Department of Diagnostic Radiology, National Cancer Institute, Cairo University, Cairo, Egypt.
AJR Am J Roentgenol. 2011 Feb;196(2):W187-93. doi: 10.2214/AJR.10.4778.
Rosai-Dorfman disease is a rare disorder characterized histologically by lymphatic sinus dilatation due to histiocyte proliferation. Our goal was to describe the CT, MRI, and (18)F-FDG (FDG) PET findings in a series of patients with this diagnosis.
We retrospectively reviewed the imaging studies of 10 patients with pathologically confirmed Rosai-Dorfman disease who were treated in our institution between January 2004 and December 2007.
We found the following areas of general involvement: three intracranial, seven head and neck, and three spinal, with some patients having more than one site. Specific sites of involvement included the following: intracranial meninges, n = 2; pituitary, n = 2; lacrimal gland, n = 1; paranasal sinus, n = 3; neck lymph nodes, n = 6; salivary gland, n = 3; tonsil, n = 1; skin, n = 1; spinal meninges, n = 2; vertebral body, n = 1; and thymus, n = 1. The MRI characteristics of the involved areas were generally T1 isointense, T2 isointense, diffusion isointense to gray matter, and intensely enhancing with gadolinium chelate contrast agents. CT images generally showed the lesions were hyperdense to gray matter and intensely enhancing. FDG PET showed variable uptake, with nodal and lacrimal disease generally being FDG avid and other sites not.
Rosai-Dorfman disease has a protean imaging appearance but most frequently presents as neck lymphadenopathy. The disease is frequently multifocal, and a diagnosis in one area should prompt suspicion that other sites may be involved also.
罗萨达-多夫曼病是一种罕见的疾病,其组织学特征为组织细胞增生导致淋巴管扩张。我们的目的是描述一系列经病理证实的罗萨达-多夫曼病患者的 CT、MRI 和(18)F-FDG(FDG)PET 表现。
我们回顾性分析了 2004 年 1 月至 2007 年 12 月在我院接受治疗的 10 例经病理证实的罗萨达-多夫曼病患者的影像学资料。
我们发现以下部位的广泛受累:3 例颅内,7 例头颈部,3 例脊柱,部分患者有多个部位受累。具体受累部位包括:颅内脑膜 2 例;垂体 2 例;泪腺 1 例;副鼻窦 3 例;颈部淋巴结 6 例;唾液腺 3 例;扁桃体 1 例;皮肤 1 例;脊髓脑膜 2 例;椎体 1 例;胸腺 1 例。受累部位的 MRI 特征一般为 T1 等信号、T2 等信号、弥散等信号与灰质,增强后明显强化。CT 图像一般显示病变密度高于灰质,增强明显。FDG PET 显示摄取程度不一,淋巴结和泪腺病变一般摄取 FDG 较多,其他部位则不然。
罗萨达-多夫曼病的影像学表现多种多样,但最常见的表现为颈部淋巴结病。该疾病常为多灶性,一个部位的诊断应提示其他部位也可能受累。