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.

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.

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.

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.

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.

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。

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。

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。

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。

腹部滤泡树突细胞肉瘤1例报道及文献复习

目的探讨发生在腹部的滤泡树突细胞肉瘤(follicular dendritic cell sarcoma,FDCS)的临床病理特征、治疗及预后。方法报道陕西中医药大学第二附属医院收治的1例罕见的胰腺FDCS,并回顾国内外相关案例报道资料,进行分析总结。结果该例胰腺FDCS病人为女性,66岁,因体检发现胰体尾占位入院,CT示肿瘤边界清楚,大小约9 cm,脾静脉受压。糖类抗原(CA)125升高,行胰体尾联合脾切除术,病理检查肿瘤呈梭形细胞,核仁明显,核分裂易见,CD21、CD35、fascin(+),EB病毒(EBV)编码的小RNA(EBV encoded RNA,EBER)原位杂交(-),Ki-67约20%,病理诊断为:胰腺FDCS。未进行其他辅助治疗,随访半年无复发转移。文献复习共收集49例腹部FDCS,加上此例共50例一并纳入研究:(1)病人的年龄范围19~77岁,平均年龄50岁,多无特殊病史。(2)肝脏及腹腔腹膜后最多见,脾脏、大肠、胰腺、小肠、回盲部、阑尾均可发生。(3)男女发病无明显差异。(4)病人可无明显症状或出现不同程度腹部症状,部分为腹部以外症状,如皮损改变、呼吸道症状等。(5)术前实验室检查多无明显异常,10%病人CA125升高。(6)超声和CT是主要的辅助检查手段,大肠FDCS内镜下均表现为息肉外观,22%的病人初诊时出现转移。(7)巨检几乎均表现为边界清楚的单发、实性肿物,肿瘤最大径为1~30 cm;确诊常需要联合多种特异性标志物,如CD21、CD35、Fascin、CD23等,并结合组织学形态做出诊断;大多数EBER原位杂交阳性。(8)96%的病人以手术治疗为主,大多数术后无瘤生存,少数出现复发、转移,少数同时辅助化疗和(或)放疗,疗效有待进一步证实。结论腹部FDCS是一类罕见的中度恶性肿瘤,具有较高的局部复发和转移的风险,临床发病特点缺乏特异性。其发病机制可能与EBV感染和免疫系统疾病有关。手术切除是该病首选的治疗方式,术后放化疗对于预后是否改善尚不确定。但如果存在高危因素,建议术后长期随访、定期复查并积极进行干预。

扁桃体滤泡树突细胞肉瘤1例并文献复习

目的探讨扁桃体滤泡树突细胞肉瘤的临床和病理特点,提高对其诊治水平。方法运用组织病理学、免疫组化及电镜观察扁桃体滤泡树突细胞肉瘤的特征,总结临床特点和治疗情况,并复习文献加以分析。结果瘤组织常排列成结节状、旋涡状和席纹状,瘤细胞呈梭形合体状,核椭圆形,染色质点彩状,有小核仁。瘤细胞间常混有小淋巴细胞。瘤细胞CD35、S-100、CD68和Vim(+),CKAEl、CKAE3和LCA(-)。电镜下见瘤细胞胞质有多个长突起彼此相连。结论滤泡树突细胞肉瘤是一种少见的低度恶性肿瘤,发生于扁桃体者更为罕见,诊断依赖组织病理学及免疫组化标记。

淋巴结内指突状树突细胞肉瘤临床病理观察

目的探讨淋巴结内指突状树突细胞肉瘤的诊断要点及其鉴别诊断。方法对1例淋巴结内指突状树突细胞肉瘤进行组织病理学、免疫组化染色及电镜观察,并随访。结果患者临床表现为右颈部孤立性无痛性淋巴结肿大,易于复发。由于患者拒绝接受治疗,于确诊后15个月死亡。镜检显示淋巴结结构破坏,由呈束状、编织状排列的梭形和卵圆形细胞替代;瘤细胞vimentin、S_100(+),LCA、CD68灶性弱(+),CD21、CD35、CD1a及T、B细胞标记(-);电镜下瘤细胞表面可见细长的指状突起,未见到Birbeck颗粒、细胞间桥粒连接及黑素小体等特殊结构。结论指突状树突细胞肉瘤极为罕见,容易漏诊和误诊,熟悉其临床病理特点,依靠免疫组化和电镜可确诊。

滤泡树突细胞肉瘤6例临床病理分析

目的探讨滤泡树突细胞肉瘤的病理诊断及鉴别诊断。方法应用组织病理学及免疫组化观察6例(包括2例细针穿刺组织)滤泡树突细胞肉瘤的特点,并结合文献探讨其病理形态及鉴别诊断等。结果镜检肿瘤细胞为梭形、卵圆形,胞质丰富,可见小核仁,细胞呈片状、束状、编织状或漩涡状排列。免疫组化显示肿瘤细胞特异性地表达CD21、CD23、CD35、fascin、clusterin、D2-40、EMA,部分病例表达CXCL-13。结论滤泡树突细胞肉瘤是少见的低度恶性肿瘤,形态学上表现为梭形细胞呈漩涡状脑膜瘤样结构,CD21、CD35、EMA对于确诊有重要意义。Fascin、clusterin及CXCL-13可能成为滤泡树突细胞肉瘤的新标记物。

滤泡树突状细胞肉瘤相关的副肿瘤天疱疮

报告1例滤泡树突状细胞肉瘤相关的副肿瘤天疱疮。患者男,16岁。因口腔、外阴溃疡伴发热2个月余,全身糜烂20d入院。临床表现为口腔、生殖器溃疡,全身水疱、糜烂及结痂,皮损组织病理改变符合副肿瘤天疱疮。腹部CT示右肾上腺占位性病变,手术切除并经组织病理证实为滤泡树突状细胞肉瘤。经大剂量静脉注射用人免疫球蛋白及口服、静脉滴注糖皮质激素和免疫抑制剂治疗后,采用手术切除肿瘤,术后7周躯干皮损基本愈合,遗留黏膜损害及肢端皮损。

指突状树突状细胞肉瘤诊治探讨

目的:探讨树突状细胞肉瘤的临床特征、诊断、治疗和预后。方法:对1例指突状树突状细胞肉瘤进行临床病理特点分析,CHOP(环磷酰胺、表阿霉素、长春地辛、地塞米松)方案化疗并观察疗效,复习文献总结国内树突状细胞肉瘤诊治情况。结果:临床表现为全身淋巴结肿大,颈部呈巨大肿块,两肺弥漫性微小结节,伴盗汗、轻度贫血。病理结果示淋巴结结构破坏,瘤细胞成梭形,形成束状,交错状排列。免疫组化S-l00(+)、Vimentin(+)、Fascin(+)、Lyso(+)、Ki-67(+),部分肿瘤细胞CD68(+)。经CHOP方案化疗后达到部分缓解。Langerhans细胞肉瘤罕见,呈高度恶性表现,常累及多个器官,目前国内仅3例报道。指突状树突状细胞肉瘤国内共报道6例,年龄19～41岁(中位33岁),颈部淋巴结好发,手术后无病生存时间2～15月(中位10月),18月总生存率40%(2/5)。滤泡树突状细胞肉瘤18例,年龄16～63岁(中位37岁),发病部位1/3以上在结外,无病生存时间5～72月(中位11.5月),18月总生存率100%(6/6)。结论:树突状细胞肉瘤诊断依赖病理和免疫组化;治疗尚需规范,可采取手术、放疗、化疗相结合的方案。