Department of Neurology, Saint Louis University, St. Louis, MO, USA.
Department of Pathology, Saint Louis University, St. Louis, MO, USA.
Br J Neurosurg. 2023 Dec;37(6):1886-1892. doi: 10.1080/02688697.2020.1834509. Epub 2020 Oct 16.
Inflammatory pseudotumors (IPTs) are rare benign conditions of unknown etiology that can affect any part of the body. IPTs are most commonly associated with Immunoglobulin G4 (IgG4)-related disease. Central nervous system IPTs, especially with pituitary involvement, are even rarer entities. The presence of an IgG4-negative pituitary IPT with simultaneous extracranial involvement has not been reported.
We present the case of a 41-year-old female with past medical history of rheumatoid arthritis and a diagnosis of pituitary IPT with coexisting sphenoidal (extracranial) involvement mimicking a pituitary macroadenoma at presentation. The patient underwent multiple consecutive biopsies, and an extensive workup prior to establishing the diagnosis. Laboratory work-up showed normal serum IgG4 and unremarkable liver function tests.
Pituitary lesions with simultaneous sphenoidal involvement in patients with IgG4-negative systemic inflammatory disease should raise the clinical suspicion for intracranial IPTs, as these tumors can mimic aggressive counterparts causing adjacent bony erosion, and local invasion.
炎性假瘤（IPT）是一种罕见的病因不明的良性疾病，可影响身体的任何部位。IPT 最常与 IgG4 相关疾病相关。中枢神经系统 IPT，特别是伴有垂体受累的，更为罕见。目前尚未报道 IgG4 阴性的伴有同时性颅外受累的垂体 IPT。
我们报告了一例 41 岁女性病例，既往有类风湿关节炎病史，诊断为垂体 IPT，同时伴有蝶窦（颅外）受累，表现为垂体大腺瘤。患者在确诊前经历了多次连续活检和广泛的检查。实验室检查显示血清 IgG4 正常，肝功能检查无明显异常。
在 IgG4 阴性的全身性炎症性疾病患者中，伴有同时性蝶窦受累的垂体病变应引起对颅内 IPT 的临床怀疑，因为这些肿瘤可能类似于侵袭性肿瘤，导致相邻骨侵蚀和局部侵犯。
