Bahauddin Rabia, Al-Taie Alaa, Al-Khafaji Fatima, Barah Ali
Radiology, Hamad General Hospital, Doha, QAT.
Clinical Radiology, Hamad General Hospital, Doha, QAT.
Cureus. 2022 Jun 25;14(6):e26317. doi: 10.7759/cureus.26317. eCollection 2022 Jun.
Rosai-Dorfman disease (also known as sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman-Destombes disease) is a rare reactive histiocytic disease, classically involving the lymph nodes of the neck, but it can also occur in extranodal sites. Isolated spinal involvement is rare but important to identify as it can mimic malignancy with males being affected more than females. We present a case of a 52-year-old female patient who had breast cancer and was admitted with long-standing anal and sacral pain. The MRI lumbar spine showed a mass concerning for metastasis. CT-guided biopsy results showed sheets of histiocyte-like cells, some of which were positive with S100 and showed emperipolesis along with plasma cells, lymphocytes, and neutrophils. Features were in keeping with Rosai-Dorfman syndrome. The patient responded to steroids. The familiarity with this entity saved the patient from going through the agony of this being considered a metastasis of her primary malignancy.
罗萨伊-多夫曼病(也称为伴有巨大淋巴结病的窦性组织细胞增多症或罗萨伊-多夫曼-德斯顿贝斯病)是一种罕见的反应性组织细胞疾病,典型表现为累及颈部淋巴结,但也可发生于结外部位。孤立性脊柱受累罕见,但因其可酷似恶性肿瘤且男性受累多于女性,故识别该病很重要。我们报告一例52岁女性患者,她患有乳腺癌,因长期肛门及骶部疼痛入院。腰椎MRI显示有一个可疑转移瘤的肿块。CT引导下活检结果显示成片的组织细胞样细胞,其中一些S100阳性,并可见吞噬现象,同时伴有浆细胞、淋巴细胞和中性粒细胞。这些特征符合罗萨伊-多夫曼综合征。患者对类固醇治疗有反应。对该疾病的熟悉使患者免于承受被认为是原发性恶性肿瘤转移所带来的痛苦。