusion criteria (primary pituitary lymphoma in adult immunocompetent subjects). Overall, 43 cases were included in the review, adding a new case diagnosed and treated in our hospital. Epidemiologic data, clinical presentation, hormonal status, radiologic findings, pathology, treatment, and outcome were extracted.
Mean age at diagnosis was 58.9 years, without gender difference. Symptoms related to mass were common (52.3%), in particular cranial nerve palsy (70.5%), headache (56.8%), and alteration in visual field (40.9%). Impaired hormonal status was detected in 89.7% of patients; of them, 58.9% presented with anterior pituitary failure (partial or total), while 25.6% presented with panhypopituitarism. Overall, diabetes insipidus was present in 30.8% and hyperprolactinemia in 41.0% of patients. The majority of patients presented a radiologically invasive mass in the suprasellar region and cavernous sinus (65.9% and 40.9%, respectively) and histologic diagnosis of diffuse B-cell lymphoma (54.5%).
The differential diagnosis of sellar and parasellar masses causing hypopituitarism should include primary pituitary lymphoma, even in absence of systemic symptoms or posterior pituitary dysfunction. The disease has a heterogeneous pattern, so a collaboration between endocrinologists, neuroradiologists, neurosurgeons, and hematologists is desirable.
非腺瘤性鞍区肿块导致垂体功能减退的鉴别诊断仍然是一个挑战。在这些肿块中，越来越多的证据表明原发性垂体淋巴瘤是一种特异性和新兴的实体。我们研究的目的是描述我们在一例原发性垂体淋巴瘤病例中的经验，并对现有文献进行回顾。
我们搜索了截至 2020 年 3 月的相关数据库，根据纳入标准（成人免疫功能正常的原发性垂体淋巴瘤）确定了 36 篇合适的文章。总体上，在综述中纳入了 43 例病例，并增加了我们医院诊断和治疗的 1 例新病例。提取了流行病学数据、临床表现、激素状态、影像学发现、病理学、治疗和结果。
诊断时的平均年龄为 58.9 岁，无性别差异。与肿块相关的症状很常见（52.3%），特别是颅神经麻痹（70.5%）、头痛（56.8%）和视野改变（40.9%）。89.7%的患者存在激素功能障碍；其中，58.9%表现为前垂体功能衰竭（部分或全部），25.6%表现为全垂体功能减退。总体而言，30.8%的患者存在尿崩症，41.0%的患者存在高催乳素血症。大多数患者表现为鞍上和海绵窦侵袭性肿块（分别为 65.9%和 40.9%），组织学诊断为弥漫性 B 细胞淋巴瘤（54.5%）。
导致垂体功能减退的鞍区和鞍旁肿块的鉴别诊断应包括原发性垂体淋巴瘤，即使没有全身症状或后叶垂体功能障碍。该疾病具有异质性模式，因此内分泌学家、神经放射学家、神经外科医生和血液科医生之间的合作是必要的。
