Intra-abdominal inflammatory pseudotumor-like follicular dendritic cell sarcoma associated with paraneoplastic pemphigus: A case report and review of the literature

BACKGROUD Follicular dendritic cell(FDC)sarcomas are rare neoplasms that occur predominantly in the lymph nodes.They can also occur extranodally.Extranodal FDC sarcomas most commonly present as solitary masses.Inflammatory pseudotumor(IPT)-like FDC sarcomas,a subcategory of FDC sarcomas,are rarer than other sarcoma subtypes.They are composed of spindle or ovoid neoplastic cells and exhibit an admixture of plasma cells and prominent lymphoplasmacytic infiltration.Paraneoplastic pemphigus(PNP),also known as paraneoplastic autoimmune multiorgan syndrome,is a rare autoimmune bullous disease that is associated with underlying neoplasms.PNP has a high mortality,and its early diagnosis is usually difficult.CASE SUMMARY We describe a 27-year-old woman who presented with stomatitis,conjunctivitis,and skin blisters and erosions as her first symptoms of PNP with an intraabdominal IPT-like FDC sarcoma.The patient underwent surgical tumor resection and received tapering oral corticosteroid treatment.She showed no recurrence at the 1-year follow-up.CONCLUSION IPT-like FDC sarcomas are rare underlying neoplasms that have an uncommon association with PNP.PNP-associated FDC sarcomas predominantly occur in intra-abdominal sites and suggest a poor prognosis.Surgical resection is an essential and effective treatment for PNP and primary and recurrent FDC sarcomas.

Clinicopathological features and prognosis assessment of extranodal follicular dendritic cell sarcoma

AIM: To establish a model for prognosis assessment of extranodal follicular dendritic cell (FDC) sarcoma.METHODS: Nine lesions were examined by routine and molecular approaches.Clinicopathological factors from the new cases and 97 reported cases were analyzed for their prognostic values.RESULTS: The current lesions were found in f ive male and four female patients,located mainly in the head and neck area and averaging 7.2 cm in size.Six patients had recurrence or metastasis and three remained free of disease.The 106 patients (male/female ratio,1.1:1) were aged from 9 to 82 years (median,44 years).The tumor sizes ranged from 1.5 to 21 cm (mean,7.4 cm).Abdominal/pelvic region was affected most frequently (43%).Surgical resection was performed in 100 patients,followed by radiation and/or chemotherapy in 35 of them.Follow-up data were available in 91 cases,covering a period of 3-324 mo (mean,27 mo;median,19 mo).Of the informative cases,38 (42%) had recurrence or metastasis,and 12 (13%) died of the disease.These tumors were classif ied histologically into lowand high-grade lesions.A size ≥ 5 cm (P = 0.003),highgrade histology (P = 0.046) and a mitotic count ≥ 5/10 HPF (P = 0.013) were associated with tumor recurrence.The lesions were def ined as low-,intermediateand high-risk tumors,and their recurrence rates were 16%,46% and 73%,and their mortality rates 0%,4% and 45%,respectively.CONCLUSION: Extranodal FDC tumors behave like soft tissue sarcomas.Their clinical outcomes are variable and can be evaluated according to their sizes and grades.

Surgical treatment of liver inflammatory pseudotumor-like follicular dendritic cell sarcoma: A case report

BACKGROUND Inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDCS)is rare with a low malignant potential.Hepatic IPT-like FDCS has similar clinical features to hepatocellular carcinoma(HCC),making it extremely difficult to distinguish between them in clinical practice.We describe the case of a young female patient diagnosed with HCC before surgery,which was pathologically diagnosed as IPT-like FDCS after the left half of the liver was resected.During 6 mo of follow-up,the patient recovered well with no signs of recurrence or metastasis.CASE SUMMARY A 23-year-old female patient with a 2-year history of hepatitis B presented to the Affiliated Hospital of Guizhou Medical University.She was asymptomatic at presentation,and the findings from routine laboratory examinations were normal except for slightly elevated alpha-fetoprotein levels.However,ultrasonography revealed a 3-cm diameter mass in the left hepatic lobe,and abdominal contrastenhanced computed tomography revealed that the tumor had asymmetrical enhancement during the arterial phase,which declined during the portal venous phase,and had a pseudo-capsule appearance.Based on the findings from clinical assessments and imaging,the patient was diagnosed with HCC,for which she was hospitalized and had undergone laparoscopic left hepatectomy.However,the tumor specimens submitted for pathological analyses revealed IPT-like FDCS.After surgical removal of the tumor,the patient recovered.In addition,the patient continued to recover well during 6 mo of follow-up.CONCLUSION Hepatic IPT-like FDCS is difficult to distinguish from HCC.Hepatectomy may provide beneficial outcomes in non-metastatic hepatic IPT-like FDCS.

Inflammatory pseudotumor-like follicular dendritic cell sarcoma:Literature review of 67 cases

Inflammatory pseudotumor(IPT)-like follicular dendritic cell(FDC)sarcoma is rare.The 2017 World Health Organization classification of tumors of hematopoietic and lymphoid tissues noted that data on its clinical outcome are limited,but that the tumor appears to be indolent.The aim of this study was to summarize the clinical characteristics,treatment outcomes,and prognostic factors for IPT-like FDC sarcoma.A literature review was conducted on retrospective analyses of clinical data and prognostic information on IPT-like FDC sarcoma reported between 2001 and 2020.A total of 67 cases of IPT-like FDC sarcoma were retrieved from the literature,documenting that it occurs predominantly in middle-aged adults,with a marked female predilection.Six patients had a separate malignancy and five had an autoimmune disease.Typically involving the spleen and/or liver,it may also selectively involve the abdomen,gastrointestinal tract,pancreas,retroperitoneum,and mesentery.Necrosis,hemorrhage,noncaseating epithelioid granulomas,and fibrinoid deposits in blood vessel walls are often present.The neoplastic cells are predominantly positive for follicular dendritic cell markers such as cluster of differentiation 21(CD21),CD23,CD35 and CNA.42 and are consistently Epstein-Barr virus(EBV)-positive.Mitoses were very rare in most cases.Most patients were treated by surgery alone.Disease status at the time of last follow-up was known for 57 patients with follow-up time ranging from 2 to 144 mo.Local and/or distant recurrence after initial treatment was seen in 15.8%of the patients.The 1-and 5-year progression-free survival for the entire group was 91.5%and 56.1%,respectively.Kaplan-Meier and multivariate analyses showed that age,sex,tumor size,and pathological features were not risk factors for disease progression.IPT-like FDC sarcoma appears to be mildly aggressive and requires annual surveillance.Surgery is the most effective treatment modality,and the role of adjuvant chemotherapy for postoperative management is unclear.EBV is likely to play an important role in the etiology of IPT-like FDC sarcoma.

Inflammatory pseudotumor-like follicular dendritic cell sarcoma: A brief report of two cases

BACKGROUND Follicular dendritic cell(FDC)sarcoma/tumor is a rare malignant tumor of follicular dendritic cells,which is considered a low-grade sarcoma that can involve lymph nodes or extranodal sites.Conventional FDC sarcomas are negative for Epstein-Barr virus(EBV),whereas the inflammatory pseudotumorlike variant consistently shows EBV in the neoplastic cells.CASE SUMMARY We report two cases of inflammatory pseudotumor-like FDC sarcoma in the liver that received 3D laparoscopic right hepatectomy and open right hepatectomy separately.CONCLUSION EBV probe-based in situ hybridization and detection of immunohistochemical markers of FDC play an important role in the diagnosis and differential diagnosis of inflammatory pseudotumor-like FDC sarcoma.Complete surgical excision combined with regional lymphadenectomy may be effective in reducing the postoperative recurrence and metastasis and improving long-term survival rates.

Primary hepatic follicular dendritic cell sarcoma: A case report

BACKGROUND Follicular dendritic cell sarcoma(FDCS) is an uncommon type of tumor with low incidence. To date,no standard treatment for the disease has been established.Surgery remains the main treatment. Adjuvant chemotherapy and radiotherapy are optional approaches. Metastatic cases require multidisciplinary collaborative treatments. However,the choice of chemotherapeutic drugs is controversial.CASE SUMMARY A 66-year-old Chinese woman presented to our hospital complaining of intermittent pain of right upper quadrant. An enhanced computed tomography(CT) scan of the abdomen revealed hepatocellular carcinoma. Subsequently,the patient underwent a radical partial hepatectomy. Primary FDCS of the liver was diagnosed pathologically. Except for regular follow-up examinations,the patient did not receive adjuvant chemotherapy or radiotherapy. However,fluorine-18-fluorodeoxyglucose positron emission tomography/CT(PET/CT) confirmed lymph node metastases in the space of ligamentum hepatogastricum and pancreatic head,as well as the portacaval space. The patient was given systemic chemotherapy with gemcitabine and docetaxel for she was unsuitable for surgery. Satisfactorily,the metastatic lymph nodes were significantly reduced to clinical complete remission after eight cycles of chemotherapy. Then,strengthened radiotherapy was followed when the patient rejected the opportunity of surgery. Eventually,the carcinoma got better control and the patient was free of progression.CONCLUSION This case highlights the importance of making suitable chemotherapy regimens for the rare tumor. The combination of gemcitabine,docetaxel,and consolidated radiotherapy may offer a new promising option for the treatment of metastatic hepatic FDCS in the future.

A Case Report and Literature Review of Hepatic Follicular Dendritic Cell Sarcoma

To investigate the clinical manifestation, imaging features, pathological characteristics, and diagnosis of hepatic follicular dentritic cell sarcoma, a case report combined with literature review was introduced. A large single mass in liver was showed in CT scan. Enhanced CT showed obvious uneven change of liver neoplasm during the arterial phase and the portal vein phase with tortuous disorganized blood vessels. Immumohistochemical staining of CD21, CD23 and EBER was positive. HFDCS is extremely rare. Except for pathologicaldiagnosis by liver biopsy, its preoperative diagnosis rate is extremely low. Its diagnosis is based on the histopathology and immumohistochemistry. HFDCS was first reported by Shek in 1996, and often occurred in women aged 19 - 82 years (with an average age of 46.7 years). Its main clinical manifestations were abdominal pain, abdominal distention, weight loss, anemia, fever, etc. Some patients were asymptomatic.

Follicular Dendritic Cell Sarcoma: A Rare Case Study of Meta-Synchronous Double Primary Cancers Follicular Dendritic Sarcoma and Invasive Breast Cancer

Follicular dendritic cell sarcomas (FDC-Sarcoma) are a rare type of tumor. Although most cases (60%) originate in the cervical, abdominal, or axillary lymph nodes, extranodal origin from secondary lymphatic tissue such as the tonsils, Waldeyer’s ring, or MALT is also prevalent (40%). We report a case of cervical FDCS in a 51-year-old female who developed invasive breast cancer during follow-up. We review the presentation and management of this disorder, emphasizing the differential diagnosis. The patient was continuously monitored and has been free of recurrences for ten years. For FDC Sarcoma of the head and neck, this case suggests surgical resection combined with chemotherapy and radiotherapy as a therapeutic option.

Follicular dendritic cell sarcoma of the liver:unusual presentation of a rare tumor and literature review

BACKGROUND:Hepatic follicular dendritic cell (FDC) sarcoma is an extremely rare neoplasm.Most commonly,FDC sarcoma presents as a solitary mass in lymph nodes,however,several extra-nodal locations have been identified.METHODS:We report a case of a 53-year-old female who presented with symptoms of abdominal pain,fever,anemia,and jaundice.After an extensive review of the literature,we have found only 12 cases of hepatic FDC sarcoma.RESULTS:The tumor was 11.5 cm in diameter and composed of spindle and epithelioid cells with ovoid nuclei and associated with mixed inflammatory infiltrate.Immunohistochemical stains were positive for CD35 and CD21.The patient underwent a left hepatic lobectomy.CONCLUSIONS:Liver follicular dendritic cell sarcoma is a very rare tumor.Most cases present with abdominal pain and weight loss,and most of them can be managed by hepatic resection with excellent short-term outcomes.

Follicular dendritic cell sarcoma detected in hepatogastric ligament:A case report and review of the literature

The most common organ where follicular dendritic cell sarcoma(FDCS) occurs is in cervical lymph nodes, while few cases are found in extranodal organs such as liver, spleen, and soft tissue. This is a case report that FDCS occurs in the hepatogastric ligament. To our knowledge, there is no such case that has been reported previously. A 47-year-old male patient was found to have an intraabdominal mass during an annual physical examination. Computed tomography showed a 4.2 cm × 4.1 cm mass located at the lesser curvature of the stomach, above the pancreas. During operation, a tumor mass was found in the hepatogastric ligament and a radical resection was performed. The tumor was diagnosed as FDCS by pathology and immunohistochemical testing. The patient had a favorable recovery, and no obvious abnormality was found 3 months postoperation.

Imaging findings of inflammatory pseudotumor-like follicular dendritic cell tumors of the liver:Two case reports and literature review

BACKGROUND Inflammatory pseudotumor-like follicular dendritic cell(IPT-like FDC)tumors of the liver is an uncommon tumor with extremely low incidence.To date,the radiologic findings of this tumor in multiphase computed tomography(CT)and magnetic resonance imaging(MRI)imaging have not been described.CASE SUMMARY Patient 1 is a 31-year-old Chinese female,whose complaining incidentally coincided with the finding of multiple liver masses.In the local hospital,an abdominal enhanced CT found two hypo-dense solid lesions,with heterogeneous sustained hypoenhancement,in the upper segment of the liver’s right posterior lobe.In our hospital,enhanced magnetic resonance imaging(MRI)with hepatocyte-specific contrast agents showed a similar enhanced pattern of lesions with patchy hyperintensity in the hepatobiliary phase(HBP).The patient underwent surgery and recovered well.The final pathology confirmed an IPTlike FDC tumor.No recurrence was found on the regular re-examination.Patient 2 is a 48-year-old Chinese male admitted to our hospital for a huge unexpected hepatic lesion.A dynamic enhanced abdominal CT revealed a huge heterogeneous enhanced solid tumor in the right lobe of the liver with a size of 100 mm×80 mm,which showed a heterogeneous sustained hypoenhancement.In addition,enlarged lymph nodes were found in the hilum of the liver.This patient underwent a hepatic lobectomy and lymph node dissection.The final pathology confirmed an IPT-like FDC tumor.No recurrence was found upon regular re-examination.CONCLUSION When a hepatic tumor shows heterogeneous sustained hypoenhancement with a patchy enhancement during HBP,an IPT-like FDC tumor should be considered in the differential diagnosis.

Two cases of extranodal follicular dendritic cell sarcoma

Follicular dendritic cell (FDC) is an essential component of the nonlymphoid, nonphagocytic immunoaccessory reticulum cells of the peripheral lymphoid tissue(1). FDCs are confined largely to the primary and secondary B-cell follicles, where they form a tight interlacing meshwork. They play a role in the capture and presentation of antigens, generation and regulation of immune complexes. FDCs can be recognized morphologically by their indistinct cellular borders, pale eosinophilic cytoplasm, round-to-ovoid nuclei with delicate nuclear membranes and clear-to-vesicular chromatin with inconspicuous or small nucleoli. FDCs are best identified through immunostaining using CD21, CD35, R4/23, KiM4, KiM4p and Ki-FDC1p. A proliferation of FDCs may be seen in a variety of lesions, including reactive follicular. hyperplasia, Castleman's disease, follicular lymphoma, mantle cell lymphoma, angioimmunoblastic T-cell lymphoma and nodular lymphocyte predominant Hodgkin's lymphoma. Previous studies(2-5) showed that follicular dendritic cell sarcoma (FDCS) affected predominantly the lymph nodes, with occasional extranodal involvement. As the tumor consists mainly ovoid-to-spindle cells, it is not uncommon to misdiagnose the lesion as a wide variety of other spindle cell sarcomas. This study focused on characterizing the histologic features of extranodal FDCS to promote the recognition of this rare entity for both clinicians and pathologists.

Clinical and pathological features of follicular dendritic cell sarcoma of appendix： a case report

Follicular dendritic cell sarcoma （FDCS） is a rare spindle cell neoplasm usually identified as lymph node-based neoplasm that occasionally occurs in extranodal regions. It was first characterized by Monda et al in 1986. In the present report, we describe the first case of FDCS arising from the appendix.

Fc-Epsilon Receptor (CD23) Expressing Follicular Dendritic Cells as a Main Prognostic Factor in Follicular Lymphoma

Fc-epsilon receptor (CD23)-expressing follicular dendritic cells is a main prognostic factor in follicular lymphoma. Falaleeva N. A., Osmanov E. A., Tupitsyn N. N. Federal State Budgetary Institute N. N. Blokhin Russian Cancer Research Center, Health Ministry of Russian Federation, Moscow, Russia SUMMARY Follicular dendritic cells, expressing FcεRII or CD23 (FcεRIIFDCs) as a component of non-tumor environment have been studied in 232 follicular lymphoma (FL) patients. FcεRIIFDCs were found in 87.5% of follicular lymphoma cases and were associated with a nodular pattern of tumor growth (p = 0.000), but not the cytological grade of lymphoma. There were no associations of FcεRIIFDC with clinical prognostic factors (FLIPI indices) or with bone marrow involvement in FL patients by histology. The presence of FcεRIIFDCs in tumor tissue was an independent prognostic factor according to treatment results, i.e. frequency of CR, duration of OS and PFS. Bone marrow involvement significantly worsened the prognosis in FcεRIIFDC-positive group of patients. We suggest a new prognostic index (FDC-IP) that allows biochemical identification of the following patient groups: FcεRIIFDC-positive patients without bone marrow involvement (good prognosis), FcεRIIF-DC-positive patients with bone marrow involvement (intermediate prognosis), FcεRIIFDC-negative patients (poor prognosis). These 3 groups significantly differ (p = 0.000) both in OS and in PFS. This is the first evidence of the possibility to assess tumor behavior and treatment results in FL according to lymphoma biochemical and other than clinical parameters.

Parotid gland carcinosarcoma with follicular dendritic cell sarcoma as mesenchymal component: a case report

The WHO has classified malignant mixed tumours of salivary glands into noninvasive carcinoma in pleomorphic adenoma, invasive carcinoma in pleomorphic adenoma, carcino-sarcoma and metastasizing mixed tumour.~1 Carcinosarcoma, or true malignant mixed tumour, is a tumour composed of both carcinomatous and sarcomatous elements. It is an exceedingly rare tumour of the salivary glands and only about 60 cases have been reported.~2 In this report we describe a case of carcinosarcoma of a parotid gland that contained an unusual mesenchymal component (follicular dendritic cell sarcoma, FDCS) in a 55-year-old man with cytological, histological and immunohistochemical findings. To our knowledge, this histological pattern has not been reported previously in the English literature.

New insights into the immunopathogenesis of systemic lupus erythematosus： the role of T follicular helper cells

Objective To review the development of T follicular helper （TFH） cells and their role in systemic lupus erythematosus （SLE） pathogenesis, the effect of dendritic cells （DCs） on TFH cells in SLE, as well as the potential use of TFH cells as a new therapeutic target in clinical practice. Data sources The data used in this review were retrieved mainly from the PubMed database （1989-2013）. The terms used in the literature search were ＂T follicular helper cells,＂ ＂systemic lupus erythematosus,＂ and ＂dendritic cells.＂ Study selection Relevant publications about the TFH cells development, the interaction between the TFH cells and the DCs, and the clinical applications of TFH cells were identified, retrieved, and reviewed. Results TFH cells, a novel distinct CD4＋ T cell subset, are specialized in providing help to B cells in the formation of germinal centers （GCs） and long-term protective humoral immune responses. The development of TFH cells from naive CD4＋ T cell is a multistep process. As the pivot of immunoregulation, DCs are indispensable for TFH cells generation. In addition to receptor-ligand interactions between TFH cells and DCs, the cytokines secreted by DCs are also necessary for TFH cell generation. TFH cell dysregulation has been implicated in the development of SLE. More evidence from animal models of SLE and SLE patients suggests that TFH cells are necessary for pathogenic autoantibody production. Therefore, therapeutically targeting TFH cells can be a promising approach to treat antibody-mediated autoimmune diseases including SLE. Conclusion TFH cells play a critical role in the pathogenesis of SLE, making them attractive therapeutic targets in clinical practice.

18例胸腹部滤泡树突细胞肉瘤患者的临床表现、病理特征、CT及MRI表现

目的探讨胸腹部滤泡树突细胞肉瘤(FDCS)的临床表现、病理特征、CT及MRI表现,提高对该病影像学的认识,减少漏诊、误诊的发生。方法回顾性分析2015年1月至2023年12月浙江大学附属第一医院收治经手术病理证实的18例胸腹部经典型FDCS或肝脾Epstein-Barr病毒(EBV)阳性炎性滤泡树突状细胞肉瘤(EBV+IFDCS)患者的临床资料,16例行CT平扫+增强检查,13例行MRI平扫+增强检查;分析FDCS及EBV+IFDCS患者的临床表现、CT表现、MRI表现、病理特征和复发、转移情况。结果18例FDCS中5例为经典型FDCS,3例来源于十二指肠旁淋巴结,1例位于胰尾部,1例位于右前上纵隔,均为单发类圆形或椭圆形肿块,1例可见钙化,5例均见囊变、坏死,呈不均匀轻中度持续强化,未见包膜结构;13例EBV+IFDCS,10例位于脾脏,3例位于肝脏;均为单发边缘清晰的肿块,呈圆形或卵圆形,1例可见钙化,9例可见坏死、囊变,肿瘤实质成分呈不均匀轻中度持续强化,2例来源于肝脏的呈明显强化,静脉期强化程度有减退;多数可见低信号/低密度包膜且包膜延迟强化。经典型FDCS及EBV+IFDCS肿瘤实性成分DWI均呈高信号,ADC图呈低信号,DWI及ADC信号与相应脾脏信号相仿。结论经典型FDCS是罕见的中度恶性肿瘤,易复发、转移,多表现为边缘清晰的单发肿块,无包膜结构,较大肿块多合并坏死、囊变,偶见钙化,增强后呈不均匀持续强化;而EBV+IFDCS是罕见的低度恶性肿瘤,多发生于肝脾,无临床症状,多为单发肿块,较大肿块多合并坏死、囊变,偶见钙化,发生于肝脾病变强化方式不一致,多数在T2WI图像上可见环状低信号包膜且包膜呈延迟强化,经典型FDCS及EBV+IFDCS肿瘤实性成分DWI均呈高信号,ADC图呈低信号,与相应脾脏一致,有助于诊断和鉴别诊断;MRI对FDCS和EBV+IFDCS诊断更有优势及价值,可以作为首选检查方法,为临床诊断及术前评估提供重要依据。

肝脏滤泡树突状细胞肉瘤的MRI特征

目的:探讨肝脏滤泡树突状细胞肉瘤(FDCS)的MRI特征。方法:回顾性分析7例经手术及病理证实的肝脏FDCS的术前MRI表现特征。MRI图像分析包括观察病灶部位、大小、形态、边缘、病灶内囊变、出血、脂肪,平扫信号、强化方式和其他伴随征象,有无肝包膜回缩,邻近胆管有无扩张、肝门区及腹膜后有无淋巴结转移等征象。结果:7个病灶均为肝内单发类圆形或椭圆形病灶,边缘清晰光滑,肿瘤实性部分T1WI呈低信号,T2WI呈高信号,DWI呈高信号,ADC图实性部分呈低或稍低信号,2个病灶表现为信号均匀的实性肿块,5个病灶为实性肿块伴有不同程度的囊变,其中1个病灶囊变明显,并见出血。动态增强扫描5个病灶表现为“速升平台型”模式;2个病灶表现为“速升缓降型”模式;1个病灶周围异常灌注,2个病灶见延迟强化的包膜;1个病灶周围胆管轻度扩张,所有病例肝门区及腹膜后未见肿大淋巴结。结论:肝脏FDCS的MRI表现有一定的特征,多表现为边界清楚的肿块,常伴有不同程度囊变,实性部分信号均质,动脉期均呈明显强化,多数表现为明显持续强化。

EBV阳性炎性滤泡树突状细胞肉瘤9例临床病理特征

目的探讨EBV阳性炎性滤泡树突状细胞肉瘤(epstein-barr virus-positive inflammatory follicular dendritic cell sarcoma,EBV^(+)IFDCS)的临床病理学特征。方法回顾性分析9例EBV^(+)IFDCS的临床病理特征、免疫表型及随访,行HE及免疫组化EnVision法检测,并复习相关文献。结果9例EBV^(+)IFDCS患者年龄22~78岁,平均44.7岁。7例发生于肝,2例发生于脾。肿瘤内可见血管壁纤维样及透明变性(6/9)、嗜酸性粒细胞浸润(3/9)和上皮样肉芽肿(2/9)。免疫表型:瘤细胞表达滤泡树突状细胞标志物CD21(7/9)、CD23(8/9)和CD35(9/9),部分表达SMA(6/9)和D2-40(1/9)。2例脾IgG4阳性浆细胞明显增高(80~135个/10 HPF),7例肝IgG4阳性浆细胞为0~36个/10 HPF。术后随访3~84个月,6例无瘤生存,2例转移,1例失访。结论EBV^(+)IFDCS罕见,低度恶性。EBER原位杂交和免疫组化检测在EBV^(+)IFDCS诊断和鉴别诊断中具有重要作用。治疗以手术切除为主,术后需长期随访。

原发性扁桃体指突状树突细胞肉瘤1例及文献复习

目的 探讨指突状树突细胞肉瘤(IDCS)的临床病理学特点、免疫学表型、诊断和鉴别诊断要点。方法 通过常规HE染色、免疫组织化学标记及EBER原位杂交检测,对1例IDCS进行显微镜下组织学形态观察,并进行文献复习。结果 IDCS细胞呈卵圆及短梭形,镜下呈弥漫性浸润生长,表面坏死、渗出,侵犯扁桃体组织。瘤细胞胞浆丰富、嗜酸、界限不清,细胞核主要呈卵圆形、短梭形,细胞有异型,核仁明显,核分裂像易见。肿瘤内散在少数正常小淋巴细胞。免疫组化染色显示肿瘤细胞S-100、CD68、Vimentin呈阳性表达,CD1a、CD23、CD35、CD3、CD20等呈阴性表达,Ki-67增殖指数为70%~80%。原位杂交检测显示EBER阴性表达。结论 指突状树突细胞肉瘤是来源于指突状树突细胞的一种少见肉瘤。病理诊断主要依靠病理形态学和免疫组织化学标记,S-100、CD68阳性表达、CD1a、CD23、CD35、CD3、CD20阴性表达是诊断该肿瘤的重要参考依据之一。鉴别诊断主要包括滤泡树突细胞肉瘤、组织细胞肉瘤、朗格汉斯细胞肉瘤、大细胞性B细胞和T细胞淋巴瘤、恶性黑色素瘤、转移性神经内分泌癌等。