Klin Onkol. 2021 Spring;34(2):120-127. doi: 10.48095/ccko2021120.
Castleman disease (CD) is a rare lymphoproliferative disorder including unicentric and multicentric forms which can further be divided into four histopathologic variants (hyaline vascular, plasma cell, mixed, and plasmablastic). Multicentric CD typically behaves as an aggressive, relapsing entity with generalized lymphadenopathy and systemic symptoms. PET/CT following 18F-fluorodeoxyglucose administration (FDG-PET/CT) represents an imaging modality commonly used in malignant lymphomas for staging purposes and response assessment. However, literature data on its role in CD have been limited.
Twenty-nine patients, 18 men and 11 women, dia-gnosed in 1998-2016 were enrolled in our retrospective study. All patients underwent FDG-PET/CT during initial staging and/or as part of response assessment. We measured the maximum diameter of a lesion and established an index value corresponding to the ratio of the maximum standardized uptake value for the observed lesion and for the liver. The information about imaging examinations, patients, and disease extensions was put in a registry and statistically analyzed.
Unicentric and multicentric CD was dia-gnosed in 17 and 12 patients, respectively. Median age at the dia-gnosis was comparable between the two groups (51 and 58 years, respectively; P = 0.352). The majority of patients with multicentric CD (83%) were men. In women, the unicentric form prevailed (82 vs. 18%) while the difference between the two forms was of borderline significance in men (44 vs. 56%; P = 0.064). Most of the patients (88%) with unicentric CD had the hyaline vascular pathology type. On the contrary, the plasma cell type was predominant in multicentric CD (42%). The most commonly included anatomic sites included the retroperitoneum (52%) and the thorax (43%). Inguinal node involvement developed only in patients with multicentric CD. In repeatedly examined patients, FDG-PET/CT demonstrated a progressively decreasing size and metabolic activity of a selected lymph node.
FDG-PET/CT represents a suitable modality for initial staging and response monitoring of CD, especially in patients with a multicentric form.
卡斯特曼病（CD）是一种罕见的淋巴组织增生性疾病，包括局灶性和多中心性两种形式，进一步可分为四种组织病理学变异型（透明血管型、浆细胞型、混合性和浆母细胞型）。多中心 CD 通常表现为一种侵袭性、复发性疾病，伴有全身淋巴结病和全身症状。氟脱氧葡萄糖（FDG）摄取的正电子发射断层扫描（PET/CT）（FDG-PET/CT）是一种常用于恶性淋巴瘤分期和疗效评估的影像学方法。然而，关于其在 CD 中的作用的文献数据有限。
我们对 1998 年至 2016 年间确诊的 29 例患者（18 名男性和 11 名女性）进行了回顾性研究。所有患者在初始分期和/或疗效评估时均行 FDG-PET/CT 检查。我们测量了病变的最大直径，并建立了一个与观察到的病变与肝脏的最大标准化摄取值之比相对应的指数值。将影像学检查、患者和疾病扩展的信息记录在一个登记表中，并进行了统计学分析。
17 例患者诊断为局灶性 CD，12 例患者诊断为多中心 CD。两组患者的中位年龄无差异（分别为 51 岁和 58 岁；P = 0.352）。大多数多中心 CD 患者（83%）为男性。女性中，局灶性 CD 占优势（82%对 18%），而在男性中，两种形式之间的差异具有边界显著性（44%对 56%；P = 0.064）。大多数局灶性 CD 患者（88%）具有透明血管组织病理学类型。相反，浆细胞型在多中心 CD 中占优势（42%）。最常见的受累解剖部位包括腹膜后（52%）和胸部（43%）。腹股沟淋巴结受累仅见于多中心 CD 患者。在反复检查的患者中，FDG-PET/CT 显示选定淋巴结的大小和代谢活性逐渐降低。
FDG-PET/CT 是 CD 初始分期和疗效监测的一种合适方法，特别是在多中心形式的患者中。
