Long-Term Remission with Novel Combined Immune-Targeted Treatment for Histiocytic Sarcoma Accompanied by Follicular Lymphoma: Case Report and Literature Review
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SUBMITTER: Zhang M
PROVIDER: S-EPMC11242306 | biostudies-literature | 2024 Jul
REPOSITORIES: biostudies-literature
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Project description:Histiocytic sarcoma (HS) is a rare hematopoietic neoplasm with an aggressive clinical course and a poor response to conventional chemotherapy. Currently, no standard treatment paradigms are available. Herein, we present a case of de novo HS treated with pembrolizumab combined with a GDP regimen (gemcitabine, cisplatin, and dexamethasone) that resulted in sustained complete remission with progression-free survival exceeding 4 years. Immunohistochemical analysis demonstrated significant overexpression of programmed death ligand 1 (PD-L1) on biopsy samples. Additionally, fluorescence in situ hybridization (FISH) with a JAK-2 probe indicated 9p24.1 amplification, suggesting reliance on the JAK-STAT pathway. Polymerase chain reaction (PCR) analysis did not reveal any BRAF-V600 mutations. Consequently, an immune checkpoint inhibitor (ICI) was administered alongside chemotherapy, resulting in sustained complete remission and progression-free survival for over 4 years. Our findings suggest that a combination of ICI and chemotherapy could represent a promising therapeutic approach for HS.
| S-EPMC11366101 | biostudies-literature
Project description:Non-Hodgkin lymphomas comprise a heterogeneous group of malignancies, with a wide scope of clinical, radiological and histological presentations. In this paper, a case is presented of a 59-year-old white male with an infraorbital follicular B-cell lymphoma, which appeared as a painless mass in the left cheek. The lymphoma achieved spontaneous remission five and a half months after his diagnostic incision biopsy. The literature is reviewed, focusing on this rare site of presentation and spontaneous remission. In literature, only four cases have been reported with a follicular B-cell lymphoma of the cheek or infraorbital region, and only 26 cases of spontaneous remission of an extracranial non-Hodgkin lymphoma in the head and neck region have been described. To the authors' best knowledge, this is the first time spontaneous remission of an infraorbital follicular lymphoma could be observed. The nature of the processes inducing spontaneous remission remains obscure. It is important to recognize this phenomenon as this might prevent unnecessary treatment.
| S-EPMC8262163 | 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:BackgroundHistiocytic sarcoma (HS) is a rare hematopoietic malignancy with an aggressive clinical presentation associated with a poor overall survival. To date, surgical resection, radiation therapy, and chemotherapy were often utilized for HS, but curative effects are rather disappointing.Case presentationA 19-year-old female was referred to our hospital with a pathologic diagnosis of HS in December 2017. The patient had a severe airway obstruction resulting from a large mass (6.0 cm × 4.4 cm) arising from the left parapharyngeal space. She did not respond to cyclophosphamide, doxorubicin, vincristine, prednisone, and etoposide (CHOEP) chemotherapy, then she was switched to radiotherapy and crizotinib according to next-generation sequencing (NGS) results (mutations in MET and MAP2K1). The patient got a partial response after radiotherapy and crizotinib, then she switched to imatinib combined with thalidomide treatment. The patient got a long-term complete response from the treatment and is alive 44 months after initial diagnosis without disease progression. Further KEGG pathway enrichment analysis of NGS results from patient's tissue revealed that phosphatidylinositol 3' kinase (PI3K)/AKT and mitogen-activated protein kinase (MAPK) pathways were activated in this HS patient. We further performed experiments in vitro in a canine histiocytic sarcoma cell line DH82, in order to explore the possible mechanism of imatinib plus thalidomide in HS. Results of cell counting kit-8 (CCK8) assays showed that the proliferation activity of DH82 was significantly inhibited by imatinib but not thalidomide. Combined thalidomide and imatinib treatment did not improve the inhibitory effects of imatinib to DH82. Results of Western blot confirmed the inhibitory effects of imatinib on DH82 by targeting activation of MAPK and PI3K/AKT pathways.ConclusionRadiotherapy combined with targeted therapy guided by NGS may be promising, and further perspective clinical trial is warranted for the localized HS.
| S-EPMC8685210 | biostudies-literature
Project description:Introduction and importanceHistiocytic sarcoma (HS) is a rare malignant neoplasm showing morphologic and immunohistochemical features of histiocytes. It is characterized typically by extranodal presentation and a poor clinical course, particularly in cases with disseminated disease.Case presentationThis report documents a case of bifocal and aggressive HS in small bowel and lung revealed by acute peritonitis in a 63-year-old man.Clinical discussionDespite its rarity, we believe that the correct diagnosis of HS is crucial for clinical treatment and prognostic prediction.ConclusionThe collection of additional cases of HS are important to obtain further progress in prognosis and guide treatment decisions.
| S-EPMC8339142 | biostudies-literature
Project description:High-throughput microarray technologies were used to study DNA methylation accompanied by transcriptional changes in follicular lymphoma (FL). Using Methylated CpG Island Amplification with Microarrays to study CpG Island DNA methylation in FL, we discovered widespread hypermethylation of homeobox genes and previously identified targets of polycomb repressive complex 2 (PRC2) in cell lines and primary tumors, but not in benign follicular hyperplasia (BFH). DNA methylation for HOXA11, HOXD10, HOXB7, HOXC12, PAX6, LHX9, SFMBT2, EN2, and PAX7 was independently validated in the RL cell line and HOXA11, HOXD10, PAX6, and EN2 in primary tumors. Combined Bisulfite Restriction Analysis (COBRA) also established DNA methylation for the previously identified PRC2 targets DCC, DES, GAD2, AQP5, GPR61, GRIA4, GJD2, and AMPH in FL but not in BFH. Gene expression analyses revealed 411 genes that were hypermethylated and transcriptionally repressed in RL, 74% of which were reactivated by the demethylating agent 5-aza-2'-deoxycytidine (5-azaD) plus or minus the histone deacetylase inhibitor trichostatin A (TSA). Forty genes were also downregulated in primary FL. Our results suggest that extensive hypermethylation in promoters of polycomb target genes is a characteristic of FL and that loss of expression of certain SUZ12 target genes could be functionally relevant for lymphomagenesis.
| S-EPMC2738630 | biostudies-literature
Project description:Histiocytic sarcoma is a rare hematologic malignancy, with very few cases of primary histiocytic sarcoma of the breast described in English scientific literature. Herein, we describe a case of primary histiocytic sarcoma of the breast in a 75-year-old woman, with no clinical history of malignant tumors, who presented with a palpable solitary breast mass. Microscopically, the resected breast mass showed large pleomorphic cells, some multinucleated giant cells, and admixed inflammatory components. The pleomorphic tumor cells further showed a diffuse, noncohesive growth pattern, an abundant eosinophilic cytoplasm, and strong and diffuse immunoreactivity for cluster of differentiation (CD) 68 and CD163. Furthermore, a whole-body positron-emission tomography/computed tomography using deoxy-2-[18F]fluoro-D-glucose performed after surgery showed no other masses or lesions. After surgical excision, the patient was followed up, and no evidence of tumor recurrence or metastasis was noted.
| S-EPMC6769387 | biostudies-literature
Project description:Follicular lymphoma (FL) occurs primarily in the gastrointestinal tract, with the stomach being one of the rarest sites. According to the literature, <20 cases of primary gastric FL have been reported, with the number of cases with detailed pathological descriptions being even less. The aim of the present study was to compare clinicopathological features of gastric FL with FL at alternative sites. A total of 3 cases of gastric FL were retrieved from among 3,216 cases in the databases of 4 university hospitals in South Korea: Seoul National University (SNU) Hospital, SNU Boramae Hospital, SNU Bundang Hospital (all Seoul, South Korea) and Dongtan Sacred Heart Hospital Hallym University (Dongtan, South Korea), including 2 primary cases and 1 case that was possibly secondary to nodal FL. The 2 primary gastric FL cases were incidentally detected in routine health check-ups. An endoscopy revealed a single polypoid submucosal mass and biopsies failed to confirm the diagnosis due to minimal mucosal involvement. Therefore, a partial gastrectomy was performed. The epicenters of the tumors were submucosal, with focal extension to the muscularis propria. However, 1 case exhibited an isolated FL nodule in the omentum. Histopathological examination revealed FL of grade 1-2 with a follicular pattern and with strong expression of germinal center markers and B-cell lymphoma 2 (BCL2). Rearrangement of BCL2 was not identified using fluorescence in situ hybridization studies in 2 cases. In contrast to these 2 cases, the remaining FL case was confirmed with an endoscopic biopsy. The endoscopy revealed multiple eroded polypoid lesions, and pathology revealed FL of grade 1-2 with a predominantly diffuse pattern, and with immunoglobulin heavy chain IGH/BCL2 translocation. In view of the extensive lymphadenopathy, the last case possibly presented as secondary involvement of nodal FL. It is challenging to diagnose FL in the stomach due to little mucosal involvement, as well as the unfamiliarity of the tumor due to its rarity. However, the results of the present study suggest that primary gastric FL may exhibit unique pathological features, including a predominantly follicular pattern and an absence of BCL2 rearrangement.
| S-EPMC6019973 | 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:Circulating tumor DNA (ctDNA) has become an attractive biomarker in human oncology, and its use may be informative in canine cancer. Thus, we used droplet digital PCR or PCR for antigen receptor rearrangement, to explore tumor-specific point mutations, copy number alterations, and chromosomal rearrangements in the plasma of cancer-affected dogs. We detected ctDNA in 21/23 (91.3%) of histiocytic sarcoma (HS), 2/8 (25%) of oral melanoma, and 12/13 (92.3%) of lymphoma cases. The utility of ctDNA in diagnosing HS was explored in 133 dogs, including 49 with HS, and the screening of recurrent PTPN11 mutations in plasma had a specificity of 98.8% and a sensitivity between 42.8 and 77% according to the clinical presentation of HS. Sensitivity was greater in visceral forms and especially related to pulmonary location. Follow-up of four dogs by targeting lymphoma-specific antigen receptor rearrangement in plasma showed that minimal residual disease detection was concordant with clinical evaluation and treatment response. Thus, our study shows that ctDNA is detectable in the plasma of cancer-affected dogs and is a promising biomarker for diagnosis and clinical follow-up. ctDNA detection appears to be useful in comparative oncology research due to growing interest in the study of natural canine tumors and exploration of new therapies.
| S-EPMC7806858 | biostudies-literature