Castleman's disease

AIM: To describe original experience in management of Castleman's disease (CD) and review literature data. MATERIAL AND METHODS: Twelve cases of HIV-free CD in patients aged 18-51 years (mean age 36 years) are reported. RESULTS: CD was plasmocell, mixed and hyalinovascular in 6, 2 and 4 patients, respectively. Histological and immunophenotypical characteristics of CD are detailed. Three patients with plasmocell CD died of severe autoimmune anemia. All the patients with hyalinovascular KD variant were treated surgically (enlarged lymph nodes were removed) and achieved remission. CONCLUSION: The diagnosis of plasmocell CD is made after exclusion of infections, collagenoses, autoimmune diseases and lymphomas. Therapy of plasmocell variant of CD has not been developed yet.     

Castleman's disease

Castleman's disease is characterized by lymph node enlargement. There are two distinct histologic subtypes. Most patients are asymptomatic and under age 30, and a mediastinal or hilar mass is found incidentally on chest radiograph. The pathogenesis is unknown, but a chronic inflammatory process has been suggested by some investigators. Complete surgical excision is the treatment of choice and is generally curative.     

Submandibular Castleman's disease

Castleman's disease is a rare benign lymphoid tumour of unknown aetiology, which usually appears as a solitary mediastinal mass. We report an unusual case of Castleman's disease, an asymptomatic submandibular mass in the lower jaw, and we discuss the computed tomography appearance.     

Ultrasound appearance of extramedullary hematopoiesis

Extramedullary hematopoiesis accompanies a wide variety of diseases and is defined as the abnormal production of blood outside the normal confines of the bone marrow. Most of the time, extramedullary hematopoiesis is found as microscopic foci in soft tissues; however, on occasion this process may reach a size that is detectable with ultrasound. Two forms are possible: "paraosseous"--in which the normal medullary tissue of the bone marrow ruptures through the bone to present as a paraosseous soft tissue mass,--and "extraosseous"--in which extramedullary hematopoiesis occurs within soft tissue. These "masses" of hematopoietic tissue can appear almost anywhere in the abdomen and pelvis and can mimic a wide variety of more serious diseases.     

Ultrasound of the parotid gland.

Five cases of parotid gland masses were studied by ultrasound. Only retention cysts and true cysts of the parotid gland appear sonolucent at low and high gain settings. Benign and malignant tumor present as solid masses except for the Warthin's tumor which is sonolucent at lower gain settings but has a lattice of internal echoes at higher gain settings.     

Ultrasound appearance of fetal greater omentum

Fetal ascites results in the ultrasonographic demonstration of normal structures within the fetal abdomen that are otherwise not seen. One such structure is a membrane that, when surrounded by intraperitoneal fluid, may simulate a loculated area of fluid or a fluid-filled viscus. On the basis of its configuration and site of insertion observed during real-time and static ultrasonography, we believe that this membrane is the greater omentum. Recognition of the typical appearance of greater omentum floating in fetal ascites will prevent the erroneous diagnosis of an abnormal intra-abdominal fluid collection.     

腹部Castleman病影像学诊断

目的：探讨腹部Castleman病的影像学表现。材料与方法：腹部Castleman病4例,女3例,男1例。均行X线、超声、CT检查并经手术及病理证实。结果：病灶为单发,肾形或椭圆形,均有完整的包膜,可见条状、绒毛状或珊瑚状钙化;超声以低回声为主,可见点条状强回声,后方伴声影;CT平扫示软组织肿块,边缘清楚,周围有点条状影,增强肿块与周围点、条状影呈明显均匀强化。结论：Castleman病的影像学表现缺乏特征性;肿块不侵及邻近组织和器官,见到肿块内出现条状、树枝样钙化应想到本病的可能。     

Mesenteric Castleman's disease: case report

An asymptomatic case of hyaline vascular–type Castleman's disease localized to the mesentery and detected incidentally by ultrasound is presented. Computed tomographic, angiographic, and histologic findings are reported. This type of Castleman's disease predominates in the thorax and is very rare in the mesentery. Received: 5 January 2000/Accepted: 26 January 2000     

MRI诊断局限型巨大淋巴结增生症

目的 探讨MRI诊断局限型巨淋巴结增生(LCD)的价值。 方法 回顾性分析25例病理学确诊的LCD患者的MR平扫、增强扫描及DWI资料,并与临床和病理学结果对比。 结果 25例LCD中,位于纵隔10例,颈部3例,腹膜后12例;均为透明血管型,以单发病灶为主(24/25,96.00%),形态多为圆形或卵圆形(23/25,92.00%),边缘多光滑规整(23/25,92.00%);MR平扫16例为T1WI均匀等稍低信号、T2WI均匀稍高信号,4例病灶内T2WI呈斑片状高信号,3例T1WI及T2WI为边缘裂隙样低信号,2例T2WI中心区低信号;动态增强扫中,6例动脉期显著均匀强化,延迟期仍持续均匀强化,2例动脉期病灶边缘强化,延迟期持续均匀强化,3例动脉期软组织区域显著强化,延迟期见分隔样强化,2例动脉期明显强化,延迟期内见线状及裂隙样不强化影;病灶包膜强化完整、连续。DWI中4例表现为均匀一致稍高信号、4例为斑片状高低混杂信号、2例中心区裂隙状低信号;病灶实质区平均ADC值为(1.78±0.14)×10^-3 mm²/s,液化、囊变区平均ADC值为(2.98±0.51)×10^-3 mm²/s 。 结论 MRI可准确显示LCD的发生部位、病灶形态及强化方式,尤其对病灶包膜的显示更清晰;DWI在显示病变范围和内部结构方面较常规MRI更佳。     

Castleman's disease and retroviral therapy.

The escalating pandemic of the acquired immunodeficiency disease in sub-Saharan Africa is associated with an increasing incidence of the lymphoproliferative disorders where evidence shows that highly active retroviral therapy can reconstitute immunologic competence and, at least in some groups exemplified by Kaposi's sarcoma, result in an outcome comparable to uninfected controls. Paradoxically other subtypes are less responsive exemplified by Burkitt lymphoma and multicentric Castleman's disease, where they are localised and may present after starting treatment. This association provides a model to test the concept that pathogenesis may reflect an aberrant response to antigens including human herpesvirus-8 thereby renewing focus on proactive inclusion of anti-herpes drugs with conventional treatment for retrovirus particularly prior to initiating chemotherapy.     

Castleman’s病的CT、MRI表现

目的：探讨Castleman’s病的CT、MRI影像特点。方法：回顾性分析经病理证实的27例Castleman’s病的CT、MRI影像表现。结果：累及淋巴结的局灶型Castleman’s病多表现为类圆形结节或肿块,密度或信号较均匀;增强后明显持续强化;病灶内可见小斑片状稍低或稍高密度影,增强后延迟强化;病灶周边可见“卫星”结节和迂曲、增粗血管影。累及淋巴结的多中心型病变表现为双侧颈部间隙、锁骨上窝、腋窝、腹股沟区及纵隔、腹膜后多发淋巴结增大;增强后可明显强化。累及淋巴结外组织和器官的Castleman’s病的部位及CT、MRI表现多变。结论：Castleman’s病的CT、MRI表现与其病理分型、临床类型及其病变部位密切相关。累及淋巴结的局灶型病变的CT、MRI表现较具特征性;累及淋巴结的多中心型病变的影像表现与淋巴瘤相似;累及结外器官和组织的Castleman’s病的发病部位及影像表现无特征性,诊断较为困难。     

Lack of cytogenetic abnormalities in Castleman's disease

BACKGROUND: Castleman's disease (CD) is a distinctive type of atypical lymph node hyperplasia that is often clonal. In a previously reported series of CD, clonal populations of plasma cells were detected by immunohistology in 4 of 39 cases (10%, lambda restricted), and immunoglobulin gene rearrangements were detected by paraffin polymerase chain reaction (PCR) analysis in 10 of 37 cases (27%). Cytogenetic analysis has been used to detect clonal proliferations of plasma cells in myeloma and clonal proliferations of lymphocytes in lymphomas and has identified critical gene loci that are important in the histopathogenesis of these disorders. Cytogenetic studies have not been done on a large series of patients with CD. Thus, we reviewed the archives of our institution for cases of CD and lymphoma that had had cytogenetic analysis. METHODS: The cytogenetic and lymphoma archives of our institution (a tertiary care center) from 1985 to 1998 were reviewed for the diagnoses of CD and lymphoma. There were 21,006 lymphomas, 701 of which had cytogenetic analysis (400 abnormal). There were 162 cases of CD, 7 of which had cytogenetic analysis. The frequency of cytogenetic abnormalities in CD was compared with that in lymphoma. The sensitivity of cytogenetics for defining clonality in CD was compared with immunohistology and paraffin PCR-amplified immunoglobulin heavy-chain gene rearrangement. RESULTS: From 1985 to 1998, 162 cases of CD and 21,006 cases of lymphoma were diagnosed. Cytogenetic analysis yielded adequate numbers of metaphases for analysis of 4 cases of CD and 701 lymphomas. Cytogenetic abnormalities were not identified in CD but were identified in 400 lymphomas (57%). Although 1 of 4 cases of CD was clonal by immunohistology (lambda restricted), no immunoglobulin gene rearrangements were detected by paraffin PCR analysis. CONCLUSIONS: The frequency of cytogenetic abnormalities in lymphomas and the lack of cytogenetic abnormalities in CD suggest that cytogenetic abnormalities, as detected by conventional cytogenetic analysis, are important in the pathogenesis of lymphoma but not CD. The lack of cytogenetic abnormalities in CD supports the hypothesis that CD is an interleukin-6-driven lymphoproliferative disorder.     

Adrenocortical neoplasm in children. Ultrasound appearance

The ultrasound appearances of three children presenting with virilization are described. All cases were also examined by computed tomography. The value of ultrasound in the initial detection of virilizing neoplasm and in follow-up studies for localization of tumor recurrence following initial surgery are outlined. The advantages of ultrasound include confirmation of extrarenal and extrahepatic location in longitudinal sections, and clear demonstration of invasion of hepatic veins, the inferior vena cava (IVC), and the right atrium on real-time scanning. Computed tomography is also useful, particularly in the localization of small lesions and the detection of pulmonary metastases.     

腮腺腺淋巴瘤的临床、病理及CT表现

目的 探讨腮腺腺淋巴瘤(Warthin's瘤)的临床、病理及CT表现.方法 回顾性分析经术后病理证实的24例腮腺Warthin's瘤的临床、病理及CT资料.结果 24例患者,男22例,女2例;单发16例,多发8例,共38个病灶,其中31个(81.6％)病灶位于腮腺后下象限.病灶多呈高密度圆形、椭圆形结节或肿块,边缘光整,密度均匀或欠均匀,增强后34个病灶中2个轻度强化,5个中度强化,27个明显强化;17个均匀强化,17个不均匀强化(直径＞2 cm),且10个伴贴边血管征.结论 符合腮腺Warthin's瘤的发病年龄、性别、部位及影像学表现的患者,诊断时应首先考虑Warthin瘤的可能.