Tentative diagnostic criteria and disease severity classification for Castleman disease: A report of the research group on Castleman disease in Japan

Objectives: To determine the tentative diagnostic criteria and disease severity classification for Castleman disease (CD) and describe the clinical and pathologic features among human herpesvirus 8 (HHV-8) negative idiopathic multicentric CD (iMCD) in the Japanese population.

Methods: We established the working groups for the research of CD in Japan and had meetings to discuss and define the tentative diagnostic criteria and disease severity classification for CD. We subsequently analyzed 142 patients classified into iMCD by using the nationwide Japanese patient registry.

Results: We proposed the preliminary diagnostic criteria and disease severity classification for CD based on our discussion. In addition, we made a proposal for the disease activity score. We identified clinical and pathological features of patients with iMCD diagnosed by these diagnostic criteria. In the disease severity classification, 37, 33 and 30% patients were categorized into mild, moderate and severe diseases, respectively.

Conclusion: This is the first proposal for diagnosis and classification of CD by the Japanese group. Further studies are required to validate whether they can distinguish CD from other inflammatory diseases and to determine their sensitivity and specificity.     

Kaposi sarcoma-associated herpesvirus in non-Hodgkin lymphoma and reactive lymphadenopathy in Uganda

Kaposi sarcoma-associated herpesvirus (KSHV) causes Kaposi sarcoma and is also associated with primary effusion lymphoma, a subset of diffuse large B-cell lymphomas, and multicentric Castleman disease. Because KSHV infection is endemic in sub-Saharan Africa, we sought to identify cases of KSHV-positive non-Hodgkin lymphomas (NHLs) and reactive lymphadenopathy in this region. One hundred forty-four cases (80 NHLs, 64 reactive lymph nodes) from the major pathology laboratory in Uganda were reviewed. One NHL was KSHV-positive, as indicated by staining for the viral latent nuclear antigen. This NHL was a diffuse large B-cell lymphoma in a 5-year-old boy. The tumor was also Epstein-Barr virus-positive. In addition, 2 reactive lymph nodes, both classified histologically as follicular involution, stained KSHV latent nuclear antigen-positive and thus most likely represent multicentric Castleman disease. In all 3 KSHV-positive cases, a minority of cells expressed KSHV viral interleukin 6, a biologically active cytokine homolog. In conclusion, we show that KSHV is rarely associated with lymphoproliferative disorders in sub-Saharan Africa. We describe the first case of a KSHV-positive NHL from this region; this case is also the first reported pediatric lymphoma associated with KSHV infection.     

甲状腺乳头状癌合并颈部局灶型Castleman病1例

摘要：目的探讨甲状腺乳头状癌(PTC)合并颈部局灶型Castleman病 (UCD)的临床诊治经验。方法回顾性分析1 例PTC合并颈部UCD的临床病例资料,并检索国内外文献。该患者术前行甲状腺结节穿刺细胞学检查(FNA)倾向PTC,合并右颈侧区淋巴结肿大,行甲状腺右侧腺叶合并峡部切除及右颈II、III、IV、VI区淋巴结清扫术。结果术后切口愈合良好,无声音嘶哑及低钙症状,术后 4 d治愈出院。术后随访6个月无复发。术后病理证实颈侧区肿大淋巴结为UCD。结论PTC合并UCD极为罕见,且UCD的术前诊断较为困难,但手术切除多可治愈;PTC合并颈部淋巴结肿大者,临床医生不仅需考虑甲状腺癌转移淋巴结可能,还需警惕UCD的存在。     

Pelvic Castleman's Disease Presenting as an Adnexal Mass in an Adolescent

BackgroundCastleman disease (CD) is a rare lymphoproliferative disorder that might present as an adnexal mass. We report a case of pelvic CD in an adolescent girl who presented with abdominal pain.CaseA 13-year-old girl presented with severe abdominal pain, nausea, and vomiting, and was found to have a solid adnexal mass. Repeat imaging revealed the mass to be retroperitoneal and in the left pelvic side wall. She underwent surgical removal via an open retroperitoneal approach, and pathology revealed CD, hyaline vascular variant subtype.Summary and ConclusionPelvic CD should be considered in the differential diagnosis for an adnexal mass in a young woman. Surgical planning is critical because of the possibility of extension and mass effect. Most pelvic CD is unicentric, hyaline vascular variant subtype, and does not recur after surgical removal.     

Dysplastic follicular dendritic cells in hyaline‐vascular Castleman disease: a rare occurrence creating diagnostic difficulty

Follicular dendritic cell (FDC) proliferations and dysplastic FDCs can be seen in Hyaline‐vascular Castleman disease (HVCD). The association between HVCD and FDC sarcoma is well‐documented; dysplastic FDCs may be precursors to FDC sarcoma. Herein, we describe a case of HVCD with strikingly large and dysplastic FDCs, which raised the differential of Hodgkin lymphoma and other neoplasms. Scattered dysplastic FDCs were predominantly in germinal centers and mantle zones, and rarely in interfollicular areas. Although occasional germinal centers contained increased FDCs, no mass forming proliferations were present to suggest FDC sarcoma. Immunostaining demonstrated that the atypical FDCs expressed CD21, clusterin and CXCL13, but not CD23, S100, pankeratin or CD30; they aberrantly expressed epidermal growth factor receptor (EGFR). The present case demonstrates that dysplastic FDCs may be present as isolated cells that require immunophenotyping to distinguish them from malignant entities with similar morphologic features. A variety of FDC markers is required to confirm their origin as the expression of any single marker is not assured, as occurred in this case. Pathologists need be aware of FDC proliferations in HVCD because of their association with FDC sarcoma. Aberrant EGFR expression by dysplastic FDCs may indicate that they are pre‐neoplastic and necessitate long‐term patient follow‐up.     

4例面颈部Castleman病的临床病理分析

Castleman病（CD）是一种少见的特殊类型的交界性淋巴组织增生性疾病。本文报道了4例面颈部CD,其临 床分型均为局灶型,组织学分型为透明血管型,手术切除后预后较好,同时结合文献对其临床表现、病因、病理、诊断及治疗等进行了文献复习。     

腹膜后局限型Castleman病的CT及MR表现

目的：分析腹膜后局限型Castleman病的影像学特征,并参考病理分型,提高对该疾病的认识。方法回顾性分析经手术病理证实的10例患者的影像学资料,10例均行CT平扫及增强扫描,5例行MR平扫及增强扫描,分析其影像学表现并总结其特征。结果本组病变病理包括透明血管型8例和浆细胞型2例。CT平扫为等密度病灶,4例伴有钙化,1例伴有囊变,CT增强扫描示7例透明血管型病灶有明显持续强化,1例透明血管型病变与2例浆细胞型病灶呈轻度中度强化。5例行MR扫描,4例为透明血管型,1例为浆细胞型。平扫T1WI呈等或略低信号、T2WI呈高信号,DWI均为较高信号,增强扫描5例均有明显持续强化。结论腹膜后局限型Castleman病以透明血管型居多,影像学表现与病理分型密切相关,透明血管型明显持续强化,且钙化常见,而浆细胞型无明显特异性。     

Chordoid meningioma

Chordoid meningioma is a relatively rare variant that is often associated with peritumoral lymphoplasmacellular infiltration causing Castleman syndrome (CS). We present a 44-year-old woman with chordoid meningioma not associated with CS. The patient presented with epilepsy and right hemiparesis (Todd's palsy) on admission. The radiological findings revealed an extraaxial mass lesion in the premotor cortex. They were compatible with a preoperative diagnosis of meningioma. No physical abnormalities related to CS were detected. A left frontal craniotomy was performed. The tumor surface was gelatinous, and it was totally resected with the attached dura mater (Simpson grade I). The patient had an uneventful recovery, and her seizures subsided. The pathological findings of the specimens revealed nests and cords of spindle and epithelioid cells with abundant myxoid matrix, mimicking the features of chordoma. On the basis of radiological, immunohistochemical, and electron microscopic findings, chordoid meningioma was verified, and a review of the literature was performed. This case was reported at the 2nd International Symposium of Brain Tumor Pathology (May 12–13, 2000, Nagoya, Japan)