背景:Castleman病(CD)是一种罕见的淋巴增生,模仿良性和恶性疾病。CD的诊断是根据临床和实验室标准的组合制定的,并最终通过组织病理学评估进行确认。由于它的稀有性,CD在治疗选择方面提出了挑战,有包括手术在内的可用选项,化疗,和自体干细胞移植。然而,研究表明,手术切除病灶是最有效的治疗方式,特别是对于单中心CD(UCD)。
方法:这里,我们描述了一个25岁妇女的情况,她出现无痛的左大腿肿胀10周。她一直遵循低脂饮食来减肥,并且实验室检查结果正常。磁共振成像显示界限清楚,位于左腹股沟区的分界囊性病变与偏心定位的信号空血管结构,尺寸4.3厘米×3厘米×3.2厘米,可能是淋巴起源。病人接受了手术切除,最终的组织病理学显示血管增生和血管壁的透明化,以及穿透血管穿过的隐窝生发中心,与CD一致。手术后一天病人出院,情况良好,在我们的门诊预约随访。
结论:尽管手术切除是UCD的主要治疗方法,由于缺乏特定的诊断特征和治疗方法,因此需要采用多学科方法.
BACKGROUND: Castleman\'s disease (CD) is a rare lymphoproliferative, emulating both benign and malignant diseases. The diagnosis of CD is formulated upon the combination of clinical and laboratory criteria and ultimately confirmed by histopathological assessment. Due to its rarity, CD presents a challenge in treatment selection, with available options encompassing surgery, chemotherapy, and autologous stem cell transplantation. However, studies suggest that surgical resection of the lesion is the most effective treatment modality, especially for unicentric CD (UCD).
METHODS: Here, we describe the
case of a 25-year-old woman who presented with painless left thigh swelling for 10 wk. She had been following a low-fat diet to lose weight and had normal laboratory results. Magnetic resonance imaging revealed a well-circumscribed, demarcated cystic lesion located in the left inguinal region with eccentrically positioned signal void vascular structures, measuring 4.3 cm × 3 cm × 3.2 cm, likely of lymphoid origin. The patient underwent surgical resection, and the final histopathology showed a vascular proliferation and hyalinization of the vessel walls, along with atretic germinal centers traversed by penetrating vessels, consistent with CD. The patient was discharged home one day after the procedure in good condition, with a follow-up appointment scheduled in our outpatient clinic.
CONCLUSIONS: Although surgical resection is the mainstay for UCD, a multidisciplinary approach is needed due the lack of specific diagnostic features and treatments.