一名儿科患者的罗赛-多夫曼病:影像学检查结果和病理学,以及文献简评。

Pub Date : 2023-08-01 eCollection Date: 2023-09-01 DOI:10.3941/jrcr.v17i8.4873
Thomas Hartmann, Nadia Solomon, Gabriel Lerner, Lauren Ehrlich
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引用次数: 0

摘要

罗赛-多夫曼病(Rosai-Dorfman Disease)又称窦性组织细胞增生症伴大量淋巴结病,是一种罕见的非郎格罕氏细胞组织细胞增生症,在美国的发病率估计为每年 100 例。由于其表现多变且临床表现无特异性,因此对儿童患者的诊断尤为困难。我们报告了一例 11 个月大的男性病例,他因右侧腹股沟肿块就诊 4 天。腹股沟和骨盆超声波显示,腹部和骨盆核磁共振成像证实其为腹股沟肿块,周围伴有淋巴结病。病理证实为罗赛-多夫曼病,患者在开始口服类固醇治疗后病情有所好转。据我们所知,这是文献中报道的首例罗赛-多夫曼病累及一岁以下婴儿腹股沟区的病例。在本病例报告中,我们讨论了影像学和组织学检查结果,并对这一诊断进行了简要的文献综述。
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Rosai-Dorfman Disease in a Pediatric Patient: Imaging Findings and Pathology with a brief review of the Literature.

Rosai-Dorfman Disease, otherwise known as sinus histiocytosis with massive lymphadenopathy, is a rare form of non-Langerhans cell histiocytosis with an estimated incidence of 100 cases per year in the United States. Due to its variable presentation and nonspecific clinical findings, it is particularly difficult to diagnose in pediatric patients. We report a case of an 11-month-old male who presented with a 4-day history of a right groin mass. Ultrasound of the groin and pelvis demonstrated, and MRI of the abdomen and pelvis confirmed an inguinal mass with surrounding lymphadenopathy. Pathology confirmed Rosai-Dorfman Disease and the patient improved after starting oral steroid therapy. To the best of our knowledge, this is the first case of Rosai-Dorfman Disease involving the inguinal region in an infant under 1 year of age reported in the literature. In this case report, we discuss the imaging and histology findings as well as provide a brief literature review for this diagnosis.

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