RESUMEN
Burkitt lymphoma is a non-Hodgkin B-cell lymphoma with a high prevalence in the pediatric population. Abdominal manifestations are well known in sporadic Burkitt lymphoma and vary from nonspecific symptoms to intestinal obstruction due to intussusception; however, mass-like splenic involvement has been scarcely described. OBJECTIVE: To present a case of a patient with a splenic mass whose histopathological analysis revealed Burkitt lymphoma. CLINICAL CASE: A 13-year-old female patient presented with abdominal pain, progressive weight loss, and fever. Imaging studies showed a splenic mass, intestinal thickening, and ileal intussusception. Histopathological analysis of spleen biopsy revealed Burkitt lymphoma. After the first cycle of chemotherapy (BFM95-NHL protocol), abdominal symptoms resolved; no other signs suggestive of intussusception were observed, as well as a significant reduction of the splenic mass was observed. CONCLUSIONS: Burkitt lymphoma in pediatric patients can present as a well-defined splenic tumor, causing no splenomegaly. In addition, its management does not require surgery since it can be resolved with chemotherapy.
Asunto(s)
Linfoma de Burkitt , Neoplasias del Bazo , Humanos , Linfoma de Burkitt/diagnóstico , Linfoma de Burkitt/patología , Linfoma de Burkitt/complicaciones , Linfoma de Burkitt/tratamiento farmacológico , Femenino , Adolescente , Neoplasias del Bazo/patología , Neoplasias del Bazo/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapéutico , Intususcepción/diagnóstico , Intususcepción/etiología , Dolor Abdominal/etiologíaRESUMEN
OBJECTIVE: To explore the genetic basis and pathogenesis for a child with type I Hereditary hemorrhagic telangiectasia (HHTâ ) and Splenic sinus shore cell hemangioma (LCA). METHODS: A child with HHT complicated with LCA diagnosed at the First Affiliated Hospital of Dali University in April 2022 was selected as the study subject. Clinical data of the child and her relatives were collected, and pathogenic variants were screened by whole exome sequencing. Candidate variant was verified by Sanger sequencing and bioinformatic analysis. RESULTS: The patient, a 16-year-old female, had recurrent epitaxis since childhood, which sometimes necessitated hemostasis treatment. She also had splenectomy due to splenic rupture and was diagnosed with LCA. Her father and grandmother also had a history of recurrent epitaxis. Her father had deceased due to cerebral vascular rupture. The child was found to harbor a c.360+1G>A variant in the ENG gene. The same variant was not found in her asymptomatic mother and brother. CONCLUSION: The c.360+1G>A variant of the ENG gene probably underlay the pathogenesis in this child.
Asunto(s)
Hemangioma , Telangiectasia Hemorrágica Hereditaria , Humanos , Femenino , Adolescente , Telangiectasia Hemorrágica Hereditaria/genética , Telangiectasia Hemorrágica Hereditaria/complicaciones , Hemangioma/genética , Linaje , Neoplasias del Bazo/genética , Neoplasias del Bazo/complicaciones , Masculino , Pruebas Genéticas , Secuenciación del ExomaRESUMEN
Bartonella species are gram-negative coccobacilli that are globally recognized as significant pathogens causing zoonotic infections. Among Bartonella species, B. henselae, B. quintana, and B. bacilliformis are prominent pathogens causing infections in humans, often manifesting as infective endocarditis. Bartonella endocarditis can pose diagnostic challenges due to its indolent presentation and limitations of standard microbiological culture techniques to identify the organism. We report a case of a 23-year-old male, who initially presented with the manifestations of hepatosplenic T-cell lymphoma, later diagnosed with blood culture-negative endocarditis caused by B. henselae. The patient had a complicated clinical course including pancytopenia, hepatosplenomegaly, and severe aortic valve regurgitation. Despite negative blood cultures, diagnostic clues included persistent fevers and bicuspid aortic valve with abscess. High Bartonella IgG titers (>1:800) supported the diagnosis. This case underscores the importance of considering Bartonella species in patients with suspected endocarditis, particularly in those with predisposing factors and negative blood cultures. Diagnosis relies heavily on serologic assays due to low sensitivity of conventional culture methods. Treatment involves a multidisciplinary approach with antibiotics and surgical intervention for optimal outcomes. Timely recognition and management are crucial to mitigate the high mortality associated with Bartonella endocarditis, and we hope this article offers insight for clinicians.
Asunto(s)
Bartonella henselae , Endocarditis Bacteriana , Linfoma de Células T , Humanos , Masculino , Bartonella henselae/aislamiento & purificación , Endocarditis Bacteriana/microbiología , Endocarditis Bacteriana/diagnóstico , Endocarditis Bacteriana/tratamiento farmacológico , Linfoma de Células T/complicaciones , Linfoma de Células T/diagnóstico , Adulto Joven , Neoplasias del Bazo/complicaciones , Enfermedad por Rasguño de Gato/diagnóstico , Enfermedad por Rasguño de Gato/complicaciones , Enfermedad por Rasguño de Gato/tratamiento farmacológico , Neoplasias HepáticasRESUMEN
Hemangiosarcoma is an aggressive tumour that most frequently occurs in larger, middle-aged dogs of certain breeds. The spleen is the most commonly affected organ. The aim of this prospective therapy study was to evaluate the clinical effect of autologous, monocyte-derived dendritic cell (DC) therapy in canine hemangiosarcoma stage II after splenectomy. Dogs (n=452) diagnosed with splenic hemangiosarcoma that underwent splenectomy were enrolled. Of these, 42 dogs with stage II entered the DC therapy study. The median survival time for the total group of 42 dogs was 203 days. The median survival for the group (n=34) that received the full DC therapy (≥3 vaccines) was 256 days, with a 29â¯% one-year survival rate and a hazard ratio of 0.30, adjusted to age and bodyweight (P=0.010). We further observed a significant increase in DC yield after each application and demonstrated that DC yield at the beginning of treatment is significantly related to patient survival. While further evidence is needed, we conclude that autologous, monocyte-derived DC therapy is a viable alternative to standard treatment methods of canine splenic stage II hemangiosarcoma.
Asunto(s)
Células Dendríticas , Enfermedades de los Perros , Hemangiosarcoma , Neoplasias del Bazo , Animales , Perros , Hemangiosarcoma/veterinaria , Hemangiosarcoma/terapia , Enfermedades de los Perros/terapia , Neoplasias del Bazo/veterinaria , Neoplasias del Bazo/terapia , Femenino , Masculino , Estudios Prospectivos , Esplenectomía/veterinaria , Trasplante Autólogo/veterinariaAsunto(s)
Hemangioma , Microfilarias , Neoplasias del Bazo , Humanos , Hemangioma/patología , Hemangioma/diagnóstico por imagen , Neoplasias del Bazo/patología , Neoplasias del Bazo/diagnóstico , Microfilarias/aislamiento & purificación , Animales , Bazo/patología , Bazo/diagnóstico por imagen , Bazo/parasitología , Microscopía , Masculino , Histocitoquímica , Filariasis/diagnóstico , Filariasis/parasitologíaRESUMEN
BACKGROUND: Extraskeletal osteosarcoma is an extremely rare malignancy that accounts for 1% of soft tissue sarcoma and 4.3% of all osteosarcoma. Extraskeletal osteosarcoma can develop in a patient between the ages of 48 and 60 years. The incidence of extraskeletal osteosarcoma is slightly higher in male patients than in females. CASE PRESENTATION: A 50-year-old Caucasian male patient presented with a 6-month history of intermittent lower-left back pain that limits his activity. Prior ultrasonography and abdominal computed tomography scan showed a diagnosis of kidney stone and tumor in the lower-left abdomen. The computed tomography urography with contrast revealed a mass suspected as a left retroperitoneal malignant tumor. Hence, the tumor was resected through laparotomy and the patient continued with histopathological and immunohistochemistry examination with the result of extraskeletal osteosarcoma. CONCLUSION: Extraskeletal osteosarcoma presents diagnostic challenges requiring multimodal examination, including histological and immunohistochemistry analyses. This case underscores the aggressive nature and poor prognosis despite undergoing the current suggested treatment.
Asunto(s)
Osteosarcoma , Tomografía Computarizada por Rayos X , Humanos , Masculino , Persona de Mediana Edad , Osteosarcoma/patología , Osteosarcoma/diagnóstico , Osteosarcoma/diagnóstico por imagen , Neoplasias Renales/patología , Neoplasias Renales/diagnóstico por imagen , Neoplasias Renales/cirugía , Neoplasias Renales/diagnóstico , Neoplasias Pancreáticas/patología , Neoplasias Pancreáticas/diagnóstico , Neoplasias Pancreáticas/diagnóstico por imagen , Neoplasias Pancreáticas/cirugía , Neoplasias Gástricas/patología , Neoplasias Gástricas/diagnóstico , Neoplasias Gástricas/diagnóstico por imagen , Neoplasias Gástricas/cirugía , Neoplasias del Bazo/patología , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/diagnóstico , Neoplasias del Bazo/diagnóstico por imagen , Neoplasias Retroperitoneales/patología , Neoplasias Retroperitoneales/diagnóstico por imagen , Neoplasias Retroperitoneales/diagnóstico , Neoplasias Retroperitoneales/cirugíaRESUMEN
Biological hallmarks of splenic marginal zone lymphoma (SMZL) remain poorly described. Herein, we performed in-depth SMZL characterization through multimodal single-cell analyses of paired blood/spleen samples. The 3'-single-cell RNA-sequencing, Cellular Indexing of Transcriptomes and Epitopes by sequencing, and 5'-V(D)J single-cell RNA-sequencing datasets were integrated to characterize SMZL transcriptome profiles, including B-cell receptor and T-cell receptor repertoires. Hyperexpanded B-cell clones in the spleen were at a memory-like stage, whereas recirculating tumor B-cells in blood encompassed multiple differentiation stages, indicating an unexpected desynchronization of the B-cell maturation program in SMZL cells. Spatial transcriptomics showed the enrichment of T-effector and T-follicular helper (TFH) signatures in the nodular subtype of SMZL. This latter also exhibited gene-based cell-cell interactions suggestive of dynamic crosstalk between TFH and cancer cells in transcriptomics, further substantiated by using imaging mass cytometry. Our findings provide a comprehensive high-resolution description of SMZL biological hallmarks and characterize, for the first time in situ, inter- and intra-patient heterogeneity at both transcriptomic and protein levels. © 2024 The Authors. The Journal of Pathology published by John Wiley & Sons Ltd on behalf of The Pathological Society of Great Britain and Ireland.
Asunto(s)
Linfoma de Células B de la Zona Marginal , Análisis de la Célula Individual , Neoplasias del Bazo , Transcriptoma , Humanos , Neoplasias del Bazo/genética , Neoplasias del Bazo/patología , Neoplasias del Bazo/metabolismo , Linfoma de Células B de la Zona Marginal/genética , Linfoma de Células B de la Zona Marginal/patología , Linfoma de Células B de la Zona Marginal/metabolismo , Linfoma de Células B de la Zona Marginal/inmunología , Perfilación de la Expresión Génica/métodos , Masculino , Femenino , Persona de Mediana Edad , Linfocitos B/patología , Linfocitos B/metabolismo , Anciano , Bazo/patología , Bazo/inmunología , Bazo/metabolismoRESUMEN
OBJECTIVES: Splenic lesions might exhibit overlapping imaging features, varying from benign entities like cysts and hemangiomas to malignancies such as lymphoma and angiosarcoma. This meta-analysis aims to delineate imaging characteristics that distinguish malignant from benign splenic lesions. METHODS: Adhering to PRISMA guidelines, we searched PubMed, Scopus, and Web of Science for studies on imaging features differentiating malignant from benign splenic lesions. We extracted data on splenic pathology and imaging characteristics and assessed the methodological quality via QUADAS-2. Odds ratio meta-analyses were performed using STATA (Version 17.0, Stata Corp, College Station, TX). RESULTS: Portal phase hypoenhancement, hypovascular enhancement pattern, diffusion restriction, and late phase hypoenhancement, with odds ratios above 10, highly indicate malignancy. Other features suggestive of malignancy include solid morphology, lymphadenopathy, presence of perisplenic fluid, arterial hypoenhancement, hypoechogenicity on ultrasound, splenomegaly, and presence of multiple lesions. In contrast, cystic morphology, hypervascular-washout and hypervascular-persistent pattern of enhancement, late phase hyperenhancement, anechogenicity on ultrasound, portal phase hyperenhancement, well-defined borders, and calcification are in favour of benign pathology. CONCLUSION: The study underscores the critical role of contrast-enhanced and diffusion-weighted imaging in distinguishing malignant from benign splenic lesions, emphasizing the role of features like portal phase hypoenhancement and restricted diffusion in diagnosing malignancies. Additionally, the study emphasizes the value of contrast-enhanced ultrasound, which allows for the visualization of key contrast-enhancement patterns without the risk of ionizing radiation exposure.
Asunto(s)
Neoplasias del Bazo , Humanos , Diagnóstico Diferencial , Neoplasias del Bazo/diagnóstico por imagen , Neoplasias del Bazo/patología , Enfermedades del Bazo/diagnóstico por imagen , Bazo/diagnóstico por imagen , Medios de ContrasteRESUMEN
BAP1-Tumor Predisposition Syndrome (TPDS) is caused by germline variants in BAP1 and predisposes to solid tumors. After observation of a radiologically malignant-appearing splenic mass with benign pathology in a patient with BAP1-TPDS, we sought to retrospectively characterize splenic lesions in individuals with BAP1-TPDS seen at a comprehensive cancer center. A dedicated radiology review for splenic abnormalities was performed. We identified 37 individuals with BAP1-TPDS, 81% with a history of cancer. Of 33 individuals with abdominal imaging, 10 (30%) had splenic lesions, and none were shown to be malignant on follow-up. Splenectomy in an individual with suspected splenic angiosarcoma showed a benign vascular neoplasm with loss of nuclear staining for BAP1 in a subset of cells. Benign splenic lesions appear to be common and potentially BAP1-driven in individuals with BAP1-TPDS; confirmation of these findings could lead to more conservative management and avoidance of splenectomy.
Asunto(s)
Proteínas Supresoras de Tumor , Ubiquitina Tiolesterasa , Humanos , Ubiquitina Tiolesterasa/genética , Ubiquitina Tiolesterasa/metabolismo , Proteínas Supresoras de Tumor/genética , Proteínas Supresoras de Tumor/metabolismo , Femenino , Masculino , Adulto , Persona de Mediana Edad , Anciano , Neoplasias del Bazo/genética , Neoplasias del Bazo/patología , Neoplasias del Bazo/metabolismo , Mutación de Línea Germinal , Predisposición Genética a la Enfermedad , Síndromes Neoplásicos Hereditarios/genética , Síndromes Neoplásicos Hereditarios/patología , Síndromes Neoplásicos Hereditarios/metabolismo , EsplenectomíaRESUMEN
RATIONALE: Sclerosing angiomatoid nodular transformation (SANT) of the spleen is an uncommon benign vascular lesion with an obscure etiology. It predominantly affects middle-aged women and presents with nonspecific clinical signs, making preoperative diagnosis challenging. The definitive diagnosis of SANT relies on pathological examination following splenectomy. This study aims to contribute to the understanding of SANT by presenting a case series and reviewing the literature to highlight the clinical presentation, diagnostic challenges, and treatment outcomes. PATIENT CONCERNS: In this retrospective study, we analyzed the clinical data of 3 patients with confirmed SANT admitted from November 2013 to October 2023. The cases include a 25-year-old male, a 15-year-old female, and a 39-year-old male, each with a splenic mass. DIAGNOSES AND INTERVENTIONS: All of the three cases were treated by laparoscopic splenectomy (LS). Pathological examination confirmed SANT in all cases. OUTCOMES: No recurrence or metastasis was observed during a 10-year follow-up for the first 2 cases, and the third case showed no abnormalities at 2 months postoperatively. Despite its rarity, SANT is a significant condition due to its potential for misdiagnosis and the importance of distinguishing it from malignant lesions. The study underscores the utility of LS as a safe and effective treatment option. LESSONS: SANT is a rare benign tumor of the spleen, and the preoperative diagnosis of whom is challenging. LS is a safe and effective treatment for SANT, with satisfactory surgical outcomes and favorable long-term prognosis on follow-up. The study contributes to the limited body of research on this rare condition and calls for larger studies to validate these findings and improve clinical management.
Asunto(s)
Esplenectomía , Neoplasias del Bazo , Humanos , Masculino , Adulto , Femenino , Esplenectomía/métodos , Adolescente , Neoplasias del Bazo/patología , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/diagnóstico , Bazo/patología , Histiocitoma Fibroso Benigno/patología , Histiocitoma Fibroso Benigno/cirugía , Histiocitoma Fibroso Benigno/diagnóstico , Estudios Retrospectivos , Laparoscopía/métodos , Diagnóstico Diferencial , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/patología , Enfermedades del Bazo/diagnósticoRESUMEN
Dogs that had splenectomy are predisposed to fatal thrombotic conditions, and thrombocytosis is a risk factor for post-splenectomy hypercoagulability. However, in veterinary medicine, there are no specific therapeutic approaches for managing this hypercoagulability. This study aimed to determine the preventive effect of clopidogrel on post-operative hypercoagulability during the first 2 weeks post-splenectomy in dogs with splenic masses. This study included 12 dogs that had splenectomy. Seven dogs received no treatment (group A), and five were treated with clopidogrel (group B). Clopidogrel was loaded at 10 mg/kg on day 2 and continued at 2 mg/kg until day 14. Blood samples were collected on the day of surgery and 2, 7, and 14 days after splenectomy in both groups. In group B, thromboelastography (TEG) was performed on the same days. In group A, there was significant elevation of platelet counts on days 7 (p = 0.007) and 14 (p = 0.001) compared to day 0. In group B, the platelet counts were significantly elevated on day 7 (p = 0.032) but no significant difference was found on day 14 compared to day 0. Platelet counts on day 14 were significantly higher in group A than in group B (p = 0.03). The lower platelet counts were correlated with alterations in TEG parameters, and no significant differences were found in the K and α-angle values at all postoperative assessment points compared to day 0. Our study suggests that clopidogrel may reduce post-operative thrombocytosis and hypercoagulability in dogs that undergo splenectomy for splenic masses.
Asunto(s)
Clopidogrel , Enfermedades de los Perros , Inhibidores de Agregación Plaquetaria , Esplenectomía , Tromboelastografía , Trombofilia , Animales , Perros , Esplenectomía/veterinaria , Esplenectomía/efectos adversos , Clopidogrel/uso terapéutico , Enfermedades de los Perros/sangre , Enfermedades de los Perros/cirugía , Enfermedades de los Perros/tratamiento farmacológico , Recuento de Plaquetas/veterinaria , Femenino , Masculino , Trombofilia/veterinaria , Trombofilia/tratamiento farmacológico , Inhibidores de Agregación Plaquetaria/uso terapéutico , Inhibidores de Agregación Plaquetaria/farmacología , Tromboelastografía/veterinaria , Complicaciones Posoperatorias/veterinaria , Complicaciones Posoperatorias/prevención & control , Neoplasias del Bazo/veterinaria , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/sangre , Enfermedades del Bazo/veterinaria , Enfermedades del Bazo/cirugía , Enfermedades del Bazo/sangre , Trombocitosis/veterinariaRESUMEN
BACKGROUND: Dogs with retroperitoneal hemangiosarcoma (HSA) exhibit variable postoperative median survival times (MST). OBJECTIVE: To retrospectively evaluate the prognostic value of selected tumour-related factors, such as tumour size, rupture, invasion into adjacent tissue, involvement of lymph node and distant metastasis, they were analysed in dogs with retroperitoneal HSA. METHODS: Ten dogs with retroperitoneal HSA managed solely with surgical excision were reviewed and compared with spleen (71) and liver (9) HSA. The Kaplan-Meier method and log-rank analysis were used compare MSTs between factors. Multivariable Cox proportional-hazard analysis was used to compare differences between arising sites. RESULTS: Retroperitoneal HSA showed comparatively longer postoperative MST compared with that of spleen and liver HSA and demonstrated significantly longer MST (p = 0.003) for tumours ≥5 cm (195 days) than <5 cm (70 days). Spleen HSA revealed significantly shorter MSTs in involvement of distant lymph nodes (23 days) and distant metastasis (39 days) than those in negative (83 days, p = 0.002 and 110 days, p < 0.001, respectively). Liver HSA also revealed significantly shorter MST (16.5 days compared with 98 days, p = 0.003) for distant metastasis. Additionally, hazard ratios (HRs) and their forest plot for overall HSA revealed as poor prognostic factors, arising sites (spleen; HR 2.78, p = 0.016 and liver; HR 3.62, p = 0.019), involvement of distant lymph nodes (HR 2.43, p = 0.014), and distant metastasis (HR 2.86, p < 0.001), and as better prognostic factor of tumour size ≥5 cm (HR 0.53, p = 0.037). CONCLUSION: In combination with overall HSA, retroperitoneal HSA shows comparatively longer postoperative MST compared to spleen and liver HSA, associated with tumour size ≥5 cm suggesting better prognostic factor.
Asunto(s)
Enfermedades de los Perros , Hemangiosarcoma , Neoplasias Retroperitoneales , Animales , Perros , Hemangiosarcoma/veterinaria , Hemangiosarcoma/patología , Hemangiosarcoma/cirugía , Hemangiosarcoma/mortalidad , Estudios Retrospectivos , Enfermedades de los Perros/patología , Enfermedades de los Perros/cirugía , Enfermedades de los Perros/mortalidad , Masculino , Femenino , Neoplasias Retroperitoneales/veterinaria , Neoplasias Retroperitoneales/patología , Neoplasias Retroperitoneales/cirugía , Neoplasias Retroperitoneales/mortalidad , Pronóstico , Neoplasias del Bazo/veterinaria , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/patología , Neoplasias del Bazo/mortalidad , Neoplasias Hepáticas/veterinaria , Neoplasias Hepáticas/mortalidad , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/patologíaRESUMEN
BACKGROUND: Spleen Epstein-Barr Virus (EBV)-positive inflammatory follicular dendritic cell sarcoma (FDCS) is rare, and the imaging signs are unclear. The COVID-19 has been confirmed to be the cause of pneumonia and can cause a variety of diseases including myocarditis. However, it has not been reported to be the cause of the exacerbation or activation of EBV-positive inflammatory FDCS. OBJECTIVE: The objective is to extract the imaging features of EBV-positive inflammatory FDCS in the spleen and analyze the reasons for the special features of this case. METHODS: By analyzing the patient's treatment process and imaging examinations (A 77-year-old female was admitted to the hospital due to generalized discomfort and pain symptoms. When she was admitted to the hospital a year earlier with COVID-19 pneumonia, a chest CT scan showed that she had a splenic tumor. During this admission, CT scans showed two irregularly shaped and unevenly dense soft tissue density masses within the spleen, with uneven enhancement on contrast-enhanced im-aging within the solid components and along the edges. PET/CT scans revealed elevated glucose metabolism in the masses. Postoperative pathological diagnosis confirmed splenic EBV-positive inflammatory FDCS.), reading the literature, sorting out the disease cognitive process, epidemiology, and pathological data of EBV-positive inflammatory FDCS, we discussed the imaging manifestations and possible differential diagnosis of the disease. RESULTS: The patient was finally diagnosed with splenic EBV-positive inflammatory FDCS. CONCLUSIONS: Imaging features of EBV-positive inflammatory FDCS in the spleen include a high incidence of hemorrhage and necrosis, persistent moderate enhancement of the solid portion, a "capsular-like enhancement" structure at the tumor edge, and possibly active glucose metabolism with high Standardized Uptake Values (SUVs). COVID-19 infection and long-term COVID-19 sequelae may exacerbate and activate EBV-positive inflammatory FDCS in the spleen, and the mechanism remains to be further studied.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Infecciones por Virus de Epstein-Barr , Tomografía Computarizada por Tomografía de Emisión de Positrones , Humanos , Femenino , Anciano , Sarcoma de Células Dendríticas Foliculares/patología , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Sarcoma de Células Dendríticas Foliculares/virología , Infecciones por Virus de Epstein-Barr/diagnóstico , Infecciones por Virus de Epstein-Barr/complicaciones , Tomografía Computarizada por Tomografía de Emisión de Positrones/métodos , COVID-19/complicaciones , Neoplasias del Bazo/patología , Neoplasias del Bazo/diagnóstico por imagen , Neoplasias del Bazo/diagnóstico , Neoplasias del Bazo/virología , Bazo/patología , Bazo/diagnóstico por imagen , Herpesvirus Humano 4/aislamiento & purificación , Tomografía Computarizada por Rayos X , SARS-CoV-2RESUMEN
OBJECTIVE: The present study aims to explore the clinicopathological characteristics of Epstein-Barr virus (EBV)-positive inflammatory follicular dendritic cell sarcoma (IFDCS; EBV+ IFDCS). CASE REPORT: The case involved a 32-year-old woman who underwent surgical resection of a splenic nodule. Histological examination and immunohistochemistry were performed using cluster of differentiation (CD) markers, and in-situ hybridization was conducted to detect EBV-encoded RNA (EBER). RESULTS: A microscopic analysis revealed neoplastic cells with various morphologies, including round, ovoid, or spindled shapes, dispersed within a prominent lymphoplasmacytic infiltrate. The tumor cells exhibited nuclear atypia, with some resembling Reed-Sternberg cells. The immunohistochemistry demonstrated focal positivity for follicular dendritic cell markers, such as CD21, CD23 and CD35, and focal negativity for other markers, including CD3, CD34, CD20, CD79a, myeloperoxidase and HMB45. Additionally, the EBER staining showed strongly positive results. The patient showed no local recurrence or metastasis during the 13-month follow-up. CONCLUSION: A comprehensive understanding of EBV+IFDCS, including its clinicopathological features and immunohistochemical characteristics, is crucial for accurate diagnosis and differential diagnosis of this rare tumor.
Asunto(s)
Sarcoma de Células Dendríticas Foliculares , Infecciones por Virus de Epstein-Barr , Herpesvirus Humano 4 , Humanos , Femenino , Sarcoma de Células Dendríticas Foliculares/patología , Sarcoma de Células Dendríticas Foliculares/virología , Sarcoma de Células Dendríticas Foliculares/diagnóstico , Adulto , Herpesvirus Humano 4/aislamiento & purificación , Infecciones por Virus de Epstein-Barr/complicaciones , Infecciones por Virus de Epstein-Barr/patología , Infecciones por Virus de Epstein-Barr/virología , Infecciones por Virus de Epstein-Barr/diagnóstico , Neoplasias del Bazo/patología , Neoplasias del Bazo/virología , Neoplasias del Bazo/diagnóstico , Inmunohistoquímica , Inflamación/patología , Inflamación/virologíaRESUMEN
While the importance of conversion surgery has increased with the development of systemic chemotherapy for gastric cancer (GC), reports of conversion surgery for patients with GC with distant metastasis and tumor thrombus are extremely scarce, and a definitive surgical strategy has yet to be established. Herein, we report a 67-year-old man with left abdominal pain referred to our hospital following a diagnosis of unresectable GC. Esophagogastroduodenoscopy and contrast-enhanced abdominal computed tomography (CT) revealed advanced GC with splenic metastasis. A splenic vein tumor thrombus (SVTT) and a continuous thrombus to the main trunk of the portal vein were detected. The patient was treated with anticoagulation therapy and systemic chemotherapy comprising S-1 and oxaliplatin. One year following chemotherapy initiation, a CT scan revealed progressive disease (PD); therefore, the chemotherapy regimen was switched to ramucirumab with paclitaxel. After 10 courses of chemotherapy resulting in primary tumor and SVTT shrinkage, the patient underwent laparoscopic total gastrectomy (LTG) and distal pancreaticosplenectomy (DPS). He was discharged without complications and remained alive 6 months postoperatively without recurrence. In summary, the wait-and-see approach was effective in a patient with GC with splenic metastasis and SVTT, ultimately leading to an R0 resection performed via LTG and DPS.
Asunto(s)
Neoplasias del Bazo , Vena Esplénica , Neoplasias Gástricas , Humanos , Neoplasias Gástricas/cirugía , Neoplasias Gástricas/tratamiento farmacológico , Neoplasias Gástricas/complicaciones , Masculino , Anciano , Vena Esplénica/cirugía , Neoplasias del Bazo/secundario , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/tratamiento farmacológico , Procedimientos Quirúrgicos Mínimamente Invasivos/métodos , Trombosis de la Vena/cirugía , Trombosis de la Vena/tratamiento farmacológico , Gastrectomía/métodosRESUMEN
A patient in his 40s with splenic angiosarcoma metastatic to the liver underwent splenectomy, chemotherapy, and partial hepatectomy before being treated on a clinical trial with CTLA4 and PD1 inhibitors. He had received pneumococcal and meningococcal vaccines post-splenectomy. On week 10, he developed grade 3 immune-related colitis, successfully treated with the anti-tumor necrosis factor-alpha inhibitor infliximab and steroids. After 4 cycles of treatment, scans showed partial response. He resumed anti-PD1 therapy, and 6 hours after the second dose of anti-PD1 he presented to the emergency room with hematemesis, hematochezia, hypotension, fever, and oxygen desaturation. Laboratory tests demonstrated acute renal failure and septicemia (Streptococcus pneumoniae). He died 12 hours after the anti-PD1 infusion from overwhelming post-splenectomy infection (OPSI). Autopsy demonstrated non-viable liver tumors among other findings. In conclusion, patients undergoing immunotherapy and with prior history of asplenia should be monitored closely for OPSI as they may be at increased risk.
Asunto(s)
Hemangiosarcoma , Neoplasias Hepáticas , Esplenectomía , Neoplasias del Bazo , Humanos , Esplenectomía/efectos adversos , Masculino , Hemangiosarcoma/terapia , Neoplasias del Bazo/secundario , Neoplasias del Bazo/terapia , Resultado Fatal , Neoplasias Hepáticas/secundario , Neoplasias Hepáticas/terapia , Inhibidores de Puntos de Control Inmunológico/efectos adversos , Inhibidores de Puntos de Control Inmunológico/uso terapéutico , Inmunoterapia/efectos adversos , Inmunoterapia/métodos , Adulto , Infecciones Neumocócicas/etiología , Receptor de Muerte Celular Programada 1/antagonistas & inhibidores , Antígeno CTLA-4/antagonistas & inhibidoresRESUMEN
A 12-year-old spayed female Dalmatian presented with acute vomiting and anorexia. The clinicopathological and imaging abnormalities included icterus, biliary obstruction, and multiple diffuse splenic hypoechogenic nodules. Cholecystectomy was performed to remove the obstruction, followed by liver biopsy and splenectomy. Histopathological and immunohistology evaluation of the spleen, liver, and gallbladder revealed splenic marginal zone lymphoma (MZL) with gallbladder and hepatic infiltration of neoplastic CD20/CD79α-positive cells. Moreover, we observed clonal rearrangements of the immunoglobulin heavy-chain (IgH) gene in all three tissues. The dog was in good condition without chemotherapy. However, there was progressive elevation of liver enzymes, which could be attributed to neoplastic hepatic infiltration. Chlorambucil and prednisolone were administered until day 108, when the liver enzyme levels normalized. On day 156, the dog developed diffuse large B-cell lymphoma (DLBCL) of the peripheral lymph nodes. Sequence analysis of the clonally rearranged IgH gene revealed that all neoplastic cells in the spleen, gallbladder, and liver at initial presentation, as well as lymph nodes on day 156, possessed the same sequence identity of the amplified IgH fragments. This demonstrated that all neoplastic cells were derived from the same B-lymphocyte clone. The DLBCL was considered to have transformed from the splenic MZL, with gallbladder involvement. In cases of splenic MZL, it is important to consider gallbladder involvement and transformation to DLBCL. Moreover, gallbladder lymphoma should be included in the differential diagnosis of dogs with gallbladder abnormalities. Further studies are warranted to investigate the prognosis of splenic MZL.
Asunto(s)
Enfermedades de los Perros , Linfoma de Células B de la Zona Marginal , Linfoma de Células B Grandes Difuso , Neoplasias del Bazo , Animales , Perros , Femenino , Enfermedades de los Perros/patología , Enfermedades de los Perros/diagnóstico , Neoplasias del Bazo/veterinaria , Neoplasias del Bazo/patología , Linfoma de Células B de la Zona Marginal/veterinaria , Linfoma de Células B de la Zona Marginal/patología , Linfoma de Células B Grandes Difuso/veterinaria , Linfoma de Células B Grandes Difuso/patología , Linfoma de Células B Grandes Difuso/diagnóstico , Neoplasias de la Vesícula Biliar/veterinaria , Neoplasias de la Vesícula Biliar/patología , Vesícula Biliar/patologíaAsunto(s)
Hemangiosarcoma , Neoplasias Hepáticas , Neoplasias del Bazo , Humanos , Hemangiosarcoma/cirugía , Hemangiosarcoma/patología , Neoplasias Hepáticas/cirugía , Neoplasias Hepáticas/patología , Neoplasias del Bazo/cirugía , Neoplasias del Bazo/patología , Procedimientos Quirúrgicos Robotizados/métodos , Hepatectomía/métodos , Masculino , Femenino , Persona de Mediana Edad , Resultado del Tratamiento , Esplenectomía/métodosRESUMEN
OBJECTIVE: To assess the predictability of the hemangiosarcoma likelihood prediction (HeLP) score and the Tufts Splenic Tumor Assessment Tool (T-STAT) for hemangiosarcoma and malignancy, respectively. ANIMALS: 261 dogs undergoing splenectomy for a splenic mass. METHODS: Medical records were retrospectively reviewed; variables for the HeLP score and T-STAT were collected, and scores were assigned. Area under the curve (AUC) was calculated for each score. RESULTS: The HeLP score included 141 dogs; hemangiosarcoma was diagnosed in 87 (61.7%) dogs. The median cumulative HeLP score was 51 (range, 17 to 82; IQR, 39 to 58) for dogs with hemangiosarcoma and 28 (range, 0 to 70; IQR, 17 to 41) for dogs without hemangiosarcoma. The categorical HeLP score was low (28; 32.2%), medium (31; 35.6%), and high (28; 32.2%) for dogs with hemangiosarcoma and was low (41; 75.9%), medium (9; 16.7%), and high (4; 7.4%) for dogs without hemangiosarcoma. The AUC of the cumulative and categorical HeLP scores for diagnosis of hemangiosarcoma were 0.79 (95% CI, 0.71 to 0.86) and 0.73 (95% CI, 0.65 to 0.82), respectively. The T-STAT included 181 dogs. Lesions were benign in 95 (52.5%) and malignant in 86 (47.5%) dogs. The median T-STAT score was 62% (range, 5% to 98%; IQR, 36% to 77%) for dogs with malignant lesions and 38% (range, 5% to 91%; IQR, 24% to 59%) for dogs with benign lesions. The T-STAT had an AUC of 0.68 (0.60 to 0.76) for diagnosis of malignancy. CLINICAL RELEVANCE: The HeLP score had acceptable performance, and the T-STAT had poor performance for diagnosis prediction. A tool with excellent or outstanding discrimination is needed to more reliably predict the presence of hemangiosarcoma or a malignant lesion preoperatively.