血管滤泡性淋巴结增生(Castleman病)MRI病例图片影像诊断分析

其他   2010-10-27

【临床病史】:患者,13岁女孩,触到颈部肿块。13-year-old female with palpable neck mass

【影像图片】MRI图像

【影像表现】:A left parotid lesion (arrowhead) appears hyperintense on T1 and T2 (Figure 1, Figure 2, and Figure 3) as compared to muscle and demonstrates moderate to intense enhancement (Figure 4 and Figure 5). The adjacent mandible is normal. Bilateral cervical lymphadenopathy (Figure 6, Figure 7, and Figure 8) is seen on T1 and T2 images (arrows), deep to the sternocleidomastoid muscle. Contrast enhancement (arrows) within these lymph nodes is present (Figure 9 and Figure 10).

    左侧腮腺病变(箭头)在T1和T2序列上显示为高信号(图1-3),相对于肌肉。有中到重度的强化(图4-5)。邻近的下颌骨未见异常,双侧颈部淋巴结肿大(图6-8),位于胸锁乳突肌内侧。增强扫描这些淋巴结有强化(图9-10)。

【影像诊断】:Castleman disease Castleman病

【诊断要点】

  • Castleman disease is a benign lymphoproliferative disorder.  Caseleman病是一种良性的淋巴结增生性病变
  • Imaging demonstrates non-specific mass lesions with or without lymphadenopathy  影像上表现为非特异性的肿块,伴有或不伴有淋巴结肿大。
  • Moderate to intense contrast enhancement can be seen.可以有中度或明显的强化。

【讨论】:Castleman disease (angiofollicular lymph node hyperplasia) is considered a benign lymphoproliferative disorder of unknown etiology and was originally described at the Massachusetts General Hospital by Dr. Benjamin Castleman in 1957. Distinction between the two subtypes - hyaline vascular or plasma cell - can be determined histologically. The hyaline vascular subtype is unicentric, more common, and typically demonstrates a relatively benign disease course. Symptomatology is often due to mass effect and compression of adjacent structures. The plasma cell subtype is often multicentric in location, thought to be a more systemic form of the disease, and associated with HIV and HHV-8. Symptoms are often non-specific. Secondary malignancies are thought to be associated with the plasma cell subtype as well.

    Castleman病(血管滤泡性淋巴结增生)被认为是一种良性的淋巴结增生性病变,病因尚不明。最初是由Dr Benjamin Castleman在1957首先报道的。通过组织学可以区别出两种亚型:透明血管型或浆细胞型。透明血管型是单中心、更常见的、通常表现为相对良性的进程。症状通常是由于肿块的占位效应和邻近结构的受压所致。浆细胞型常常是多中心,被认为是一种全身性的病变形式,并且与HIV和HIV-8有相关性。症状通常无特异性。继发的恶性肿瘤也被认为与浆细胞型相关。

    Imaging characteristics are non-specific but can demonstrate a homogenous mass with moderate to intense contrast enhancement (more prominent in the hyaline vascular subtype). Some studies report T2 hypointense linear characteristics which are hypothesized to be fibrosis. The mediastinum is the most common site for unicentric Castleman lymphadenopathy followed by cervical involvement. Pelvic lymphadenopathy has been reported to demonstrate calcifications.

    影像特征是非特异性的,但是可以显示为一个均匀的肿块,伴有中度到重度的强化(尤其是透明血管型)。一些研究报道T2序列上低信号线样特征被认为是纤维变性。单中心的Castleman病最常见于纵隔,其次是颈部。盆腔的淋巴结增生被报道会有钙化。

    This case was biopsy proven to be Castleman disease of the hyaline vascular subtype. The referring physician opted for observation and several repeat imaging studies have demonstrated stability of disease.

    本例活检证实为透明血管型Castleman病,相关医生选择了观察并进行了数次影像检查以观察病变的稳定性。

    Treatment for unicentric Castleman disease is typically surgical as it is often curable. Multicentric disease is more difficult to treat and therapy can include surgery, chemotherapy, anti-virals, and radiation.

    对于单中心的Castleman病通常采用外科手术即可治愈,多中心的病变处理上更困难些,处理可能包括手术治疗、化疗、抗病毒以及放疗。

(参考来源:,ACR Thursday, September 17, 2009,作者:freemanpyw译)
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