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Blood Oct 2020Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm that most commonly arises from a small subset of γ/δ T-cell receptor-expressing lymphocytes. HSTCL is... (Review)
Review
Hepatosplenic T-cell lymphoma (HSTCL) is a rare T-cell neoplasm that most commonly arises from a small subset of γ/δ T-cell receptor-expressing lymphocytes. HSTCL is more common in adolescent and young adults and has a rapidly progressive clinical course and poor outcome due to its refractoriness to conventional chemotherapy regimens. Approximately 20% of the cases arise in the background of chronic immunosuppression or immune dysregulation. Patients commonly present with constitutional symptoms, hepatic and liver enlargement, and cytopenias; hematophagocytic syndrome can also occur. The most frequent chromosomal aberrations associated with HSTCL are isochromosome 7q and trisomy 8, and most cases harbor mutations in genes involved in chromatin modification or the JAK/STAT pathway. The rarity of this disease, along with lack of nodal involvement and presenting symptoms that mimic different entities including infectious etiologies, makes this lymphoma a significant diagnostic challenge. In this review, we highlight the clinical and pathologic features of HSTCL. Moreover, we summarize the results of recent molecular studies suggesting potential targets for novel therapeutics strategies.
Topics: Animals; Humans; Liver Neoplasms; Lymphoma, T-Cell; Splenic Neoplasms
PubMed: 32756940
DOI: 10.1182/blood.2019004118 -
Archives of Pathology & Laboratory... Feb 2015Splenic lymphangioma is a rare malformation of the splenic lymphatic channels, mostly seen in children. It is characterized by the presence of cysts, resulting from... (Review)
Review
Splenic lymphangioma is a rare malformation of the splenic lymphatic channels, mostly seen in children. It is characterized by the presence of cysts, resulting from increases in the size and number of thin-walled lymphatic vessels that are abnormally interconnected and dilated. The condition may be restricted to the spleen, but in most cases it involves multiple organs (systemic lymphangiomatosis). The clinical picture is variable; small lesions are often incidentally detected through imaging studies, while larger lesions can result in compression of organs, causing pain or rupture even after minor trauma. Therefore, splenic lymphangiomas should be considered in the differential diagnosis of splenomegaly or left upper quadrant pain even among adults and should be immediately treated with splenectomy; delay in the therapeutic intervention can lead to life-threatening complications.
Topics: Adult; Child; Cysts; Diagnosis, Differential; Humans; Lymphangioma; Prognosis; Spleen; Splenectomy; Splenic Neoplasms; Tomography, X-Ray Computed
PubMed: 25611113
DOI: 10.5858/arpa.2013-0656-RS -
Ugeskrift For Laeger May 2023Littoral cell angioma is a benign vascular tumour of the spleen, and malign transformation is seldom. The angioma is associated with a high risk of simultaneous...
Littoral cell angioma is a benign vascular tumour of the spleen, and malign transformation is seldom. The angioma is associated with a high risk of simultaneous occurrence of other primary cancers, and it is of utmost importance to perform extensive diagnostic investigations to detect other cancers. Definitive treatment of littoral cell angioma is surgical resection of the spleen. This is a unique case report about a 73-year-old woman who had a simultaneous adenocarcinoma of the colon and a gastrointestinal stromal tumour. She underwent simultaneous splenectomy with colonic and gastric resection.
Topics: Female; Humans; Aged; Splenic Neoplasms; Hemangioma; Splenectomy
PubMed: 37264861
DOI: No ID Found -
RoFo : Fortschritte Auf Dem Gebiete Der... Jun 2024Splenic lesions are rare and mostly incidental findings on cross-sectional imaging. Most lesions are of benign nature and can be correctly identified based on imaging... (Review)
Review
BACKGROUND
Splenic lesions are rare and mostly incidental findings on cross-sectional imaging. Most lesions are of benign nature and can be correctly identified based on imaging characteristics. Further, invasive evaluation is only necessary in cases of splenic lesions with uncertain or potentially malignant etiology.
METHOD
While in most cases a correct diagnosis can be made from computed tomography (CT), (additional) magnetic resonance imaging (MRI) can aid in the identification of lesions. As these lesions are rare, only a few of the differential diagnoses are regularly diagnosed in the clinical routine.
RESULT AND CONCLUSION
This review presents the differential diagnoses of splenic lesions, including imaging characteristics and a flowchart to determine the right diagnosis. In conjunction with laboratory results and clinical symptoms, histological workup is necessary only in a few cases, especially in incidental findings. In these cases, image-guided biopsies should be preferred over splenectomy, if possible.
KEY POINTS
· Splenic lesions are rare and are usually incidental findings on abdominal imaging. · CT imaging and MRI imaging are the diagnostic tools of choice for the further workup of splenic lesions. · Based on their image morphological characteristics, a large number of splenic lesions can be assigned to one entity and do not need histological analysis.
CITATION FORMAT
· Krähling H, Seidensticker M, Heindel WL et al. Diagnostic approach to splenic lesions. Fortschr Röntgenstr 2024; 196: 573 - 581.
Topics: Humans; Magnetic Resonance Imaging; Tomography, X-Ray Computed; Diagnosis, Differential; Splenic Diseases; Incidental Findings; Spleen; Splenic Neoplasms; Splenectomy
PubMed: 37967822
DOI: 10.1055/a-2193-2292 -
Cancer Imaging : the Official... Aug 2010With the exception of lymphoma involving the spleen, other primary and secondary neoplasms are rare and infrequently encountered. Primary malignant neoplasms involving... (Review)
Review
With the exception of lymphoma involving the spleen, other primary and secondary neoplasms are rare and infrequently encountered. Primary malignant neoplasms involving the spleen are lymphoma and angiosarcoma. Primary benign neoplasms involving the spleen include hemangioma, lymphangioma, littoral cell angioma and splenic cyst and solid lesions such as hamartoma and inflammatory pseudotumor.
Topics: Cysts; Diagnostic Imaging; Granuloma, Plasma Cell; Hamartoma; Hemangioma; Hemangiosarcoma; Humans; Lymphangioma; Lymphoma; Neoplasms, Multiple Primary; Splenic Diseases; Splenic Neoplasms
PubMed: 20713317
DOI: 10.1102/1470-7330.2010.0026 -
Blood Apr 2003Splenic marginal zone lymphoma (SMZL) is a specific low-grade small B-cell lymphoma that is incorporated in the World Health Organization classification. Characteristic... (Review)
Review
Splenic marginal zone lymphoma (SMZL) is a specific low-grade small B-cell lymphoma that is incorporated in the World Health Organization classification. Characteristic features are splenomegaly, moderate lymphocytosis with villous morphology, intrasinusoidal pattern of involvement of various organs, especially bone marrow, and relative indolent course. Tumor progression with increase of blastic forms and aggressive behavior are observed in a minority of patients. Molecular and cytogenetic studies have shown heterogeneous results probably because of the lack of standardized diagnostic criteria. To date, no definitive therapy has been established. Therapeutic options include treatment abstention, splenectomy, splenic irradiation, and chemotherapy.
Topics: Animals; Cytogenetic Analysis; Diagnosis, Differential; Humans; Immunophenotyping; Lymphoma, B-Cell; Lymphoma, Non-Hodgkin; Splenic Neoplasms
PubMed: 12446449
DOI: 10.1182/blood-2002-07-2216 -
Medicine Jan 2022Primary splenic cancers represent a small number of cancer cases and studies on its clinicopathological features and outcomes are limited. Splenic lymphomas and primary...
Primary splenic cancers represent a small number of cancer cases and studies on its clinicopathological features and outcomes are limited. Splenic lymphomas and primary splenic angiosarcoma (PSA) are the 2 most common histological types of splenic cancers. This population-based study aimed to investigate the clinical characteristics and survival outcomes of patients with splenic lymphomas or PSA.Patients diagnosed with splenic lymphomas or PSA between 2000 and 2015 were identified from the Surveillance Epidemiology and End Results database of the National Cancer Institutes. Overall survival (OS) and cancer-specific survival (CSS) rates were calculated using the Kaplan-Meier method. A Cox proportional hazard models were used to identify independent predictors of cancer-specific mortality.A total of 700 patients with splenic lymphoma and 48 patients with PSA were included in this study. The median age of patients with splenic lymphoma was 65 years and 57 years for patients with PSA. For patients with splenic lymphoma, the most prevalent histological subtypes were splenic marginal zone lymphoma and diffuse large B-cell lymphoma. A total of 52.6% of the cases had stage IV disease based on the Ann Arbor staging system. Five-year OS and CSS were 76.9% and 83.4%, respectively. Multivariate analysis revealed that independent predictors of splenic lymphoma CSS included race, stage, chemotherapy, and histological subtype. However, a much shorter OS time was seen in the PSA cohort which had a 5-year OS of 11.8%, a median OS of 10.0 months and the 5-year CSS of 12.4%. Chemotherapy was correlated with better outcomes in patients with PSA. However, the survival benefits of surgery for splenic cancer were not statistically significant in our study.The current study is the largest cohort of primary splenic cancer presented in literature based on the Surveillance Epidemiology and End Results database and our large series describe the characteristics and survival outcomes of such rare diseases which may provide reliable information for further studies and clinicians.
Topics: Adult; Aged; Aged, 80 and over; Female; Hemangiosarcoma; Humans; Lymphoma, Large B-Cell, Diffuse; Male; Middle Aged; Neoplasm Staging; Prognosis; SEER Program; Splenic Neoplasms; Survival Rate
PubMed: 35060511
DOI: 10.1097/MD.0000000000028539 -
Blood Advances Jul 2023The genetic mechanisms associated with splenic marginal zone lymphoma (SMZL) transformation are not well defined. We studied 41 patients with SMZL that eventually...
The genetic mechanisms associated with splenic marginal zone lymphoma (SMZL) transformation are not well defined. We studied 41 patients with SMZL that eventually underwent large B-cell lymphoma transformation. Tumor material was obtained either only at diagnosis (9 patients), at diagnosis and transformation (18 patients), and only at transformation (14 patients). Samples were categorized in 2 groups: (1) at diagnosis (SMZL, n = 27 samples), and (2) at transformation (SMZL-T, n = 32 samples). Using copy number arrays and a next-generation sequencing custom panel, we identified that the main genomic alterations in SMZL-T involved TNFAIP3, KMT2D, TP53, ARID1A, KLF2, 1q gains, and losses of 9p21.3 (CDKN2A/B) and 7q31-q32. Compared with SMZL, SMZL-T had higher genomic complexity, and higher incidence of TNFAIP3 and TP53 alterations, 9p21.3 (CDKN2A/B) losses, and 6p gains. SMZL and SMZL-T clones arose by divergent evolution from a common altered precursor cell that acquired different genetic alterations in virtually all evaluable cases (92%, 12 of 13 cases). Using whole-genome sequencing of diagnostic and transformation samples in 1 patient, we observed that the SMZL-T sample carried more genomic aberrations than the diagnostic sample, identified a translocation t(14;19)(q32;q13) present in both samples, and detected a focal B2M deletion due to chromothripsis acquired at transformation. Survival analysis showed that KLF2 mutations, complex karyotype, and International Prognostic Index score at transformation were predictive of a shorter survival from transformation (P = .001; P = .042; and P = .007; respectively). In summary, SMZL-T are characterized by higher genomic complexity than SMZL, and characteristic genomic alterations that could represent key players in the transformation event.
Topics: Humans; Splenic Neoplasms; Mutation; Translocation, Genetic; Lymphoma, Large B-Cell, Diffuse; Leukemia, Lymphocytic, Chronic, B-Cell
PubMed: 36995085
DOI: 10.1182/bloodadvances.2022009415 -
Clinical Cancer Research : An Official... Oct 2022Regulatory T-cells (Treg) are essential to Tregs homeostasis and modulate the antitumor immune response in patients with lymphoma. However, the biology and prognostic...
PURPOSE
Regulatory T-cells (Treg) are essential to Tregs homeostasis and modulate the antitumor immune response in patients with lymphoma. However, the biology and prognostic impact of Tregs in splenic marginal zone lymphoma (SMZL) have not been studied.
EXPERIMENTAL DESIGN
Biopsy specimens from 24 patients with SMZL and 12 reactive spleens (rSP) from individuals without lymphoma were analyzed by using CITE-seq (cellular indexing of transcriptomes and epitopes by sequencing), CyTOF (mass cytometry) analysis, and flow cytometry to explore the phenotype, transcriptomic profile, and clinical significance of intratumoral Tregs and their subsets. The biological characteristics and cell signaling pathways of intratumoral Treg subsets were confirmed by in vitro functional assays.
RESULTS
We found that Tregs are more abundant in SMZL patients' spleens than rSP, and Tregs from patients with SMZL and rSP can be separated into CD161+Treg and CD26+Treg subsets. CD161+Tregs are increased in SMZL but have dysregulated immune function. We found that CD161+Treg and CD26+Tregs have unique gene expression and phenotypic profiles and are differentially correlated with patient outcomes. Specifically, increased CD161+Tregs are significantly associated with a favorable prognosis in patients with SMZL, whereas CD26+Tregs are associated with a poor prognosis. Furthermore, activation of the IL2/STAT5 pathway contributes to the induction of CD26+Tregs and can be reversed by STAT5 inhibition.
CONCLUSIONS
IL2/STAT5-mediated expansion of CD26+Tregs contributes to a poor clinical outcome in SMZL and may represent a therapeutic opportunity in this disease.
Topics: Dipeptidyl Peptidase 4; Epitopes; Humans; Interleukin-2; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphoma; Lymphoma, B-Cell, Marginal Zone; Phenotype; STAT5 Transcription Factor; Splenic Neoplasms
PubMed: 35686915
DOI: 10.1158/1078-0432.CCR-22-0977 -
Current Oncology (Toronto, Ont.) Dec 2021Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare disease, representing <1% of all non-Hodgkin lymphomas (NHL). The most common clinical manifestations... (Review)
Review
Splenic diffuse red pulp small B-cell lymphoma (SDRPL) is a rare disease, representing <1% of all non-Hodgkin lymphomas (NHL). The most common clinical manifestations include splenomegaly, lymphocytosis, and hemocytopenia. A diagnosis of SDRPL can be challenging, as it shares multiple clinical and laboratory features with splenic marginal zone lymphoma (SMZL), hairy cell leukemia (HCL), and HCL variant (HCL-v). Obtaining splenic tissue remains the gold standard for diagnosis. In the cases where splenic tissue is not available, diagnosis can be established by a review of peripheral blood and bone marrow studies. SDRPL is characterized by a diffuse involvement of the splenic red pulp by monomorphous small-to-medium sized mature B lymphocytes effacing the white pulp. The characteristic immunophenotype is positive for CD20, DBA.44 (20 to 90%), and IgG, and typically negative for CD5, CD10, CD23, cyclin D1, CD43, annexin A1, CD11c, CD25, CD123, and CD138. The Ki-67 proliferative index is characteristically low. Cyclin D3 is expressed in the majority of SDRPL in contrast with other types of small B-cell lymphomas, thus facilitating the recognition of this disease. There is no standard treatment regimen for SDRPL. Initial treatment options include splenectomy, rituximab monotherapy, or a combination of both. Chemoimmunotherapy should be considered in patients with advanced disease at baseline or progression.
Topics: Humans; Immunophenotyping; Leukemia, Hairy Cell; Leukemia, Lymphocytic, Chronic, B-Cell; Lymphoma, B-Cell; Splenic Neoplasms
PubMed: 34940070
DOI: 10.3390/curroncol28060431