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.

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.

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.

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.

脾脏炎性假瘤样滤泡树突细胞肉瘤影像学表现并文献复习

患者,女,69岁,8个月前无明显诱因出现胃纳差,呈食欲不振,消瘦、体重进行性下降约14斤。专科检查无特殊;实验室检查:白蛋白31.3 g/L;血红蛋白89.00g/L;其余实验室检查及肿瘤标志物均呈阴性。行上腹部CT、MRI及PET/CT,脾脏内前缘见类圆形肿块影,大小为8.2cm×6.9cm×6.5cm,边界清,密度不均(图1A),中央坏死,边缘见假包膜;MRI上T_(1)WI呈等稍高信号T_(2)WI稍高信号,压脂未见信号减低(图2-图4);DWI高信号(图5),ADC值为1.125×10^(-3)mm^(2)/s;呈渐进性强化(图1B-图1D)。

肠息肉样变的炎性假瘤样滤泡树突状细胞肉瘤2例并文献复习

通过对2例以结肠息肉为表现的炎性假瘤样滤泡树突状细胞肉瘤(inflammatory pseudotumor-like follicular dendritic cell sarcoma,IPT-like FDCS)的临床资料、组织学形态、免疫组织化学、原位杂交进行分析,并复习相关文献,探讨IPT-like FDCS的临床病理学特征。病例1,女,50岁;病例2,女,56岁。2例均因粪便潜血试验阳性行肠镜检查时发现肠息肉就诊。镜下肿瘤边界清楚,黏膜表面溃疡,黏膜层和黏膜下层可见由明显的淋细胞和浆细胞浸润而形成的带蒂息肉样病变,肿瘤性的梭形细胞分散于显著的淋巴浆细胞背景中,瘤细胞呈圆形、卵圆形或梭形,细胞质淡染透明,核膜薄,染色质分布均匀,核仁小而清晰。免疫表型:病例1 D2-40阳性。病例2 CD21,CD23、CD35和D2-40均阳性。2例原位杂交Epstein-Barr编码区(Epstein-Barr encoding region,EBER)均阳性。病例1术后随访65个月,病例2术后随访40个月,均预后良好。呈肠息肉样表现的IPT-like FDCS非常罕见。认识本病可避免误诊为其他常见类型的结肠息肉病。EBER和FDC相关免疫组织化学标记在鉴别诊断中起重要作用。

肝脏炎性假瘤与炎性假瘤样滤泡树突状细胞肿瘤的病理学诊断

目的探讨肝脏炎性假瘤及肝脏炎性假瘤样滤泡树突状细胞肿瘤的临床病理学特征及诊断、鉴别诊断要点。方法对肝脏炎性假瘤及肝脏炎性假瘤样滤泡树突状细胞肿瘤各1例进行临床病理分析、免疫组织化学染色及EBV-encoded RNA(EBER)原位杂交检测。结果肝脏炎性假瘤的临床症状包括右上腹不适或疼痛、发热、肝肿大、体重减轻等。大体肿瘤呈实性,境界清楚;镜下肿瘤细胞呈梭形,波浪状排列,其间可见大量淋巴细胞及浆细胞浸润以及散在分布的大的多形性细胞,核仁明显。肝脏炎性假瘤样树突状细胞肿瘤的临床症状、影像学表现及镜下表现均与肝脏炎性假瘤十分相似,但肿瘤细胞边界不清,胞质嗜酸性,除R-S样细胞外,还可见到不少形态怪异的巨细胞,且免疫表型CD21、CD35阳性,EBER(EBV-encoded RNA原位杂交)阳性。结论肝脏炎性假瘤样滤泡树突状细胞肿瘤是罕见的肿瘤,诊断时需注意与肝脏梭形细胞肿瘤甚至霍奇金淋巴瘤鉴别,树突状细胞免疫标记CD21、CD35阳性,特别是EBER原位杂交阳性有助于诊断。

炎性假瘤样滤泡树突状细胞肿瘤5例临床病理学特征

目的探讨炎性假瘤样滤泡树突状细胞肿瘤(IPT-like FDCT)的临床病理学特征,提高对该病的认识。方法报告5例炎性假瘤样滤泡树突状细胞肿瘤,结合文献分析其临床病理学特征、免疫表型及原位杂交检测EBV转录的核内小RNA(EBER)。结果5例炎性假瘤样滤泡树突状细胞肿瘤患者年龄35~78岁,平均52.6岁,其中女性2例,男性3例。3例肿瘤位于脾,1例位于肝,1例位于腹膜后。肿瘤通常与周围组织分界较清晰。镜下大量淋巴细胞、浆细胞背景中肿瘤细胞呈束状排列或散在分布;瘤细胞呈短梭形,胞质丰富,淡伊红染色,核呈梭形或卵圆形,空泡状,可见核仁,仅有轻度异型性。免疫表型:瘤细胞CD21、CD35及簇蛋白(clusterin)(+),EBER散在(+)。值得注意的是,其中3例伴随有IgG4阳性浆细胞的高表达。所有病例均经手术切除,随访2~96个月,其中1例失访,余4例均未出现复发和转移。结论炎性假瘤样滤泡树突状细胞肿瘤是一种罕见的低度恶性肿瘤,临床病理对其认识不足,易误诊,有必要加强对这种罕见疾病的认识。

肝脏炎性假瘤样滤泡树突状细胞肿瘤1例报告及文献复习

目的:探讨肝脏炎性假瘤样滤泡树突状细胞肿瘤(IPT-like FDCT)的临床病理学特征。方法:对1例肝脏IPT-like FDCT患者(40岁男性,无临床症状,体检时发现肝脏内有占位性病变)的肿瘤组织进行临床病理学、免疫组织化学及行EBV转录的核内小RNA(EBER)原位杂交分析。结果:肿瘤与周围组织间有明显界限,中央可见坏死和出血。炎性背景较突出,主要包含淋巴细胞和浆细胞及少量嗜酸性细胞、中性粒细胞。肿瘤细胞呈束状、席纹状排列,更多的是散在分布于炎细胞间;瘤细胞呈短梭形,胞质丰富,淡伊红染色,核呈梭形或卵圆形,具有异型性且呈空泡状,可见核仁。免疫表型示,瘤细胞表达CD21、CD35和簇蛋白(clusterin),EBER原位杂交阳性。手术切除肿瘤后随访3个月,患者未出现复发和转移。结论:IPT-like FDCT是一种罕见的低度恶性肿瘤,其诊断与鉴别诊断依赖于组织病理学检查及免疫组化标记。

Feasibility of laparoscopic isolated caudate lobe resection for rare hepatic mesenchymal neoplasms

BACKGROUND Mesenchymal tumors such as perivascular epithelioid cell neoplasm(PEComa)and inflammatory pseudotumor-like follicular dendritic cell sarcoma(IPT-like FDC sarcoma)are relatively uncommon in the liver and are particularly rare in the caudate lobe.The clinical manifestations and available imaging tests lack specificity for hepatic mesenchymal tumors.To the best of our knowledge,no caudate PEComa or IPT-like FDC sarcoma has been completely resected by laparoscopy.The standard laparoscopic technique,surgical approaches,and tumor margins for potentially malignant or malignant caudate mesenchymal tumors are still being explored.AIM To assess both the safety and feasibility of laparoscopic resection for rare caudate mesenchymal neoplasms.METHODS Eleven patients who underwent isolated caudate lobe resection from 2003 to 2017 were identified from a prospective database.Three consecutive patients with rare caudate mesenchymal tumors underwent laparoscopic resection.Patient demographic data,intraoperative parameters,and postoperative outcomes were assessed and compared with the open surgery group.RESULTS All procedures for the three resection patients with caudate mesenchymal tumors were completed using a total laparoscopic technique by two different approaches.The average operative time was 226 min,and the estimated blood loss was 133 mL.The average length of postoperative hospital stay was 6.3±0.3 d for the laparoscopy group and 15.5±2.3 d for the open surgery group(P<0.05).There were no perioperative complications or patient deaths in this series.CONCLUSION Laparoscopic isolated caudate lobe resection for rare mesenchymal neoplasms is a feasible and curative surgical option in selected patients.

少见类型的肝脏良性肿瘤和瘤样病变

目的 总结少见类型的肝脏良性肿瘤和瘤样病变的诊治经验。方法 对 1980年1月～ 2 0 0 0年 6月 ,收治的并经手术和病理证实的 5 8例原发于肝脏的少见的良性肿瘤和瘤样病变的临床资料进行回顾性分析。结果 5 8例中炎性假瘤 2 6例 ,局灶性增生性病变 8例 ,肝细胞腺瘤 7例 ,脂肪类肿瘤 7例 ,囊腺瘤 5例 ,肝结核 3例及错构瘤和神经纤维瘤各 1例。男性 33例 ,女性 2 5例。年龄 2 0～ 6 6岁 ,平均 42 7岁。 82 8%的病人有临床症状 ,17 2 %的病人无任何症状。 93 1%为单发病灶 ,6 9%为多发病灶。手术方式 :病灶局部切除 30例 ,肝段切除 14例 ,肝叶切除 9例。肝活检 2例 ,肝动脉结扎 2例 ,囊腔引流加肝动脉结扎 1例。未发生严重并发症和手术死亡。结论 近年随着影像检查技术的普及 ,少见类型的肝脏良性肿瘤和瘤样病变的发生率有所增加。然而影像技术在诊断上缺乏特异性 ,在鉴别诊断上往往存在困难。故我们应熟悉上述病变的临床情况。

肝脏炎性假瘤样滤泡树突细胞肉瘤一例及文献复习

目的探讨肝脏炎性假瘤样滤泡树突细胞肉瘤的临床诊断及影像学特点。方法回顾性分析一例肝炎性假瘤样滤泡树突细胞肉瘤患者的临床资料及影像学检查,并复习有关文献。结果肝炎性假瘤样滤泡树突细胞肉瘤是一种极为罕见的低度恶性肿瘤,临床表现为不明原因发热、消瘦,血清学肿瘤标志物阴性。超声表现为肝内巨大实性占位,边界清晰。彩色多普勒显示肿块周边血流信号,内部血流信号稀少。CT增强动脉期病灶不均匀性强化,门脉期、延迟期消退不明显,中央多见液化坏死无增强区。结论肝脏炎性假瘤样滤泡树突细胞肉瘤影像学具有一定的特点,但特异性不明显。主要依靠病理组织学及免疫组化确诊,手术切除肿瘤是有效的治疗手段。

脑内假瘤样脱髓鞘的MRI表现特点

目的:探讨脑组织内假瘤样脱髓鞘的MRI表现特点。方法:对7例假瘤样脱髓鞘的MRI表现进行回顾性分析,并总结其表现特点。结果:MRI显示7例均表现为单发局限性肿块样病灶,呈斑片长T1长T2信号灶,Gd-DTPA增强显示不同程度增强,5例表现为不完整开环样或弓状强化,2例表现为斑片样强化,5例经激素治疗3周后复查病灶均缩小并无明显强化。结论:MRI能清楚的显示假瘤样脱髓鞘的病变特点,对早期诊断有一定的特征性,并能监测病灶的转归情况。

脾脏炎性假瘤样滤泡树突细胞肉瘤CT表现及病理学特征

目的探讨脾脏炎性假瘤样(IPT-like)滤泡树突细胞肉瘤(FDCS)的CT表现及病理学特征。方法收集经病理学证实的脾脏IPT-like FDCS 4例,其中男性1例,女性3例;年龄24~68岁,中位年龄52岁。结合4例患者CT平扫加增强图像,分析病灶的位置、大小、形态、密度及强化方式和强度,探讨其CT表现与病理间的联系。结果病理所见IPTlike FDCS以淋巴细胞、浆细胞为背景,部分病灶见大量炎性细胞浸润,肿瘤细胞混杂其中,免疫组织化学标志物CD21、CD34、CD35表达阳性。EB病毒编码的小分子RNA (EBER)原位杂交显示EBER-ISH(+)。4例脾脏IPT-like FDCS的CT平扫表现为等或稍低密度肿块,内见液化、坏死区,境界尚清,动脉期病灶边缘强化,门静脉期及延迟期实性部分持续性强化。结论脾脏IPT-like FDCS的CT表现具有一定特征性,病灶一般较大,常合并出血、坏死,平扫以等或稍低密度为主,强化方式呈持续性强化,可为临床诊断及术前评估提供重要依据,但最后确诊仍依赖病理学检查。

脾脏炎性假瘤样滤泡树突细胞瘤的CT表现及文献复习

目的:研究脾脏炎性假瘤样滤泡树突细胞瘤的CT表现及病理学特征。方法:回顾性分析2例经手术和病理证实为炎性假瘤样滤泡树突细胞瘤的临床特点、CT表现及病理资料,并复习相关文献,总结炎性假瘤样滤泡树突细胞瘤的CT及病理学特征。结果:2例炎性假瘤样滤泡树突细胞瘤CT平扫表现为类圆形低密度肿块影,边界清晰,密度均匀,增强扫描动脉期示病灶边缘强化,门脉期病灶呈渐进性持续强化并向中央填充。病理:炎性假瘤样滤泡树突细胞瘤除了梭形细胞和炎细胞外,还有由少许多核巨细胞和组织细胞构成的伴有中心坏死的肉芽肿样改变;EBER原位杂交:EBER-ISH(+)。结论:脾脏炎性假瘤样滤泡树突细胞瘤CT动态增强扫描具有渐进性、持续性强化的特点,其CT表现与部分脾脏炎性假瘤相似,确诊需镜检及免疫组织化学检查。

脾脏炎性假瘤样滤泡树突状细胞肉瘤1例报告并文献复习

炎性假瘤样滤泡树突状细胞肉瘤(inflammatory pseudotumor-like follicular dendritic cell sarcoma,IPT-FDCS)是一种非常罕见的肿瘤,恶性程度较低,大多发生于肝脏和脾脏。本文报告1例与Epstein-Barr病毒相关的IPT-FDCS患者的诊治情况,并结合文献分析认为脾脏IPT-FDCS的发病可能与Epstein-Barr病毒感染和免疫球蛋白G相关性疾病相关。目前,脾脏IPT-FDCS的诊断主要依赖于显微镜下的形态学和免疫组织化学染色检查,首选治疗方案是根治性脾脏切除术,患者预后较好。

谢立科主任治疗眼眶炎性假瘤经验撷菁

眼眶炎性假瘤是一种常见的眼眶疾病,病因不明,临床表现多样,西医多采用皮质类固醇等抗炎治疗,较易复发,预后较差。谢立科主任在临床中着重以中西医结合治疗,效果较佳,本文就谢师对其病因、分型、表现、鉴别、治疗、愈后护理等方面进行探析。

炎性假瘤样滤泡树突细胞肉瘤的临床病理学特征

目的探讨炎性假瘤样滤泡树突细胞肉瘤(IPT-like FDCS)的临床病理学特征及其鉴别诊断。方法运用组织病理学、免疫组织化学染色,回顾性分析3例IPT-like FDCS的特点,并结合相关文献进行讨论。结果肿瘤由圆形、卵圆形及梭形细胞组成,呈编织状、漩涡状或席纹状排列,与周围组织分界清。肿瘤细胞呈合体状,染色质细颗粒状,可见单个小核仁,细胞轻度异型,其间见多量淋巴细胞、浆细胞混杂。免疫组化结果:肿瘤细胞不同程度地表达CD21、CD35、CD23滤泡树突细胞标记物,伴随IgG4浆细胞局灶性高表达。EBER原位杂交显示瘤细胞散在阳性。结论IPT-like FDCS是一种好发于女性的少见低度恶性肿瘤,与EB病毒感染有关,最常见的发病部位是脾脏和肝脏,对其认识不足时很容易误诊或漏诊。

脾脏炎性假瘤样滤泡树突状细胞肿瘤1例

患者男,47岁,因“反复低热伴下肢乏力半年余”入院;既往体健。查体无特殊,双下肢肌力正常(5级)。实验室检查C反应蛋白升高(10μg/ml)。腹部超声:脾脏内5.5 cm×5.0 cm混合回声团块,边界清晰,内部回声不均匀,CDFI于其内见短线状血流信号;超声造影示脾内病灶动脉期快速增强,静脉期减退呈低回声改变,内见无强化区(图1A、1B);考虑脾少见类型恶性肿瘤可能。