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.

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.

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.

Isolated lymphoid follicles in colon: Switch points between inflammation and colorectal cancer?

Gut-associated lymphoid tissue is supposed to play a central role in both the organization of colonic repair mechanisms and colorectal carcinogenesis. In inflammatory conditions, the number, diameter and density of isolated lymphoid follicles (ILFs) increases. They are not only involved in immune surveillance, but their presence is also indispensable in normal mucosal regeneration of the colon. In carcinogenesis, ILFs may play a dual role. On the one hand they may support tumor growth and the metastatic process by vascular endothelial growth factor receptor signaling and producing a specific cytokine and cellular milieu, but on the other hand their presence is sometimes associated with a better prognosis. The relation of ILFs to bone marrow derived stem cells, follicular dendritic cells, subepithelial myofibroblasts or crypt formation, which are all involved in mucosal repair and carcinogenesis, has not been directly studied. Data about the putative organizer role of ILFs is scattered in scientific literature.

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.

B cell development and antibody responses in human immune system mice:current status and future perspective

Humanized immune system(HIS)mice have been developed and used as a small surrogate model to study human immune function under normal or disease conditions.Although variations are found between models,most HIS mice show robust human T cell responses.However,there has been unsuccessful in constructing HIS mice that produce high-affinity human antibodies,primarily due to defects in terminal B cell differentiation,antibody affinity maturation,and development of primary follicles and germinal centers.In this review,we elaborate on the current knowledge about and previous attempts to improve human B cell development in HIS mice,and propose a potential strategy for constructing HIS mice with improved humoral immunity by transplantation of human follicular dendritic cells(FDCs)to facilitate the development of secondary follicles.