Zhai Hong-Yan, Zhu Xin-Yuan, Zhou Gui-Ming, Zhu Li, Guo Dan-Dan, Zhang Hao
Department of Ultrasound, Tianjin Medical University General Hospital, Tianjin 300005, China.
Department of Cardivascular, Tianjin Medical University General Hospital, Tianjin 300005, China.
World J Clin Cases. 2022 Feb 6;10(4):1278-1285. doi: 10.12998/wjcc.v10.i4.1278.
Castleman's disease (CD) is a lymphatic proliferative disorder of unknown cause and is rarely seen clinically. It has been divided into unicentric and multicentric types. Unicentric CD (UCD) occurs as a solitary enlarged mass and mediastinal lymph nodes are the most common site. Surgical excision has proven to be curative for UCD. Multicentric CD (MCD) appears as a systemic disease with peripheral lymphadenopathy. MCD had a poor response to surgery and monoclonal antibodies with rituximab have become a research hotspot.
A 44-year-old woman presented with a pancreatic mass during routine physical examination. She had no obvious symptoms, such as fever, abdominal pain, abdominal distension, or jaundice. Ultrasound examination indicated a hypoechoic mass between the body of the pancreas, left lobe of the liver and stomach. It had a clear boundary, irregular shape, uneven echo, and no obvious blood flow signals. To clarify the diagnosis, contrast-enhanced ultrasound examination was performed, which showed a benign pancreatic lesion. Neuroendocrine or solid pseudopapillary tumor was a possible diagnosis. The patient underwent further contrast-enhanced computed tomography and contrast-enhanced magnetic resonance imaging, which were suggestive of solid pseudopapillary tumor or neuroendocrine tumor. All the examinations failed to give a definitive diagnosis, and the patient underwent surgery. The final pathological and immunohistochemical results showed that the mass was CD.
This case highlights when lymphadenopathy is encountered clinically, CD should be considered and a biopsy should be performed.
卡斯特曼病（CD）是一种病因不明的淋巴组织增生性疾病，临床罕见。它分为单中心型和多中心型。单中心型卡斯特曼病（UCD）表现为单个肿大的肿块，纵隔淋巴结是最常见的部位。手术切除已被证明对UCD有治愈作用。多中心型卡斯特曼病（MCD）表现为一种伴有外周淋巴结病的全身性疾病。MCD对手术反应不佳，含利妥昔单抗的单克隆抗体已成为研究热点。
一名44岁女性在常规体检时发现胰腺肿块。她没有发热、腹痛、腹胀或黄疸等明显症状。超声检查显示胰腺体部、肝左叶和胃之间有一个低回声肿块。边界清晰，形状不规则，回声不均匀，无明显血流信号。为明确诊断，进行了超声造影检查，结果显示为良性胰腺病变。可能的诊断为神经内分泌肿瘤或实性假乳头状肿瘤。患者进一步接受了增强CT和增强磁共振成像检查，提示为实性假乳头状肿瘤或神经内分泌肿瘤。所有检查均未能明确诊断，患者接受了手术。最终病理及免疫组化结果显示肿块为卡斯特曼病。
本病例强调，临床上遇到淋巴结病时，应考虑卡斯特曼病并进行活检。
