Serpa Carmoma Frans, Cueva Marta, Serpa Carmoma Frans, Molina Gabriel A, Yu Lee Alberto, Flores Emilia
Hospital Metropolitano Department of General Surgery, Quito, Ecuador.
Universidad San Francisco de Quito (USFQ), Quito, Ecuador.
Ann Med Surg (Lond). 2021 Jun 9;67:102494. doi: 10.1016/j.amsu.2021.102494. eCollection 2021 Jul.
Castleman's disease was first reported by Benjamin Castleman et al., in 1954 and described it as a sporadic lymphoproliferative disorder. The pathophysiology to this day is still unknown, although IL-6 is suspected to play an important role. Preoperative diagnosis is challenging due to its non-specific symptoms, and that imaging cannot clearly distinguish the disease from other processes. High clinical awareness is necessary to reach a diagnosis. If the disease is localized, complete recovery can be achieved through surgery.
Patient is a 68-year-old woman with a three-month history of recurrent episodes of fever, myalgias, and night sweats. She started to experience lower abdominal pain and presented to the emergency room. A contrast-enhanced abdominal computed tomography revealed a 5 cm well-circumscribed focal heterogeneously enhancing hyperplastic mass between the portal vein and the inferior vena cava. After successful laparoscopic surgery, the mass was resected, and the patient fully recovered. Unicentric Castleman's disease was the final diagnosis.
Castleman's disease is an uncommon pathology with a challenging diagnosis. When approaching an abdominal mass, unicentric Castleman's disease should always be a differential diagnosis, as treatment can be curative with surgical resection. With the advent of laparoscopic and robotic surgery, these techniques can improve patients' outcomes in these rare pathologies, especially when they appear in complex regions.
1954年本杰明·卡斯尔曼等人首次报道了卡斯尔曼病，并将其描述为一种散发性淋巴增生性疾病。尽管怀疑白细胞介素-6起重要作用，但至今其病理生理学仍不清楚。由于其症状不具有特异性，且影像学检查无法将该疾病与其他病症明确区分，术前诊断具有挑战性。需要高度的临床警觉性才能做出诊断。如果疾病局限，通过手术可实现完全康复。
患者为一名68岁女性，有三个月反复发热、肌痛和盗汗病史。她开始出现下腹部疼痛，并前往急诊室就诊。腹部增强计算机断层扫描显示在门静脉和下腔静脉之间有一个5厘米边界清晰、局灶性不均匀强化的增生性肿块。在成功进行腹腔镜手术后，切除了肿块，患者完全康复。最终诊断为单中心卡斯尔曼病。
卡斯尔曼病是一种罕见的病理情况，诊断具有挑战性。在处理腹部肿块时，单中心卡斯尔曼病应始终作为鉴别诊断，因为手术切除可治愈。随着腹腔镜和机器人手术的出现，这些技术可改善这些罕见病症患者的治疗效果，尤其是当它们出现在复杂区域时。
