Imaging findings of inflammatory pseudotumor-like follicular dendritic cell sarcoma of the spleen: A case report and literature review
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SUBMITTER: Zhang J
PROVIDER: S-EPMC9533655 | biostudies-literature | 2022 Sep
REPOSITORIES: biostudies-literature
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Project description:BackgroundFollicular dendritic cell sarcoma is a rare stromal tumor with no standard treatment. However, some reports have revealed that follicular dendritic cell sarcoma has an inflammatory pseudotumor variant associated with Epstein-Barr virus infection that has a relatively good prognosis. In this report, we present a case of a resected inflammatory pseudotumor variant of follicular dendritic cell sarcoma of the liver, and have reviewed the literature on the clinicopathological, molecular, and genomic features of this tumor.Case presentationThe inflammatory pseudotumor variant of follicular dendritic cell sarcoma originates only in the liver or spleen, causes no symptoms, and is more common in middle-aged Asian women. It has no characteristic imaging features, which partially explains why the inflammatory pseudotumor variant of follicular dendritic cell sarcoma is difficult to diagnose. Pathologically, the inflammatory pseudotumor variant of follicular dendritic cell sarcoma has spindle cells mixed with inflammatory cells and is variably positive for follicular dendritic cell markers (CD21, CD23, and CD35) and Epstein-Barr virus-encoded RNA. On genetic analysis, patients with this tumor high levels of latent membrane protein 1 gene expression and extremely low levels of host C-X-C Chemokine Receptor type 7 gene expression, indicating that the inflammatory pseudotumor variant of follicular dendritic cell sarcoma has a latent Epstein-Barr virus type 2 infection.ConclusionsThe inflammatory pseudotumor variant of follicular dendritic cell sarcoma is an Epstein-Barr virus-associated tumor and a favorable prognosis by surgical resection, similar to Epstein-Barr virus-associated gastric cancer.
| S-EPMC9733763 | biostudies-literature
Project description:RationaleHepatic inflammatory pseudotumor (IPT)-like follicular dendritic cell (FDC) sarcoma is a very rare disease. Till now, only 19 cases were reported in the English literature. However, the coexistence of IPT-like sarcoma and non-Hodgkin lymphoma (NHL) in the same patient has never been reported. In this report, we presented a case of hepatic IPT-like FDC with hepatic NHL history of which both were successfully resected.Patient concernsWe reported a case of a 47-year-old male patient who presented with right upper abdominal discomfort. Nineteen years ago, he underwent liver resection of segment VII for hepatic NHL (B-cell lymphoma). He had a history of chronic hepatitis B virus infection. Serum alpha fetoprotein level was normal. However, imaging studies revealed a well-circumscribed, solid mass in the right hepatic lobe, he came back to the clinic because he was worried about a recurrence of the B-cell lymphoma.DiagnosesBased on the patient's past medical history and magnetic resonance imaging results, and he was diagnosed as hepatocellular carcinoma or hepatic NHL preoperatively.InterventionsRight hemi-hepatectomy was performed on this patient.OutcomesHistological report showed features of a mixture of chronic inflammatory cells and variable amounts of spindle cells. Also, immuno-histo-chemical studies demonstrated that all the tumor cells showed strong nuclear in situ labeling for EBV-encoded small RNAs and strongly positive stainings with CD21 and CD35. The patient tolerated the surgery well, recovered smoothly and he was discharged on postoperative day 7 (day 7). The patient is still disease free after a follow-up of over 50 months.ConclusionsTo our knowledge, this is the first report demonstrating hepatic IPT-like FDC sarcoma in a patient with primary hepatic NHL history. In regards to treatment, complete surgical resection should be performed and would acquire excellent long-term outcomes.
| S-EPMC8483863 | biostudies-literature
Project description:Pancreatic follicular dendritic cell sarcoma (FDCS) is a rare neoplasm with unclear pathological characteristics. In this study, we report one case of pancreatic FDCS and review published cases to summarize the characteristics and treatment of pancreatic FDCS. A man in his early 30 s was admitted for jaundice, abdominal fullness, and weight loss for 15 days. Computed tomography revealed a large capsule solid mass in the pancreatic head together with a dilated bile duct and enlarged retroperitoneal lymph nodes. Serum biochemistry revealed high total bilirubin levels (313.9 μmol/L) and normal tumor marker levels. Pancreatoduodenectomy was performed, but no chemotherapy was administrated at the patient's behest. The pathologic diagnosis was pancreatic FDCS infiltrating the duodenal seromuscular layer and common bile duct. The patient presented with liver metastasis 3 months after surgery and died 8 months after surgery from multiorgan failure. Pancreatic FDCS is a rare disease with high invasiveness. Our previous case exhibited paraneoplastic syndrome together with this disease, and further investigation is needed to confirm whether paraneoplastic syndrome is a typical syndrome of pancreatic FDCS.
| S-EPMC9791293 | biostudies-literature
Project description:Primary pulmonary inflammatory pseudotumor-like follicular dendritic cell sarcoma (IPT-like FDCS) is extremely rare. Here, we report a case of a 64-year-old female with primary pulmonary IPT-like FDCS. The patient was found to have a solid nodule in the right lower lobe (RLL) of the lung incidentally without any symptoms or signs of discomfort. The chest computed tomography (CT) showed that there was an irregular nodule in the basal segment of the RLL, approximately 2.0 cm × 1.1 cm × 1.0 cm in size, of 15 HU in CT value. While the result of the fiberoptic bronchoscope-guided biopsy of the mass showed that there was inflammatory cell infiltration, no evidence of malignancy was found. After a thorough discussion of the multidisciplinary team, lobectomy of the RLL and systematic lymph node dissection were performed for the patient. Histologic analysis of the resected mass revealed infiltration of a large number of lymphocytes and plasma cells with the expression of CD21, CD23, CD35 were positive. In addition, the Epstein-Barr virus (EBV) probe in situ hybridization were positive. As a result, the diagnosis of EBV-positive IPT-like FDCS was strongly supported. No recurrence or any signs of metastasis were found during a 10-month follow-up time. As we have reported in this rare case, the diagnosis of primary pulmonary IPT-like FDCS should be considered even when there is only lymphoplasmacytic infiltration and no evidence of malignant tumor cells in the lung.
| S-EPMC7859798 | biostudies-literature
Project description:GoalsThe main purpose of this study is to broaden the clinicopathological spectrum and increase recognition of follicular dendritic cell sarcoma (FDCS) through analysis of the clinical and pathological features of 50 cases.MethodsThe clinicopathological features of total 50 cases of FDCS were analyzed including a review of 44 cases reported in Chinese literature before October 2009 and six original cases from the pathology files conducted by the authors.ResultsThe youngest patient came under observation in this study is only seven years old. Including the cases contributed by the authors, our literary review indicated that male dominated the tumor cases (M: F = 3: 2). 28 cases (56%) present with this disease in extranodal sites. Tumor cells demonstrated positive staining for the follicular dendritic cell markers CD21 (47/49), CD35 (43/45), CD23 (20/23) and CD68 (23/25). In situ hybridization for Epstein-Barr virus-encoded RNA was performed in 10 cases. Nevertheless, EBV expression was absent in all these cases. The follow-up analysis of all cases shows that 26 (81.2%) patients were alive and disease free; 6 (18.8%) patients were alive with recurrent disease or metastasis; and nobody had died of this disease at the time of last follow-up.ConclusionsThe diagnosis of the FDCS is based on the findings of morphology and immunohistochemistry. The FDCS occurred in China should be viewed and treated as a low-grade sarcoma, and the role of the EBV in the pathogenesis of this tumor is still uncertain. There is a possibility that the tumor might be racial or geographic correlated, because most cases were reported from Eastern Asia area; it's particular the case of the liver or spleen tumor.
| S-EPMC2964601 | biostudies-literature
Project description:EBV-positive inflammatory follicular dendritic cell sarcoma (EBV+ IFDCS) is an uncommon disease primarily observed in Asia. It is characterized by the development of tumors believed to originate from follicular dendritic cells (FDC). The consistent association between this condition and clonal EBV infection suggests EBV's involvement as an etiological factor. However, diagnosing EBV+ IFDCS can be challenging due to its morphological variability and diverse immunohistochemical staining patterns. The genetic characteristics of EBV+ IFDCS remain insufficiently understood. To address this knowledge gap, we present a case study of a 47-year-old male patient diagnosed with EBV+ IFDCS. We utilized a Next-generation sequencing (NGS) platform to investigate the genetic profile of the tumor cells. We identified a single pathogenic mutation (G618R) in the STAT3 gene. This finding provides valuable insights into the genetic alterations associated with EBV+ IFDCS and potentially contributes to our understanding of the disease's pathogenesis.
| S-EPMC10640977 | biostudies-literature
Project description:Follicular dendritic cell sarcoma (FDCS) is a rare malignant neoplasm that was classified by the World Health Organization (WHO) under histiocytic and dendritic cell neoplasms in the 2016 revision. Considering the rarity of this tumor, there is no standardized treatment. It is usually treated by complete surgical resection. Adjuvant chemotherapy and radiotherapy are alternative methods. Immune checkpoint inhibitors (ICIs) represented by the programmed death receptor 1/programmed death ligand 1 (PD-1/PD-L1) antibody have achieved significant clinical benefits in a variety of solid tumors. However, reports on the treatment of FDCS with ICIs are rare. FDCS often expresses high levels of PD-L1, which provides a rationale to use immunotherapy in cases of FDCS. Here, we present a 51-year-old Filipino-Chinese man with FDCS who was treated with multimodal treatment, including the PD-1 inhibitor pembrolizumab and achieved a relatively long disease-free survival of 24 months. This case emphasizes that the application of ICIs under the guidance of NGS technology seems to be a meaningful treatment option for patients with FDCS.
| S-EPMC8922364 | biostudies-literature
Project description:Immunoglobulin G4-related disease (IgG4-RD) is an autoimmune disease that affects several organs. An inflammatory pseudotumor is a histologically proven benign tumor-like lesion that most commonly involves the lung and orbit. It is rare in the central nervous system, but rarest in the spinal canal. In this report, we present a case of IgG4-related intramedullary spinal inflammatory pseudotumor, along with a literature review. A 29-year-old male was transferred to the Department of Neurosurgery of Lanzhou University Second Hospital with progressive quadriparesis after numbness and weakness in both lower limbs for 50 days. Enhanced magnetic resonance imaging (MRI) of the spine revealed an isointense signal on T1-weighted images and a hyperintense signal on T2-weighted images from an enhanced mass located at the thoracic vertebrae region, for which a schwannoma was highly suspected. Then, a posterior median approach was adopted. The lesion was resected. The patient received further glucocorticoid after the diagnosis of an IgG4-related inflammatory pseudotumor was established, and the patient's symptoms improved, such as quadriparesis and lower limb weakness. This case highlights the importance of considering IgG4-related inflammatory pseudotumor as a differential diagnosis in patients with lesions involving the spinal intramedullary compartment and lower limb weakness when other more threatening causes have been excluded. IgG4-related inflammatory pseudotumor is etiologically unclear and prognostically unpredictable, and imaging may not help establish the diagnosis of IgG4-related inflammatory pseudotumor due to its resemblance to malignant tumors, and total resection might not be warranted. Glucocorticoid and surgery are usually the first-line treatments used.
| S-EPMC9275449 | biostudies-literature
Project description:Angioimmunoblastic T-cell lymphoma (AITL) is a common type of nodal peripheral T-cell lymphoma, which always presents with extensive follicular dendritic cell (FDC) meshwork. Here, we report a case of AITL combined with extensive spindle cell meshwork. Spindle cells occupied were positive for the FDC markers CD21, CD23, and CD35. Furthermore, some cells were positive for desmin and smooth muscle actin (SMA), suggesting the differentiation of fibroblastic reticular cell (FRC). Interestingly, the proliferation of spindle cells was so extensive that was easily misdiagnosed as FDC sarcoma (FDCS). Next-generation sequencing showed that the common mutations reported in AITL, including RHOA, TET2, and IDH2, were also detected in this case, while the genes that are recurrently mutated in FDCS were not detected. Regrettably, the patient died 19 months later. Overall, we highlight the unusual morphologic features in an AITL patient with extensive FDC and FRC network that may be misdiagnosed as FDCS, and careful morphological observation and immunochemical and molecular examinations are crucial for an accurate diagnosis.
| S-EPMC9638041 | biostudies-literature
Project description:Follicular dendritic cell sarcoma (FDCS) is a rare sarcoma subtype, presenting as a relatively indolent disease in most cases. Given its rarity, clinicopathological characteristics and behavior as well as treatment, are reported in the literature through case reports and case series. Bone metastasis in FDCS is extremely rare and the outcome of the disease in this group of patients is unknown. We report one case of FDCS with bone involvement as the first site of metastasis. We present the progression of the disease over a period of almost a decade in a detailed manner, particularly the therapies used including immunotherapy with checkpoint inhibitors. In parallel, we provide a comparison with other cases of FDCS metastatic to the bone (total of eight cases) through a systematic review of the literature on the clinical and pathological manifestations as well as the outcomes of all such cases reported to date, to the best of our knowledge. These cases highlight the challenges associated with setting the correct diagnosis at presentation, the lack of evidence to support the role of adjuvant therapy following primary surgery but also the role and/or sequence of systemic options in the advanced setting.
| S-EPMC11877466 | biostudies-literature