PDF(Pubmed)
Abstract：
BACKGROUND: Castleman Disease (CD) is a rare benign disorder characterized by abnormal lymphoid tissue growth. It can be classified as unicentric CD (UCD) affecting a single lymph node region or multicentric CD (MCD) involving multiple regions. While UCD typically occurs in the mediastinum, MCD is associated with inflammatory symptoms and abnormal lab findings. We present a case of a young Syrian male with pelvic UCD, exhibiting unusual symptoms. Surgical excision led to complete recovery.
METHODS: A 27-year-old male presented with a well-defined pelvic mass discovered during routine ultrasonography. Symptoms included fever, fatigue, weight loss, and lower extremity numbness. Laboratory analysis revealed lymphocytopenia. Imaging identified a retroperitoneal pelvic mass, raising concerns of lymphoma. Surgical resection confirmed Castleman disease, specifically the hyaline vascular variant, dispelling malignancy concerns. Post-surgery, the patient demonstrated complete recovery, with normalized lymphocyte count and resolution of symptoms, affirming the effectiveness of the treatment.
CONCLUSIONS: Castleman Disease (CD) is characterized by abnormal lymphoid tissue growth. It presents as Unicentric (UCD) or Multicentric (MCD) forms. UCD cases are usually asymptomatic or show compressive symptoms due to mass effect, while MCD is associated with systemic symptoms. The estimated UCD incidence is 16 cases per million person-years, with median age at diagnosis around 30-34 years. Histologically, UCD is categorized into hyaline vascular (HV) and plasma cell (PC) variants. In this case, systemic symptoms and lymphocytopenia deviated from typical UCD presentation, leading to initial lymphoma suspicions. Surgical intervention facilitated recovery without adjuvant therapies.
CONCLUSIONS: This study emphasizes the spectrum of Castleman Disease (CD) manifestations, distinguishing Unicentric (UCD) and Multicentric (MCD) forms crucial for accurate management. It highlights atypical pelvic UCD presentation and successful surgical treatment\'s importance.
METHODS: A 27-year-old male presented with a well-defined pelvic mass discovered during routine ultrasonography. Symptoms included fever, fatigue, weight loss, and lower extremity numbness. Laboratory analysis revealed lymphocytopenia. Imaging identified a retroperitoneal pelvic mass, raising concerns of lymphoma. Surgical resection confirmed Castleman disease, specifically the hyaline vascular variant, dispelling malignancy concerns. Post-surgery, the patient demonstrated complete recovery, with normalized lymphocyte count and resolution of symptoms, affirming the effectiveness of the treatment.
CONCLUSIONS: Castleman Disease (CD) is characterized by abnormal lymphoid tissue growth. It presents as Unicentric (UCD) or Multicentric (MCD) forms. UCD cases are usually asymptomatic or show compressive symptoms due to mass effect, while MCD is associated with systemic symptoms. The estimated UCD incidence is 16 cases per million person-years, with median age at diagnosis around 30-34 years. Histologically, UCD is categorized into hyaline vascular (HV) and plasma cell (PC) variants. In this case, systemic symptoms and lymphocytopenia deviated from typical UCD presentation, leading to initial lymphoma suspicions. Surgical intervention facilitated recovery without adjuvant therapies.
CONCLUSIONS: This study emphasizes the spectrum of Castleman Disease (CD) manifestations, distinguishing Unicentric (UCD) and Multicentric (MCD) forms crucial for accurate management. It highlights atypical pelvic UCD presentation and successful surgical treatment\'s importance.
摘要：
背景：Castleman病（CD）是一种罕见的良性疾病，其特征是淋巴组织异常生长。它可以分为影响单个淋巴结区域的单中心CD（UCD）或涉及多个区域的多中心CD（MCD）。虽然UCD通常发生在纵隔，MCD与炎症症状和异常实验室发现相关。我们介绍了一个年轻的叙利亚男性骨盆UCD的病例，表现出不寻常的症状。手术切除导致完全恢复。
方法：一名27岁男性在常规超声检查中发现盆腔肿块。症状包括发烧，疲劳,减肥,下肢麻木。实验室分析显示淋巴细胞减少。影像学发现腹膜后盆腔肿块,引起淋巴瘤的担忧。手术切除证实Castleman病,特别是透明血管变体，消除恶性肿瘤的担忧。手术后,患者表现出完全康复，淋巴细胞计数正常化，症状消退，确认治疗的有效性。
结论：Castleman病（CD）的特征是淋巴组织异常生长。它呈现为单中心（UCD）或多中心（MCD）形式。UCD病例通常无症状或显示压缩症状，由于质量效应，而MCD与全身症状有关。估计UCD发病率为每百万人年16例，诊断时的中位年龄约为30-34岁。组织学上,UCD分为透明血管（HV）和浆细胞（PC）变体。在这种情况下,系统症状和淋巴细胞减少症偏离典型的UCD表现，导致最初的淋巴瘤怀疑。手术干预有助于恢复，无需辅助治疗。
结论：这项研究强调了Castleman病（CD）的表现，区分单中心（UCD）和多中心（MCD）形式对于准确管理至关重要。它强调了不典型的盆腔UCD表现和成功的手术治疗的重要性。
方法：一名27岁男性在常规超声检查中发现盆腔肿块。症状包括发烧，疲劳,减肥,下肢麻木。实验室分析显示淋巴细胞减少。影像学发现腹膜后盆腔肿块,引起淋巴瘤的担忧。手术切除证实Castleman病,特别是透明血管变体，消除恶性肿瘤的担忧。手术后,患者表现出完全康复，淋巴细胞计数正常化，症状消退，确认治疗的有效性。
结论：Castleman病（CD）的特征是淋巴组织异常生长。它呈现为单中心（UCD）或多中心（MCD）形式。UCD病例通常无症状或显示压缩症状，由于质量效应，而MCD与全身症状有关。估计UCD发病率为每百万人年16例，诊断时的中位年龄约为30-34岁。组织学上,UCD分为透明血管（HV）和浆细胞（PC）变体。在这种情况下,系统症状和淋巴细胞减少症偏离典型的UCD表现，导致最初的淋巴瘤怀疑。手术干预有助于恢复，无需辅助治疗。
结论：这项研究强调了Castleman病（CD）的表现，区分单中心（UCD）和多中心（MCD）形式对于准确管理至关重要。它强调了不典型的盆腔UCD表现和成功的手术治疗的重要性。
