Extraskeletal osteosarcoma infiltrating pancreas, spleen, gastric, and left kidney: a case report.

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.

Integrated spatial and multimodal single-cell transcriptomics reveal patient-dependent cell heterogeneity in splenic marginal zone lymphoma.

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.

Evaluation of clopidogrel, hypercoagulability, and platelet count in dogs undergoing splenectomy for splenic masses.

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.

Sclerosing angiomatoid nodular transformation of the spleen: Case reports and literature review.

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.

Prognostic value of tumour-related factors associated with canine retroperitoneal hemangiosarcoma in comparison with other anatomic presentations: A retrospective observational study.

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.

Clinicopathologic findings of splenic marginal zone lymphoma with gallbladder involvement that progressed to diffuse large B-cell lymphoma in a dog.

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.

Imaging findings of Epstein-Barr Virus-positive inflammatory follicular dendritic cell sarcoma of spleen: A case report.

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.

Minimally Invasive Conversion Surgery for Unresectable Gastric Cancer with Splenic Metastasis and Splenic Vein Tumor Thrombus: A Case Report.

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.

Clinicopathological Analysis of Epstein-Barr Virus-Positive Inflammatory Follicular Dendritic Cell Sarcoma of the Spleen: A Case Report.

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.

Case report: Fatal overwhelming post-splenectomy infection in a patient with metastatic angiosarcoma treated with immunotherapy.

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.

Improved predictability is needed for calculators used to preoperatively determine the etiology of splenic masses in dogs: an external validation study of the HeLP score and T-STAT.

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.

Real-world evidence of treatment patterns and survival of metastatic gastric cancer patients in Germany.

BACKGROUND: Patients with metastatic gastric cancer (mGC) have poor prognosis. This real-world study aimed to describe treatment regimens and survival of mGC patients. METHODS: A retrospective analysis was conducted using anonymized German claims data (AOK PLUS) covering a period from 2010 to 2021. The study population included newly diagnosed mGC cases identified from 2011 to 2020. The index date was defined as the first diagnosis of metastasis on or after gastric cancer diagnosis. Therapy regimens were identified based on inpatient and outpatient data, and subsequently stratified by line of treatment. Survival analyses were conducted using the Kaplan-Meier method. RESULTS: The cohort consisted of 5,278 mGC incident cases (mean age: 72.7 years; male: 61.9%). Nearly half of the incident cases received mGC-related treatment (49.8%). Treated patients were more often male, younger, and had fewer comorbidities compared to untreated patients. Of the 2,629 mGC patients who started the first line of treatment (1LOT), 32.8% switched to 2LOT, and 10.2% reached 3LOT. Longer survival time was observed among disease-specific treated cases compared with untreated cases (median real-world overall survival (rwOS): 12.7 months [95%CI 12.1 - 13.3 months] vs. 3.7 months [95%CI 3.4 - 4.0 months]). CONCLUSION: Systemic therapy was not received in almost half of the mGC patients. In those patients, a very short median rwOS was observed. Treatment patterns were generally in line with the guideline recommendations, however, therapy switching rates and poor prognosis indicate high unmet needs also in the treated population.

Incidental Detection of Synchronous Benign Hepatic and Splenic Hemangiomas on 18 F-PSMA PET/CT.

ABSTRACT: A 51-year-old man with biochemical failure after brachytherapy for prostatic adenocarcinoma (PSA 5 µg/L rising to 6.45 µg/L) underwent a PSMA PET/CT scan. The scan revealed focal 18 F-PSMA activity at the right apex suggestive of local residual or recurrent disease. In addition, 18 F-PSMA images demonstrated 2 focal 18 F-PSMA-avid liver and spleen lesions; both lesions were further evaluated by abdominal MRI, and the final radiological diagnosis was synchronous hepatic and splenic hemangiomas.

Littoral cell angioma of the spleen: A study of 10 cases case series and literature review.

BACKGROUND: Current study aimed to investigate the clinical characterization, differential diagnosis, and treatment of splenic littoral cell angioma (LCA). METHODS: A retrospective analysis was performed for 10 LCA cases admitted to Huzhou Central Hospital from 2007 to 2023, for clinical manifestations, hematological tests, imaging features, pathological features, treatment methods, and prognosis along with the relevant literature was also reviewed. RESULTS: During examinations, no specific clinical manifestations and hematological abnormalities were seen in all 10 cases of LCA. Imaging observations depicted single or even multiple spherical lesions in the spleen. Plains shown by computed tomography (CT) were found somewhat equal or slightly lower in density. On the other hand, magnetic resonance imaging (MRI) plain scans viz. T1 weighted image showed equal low and mixed signals while T2-weighted showed high and low mixed signals. Moreover, punctate low signals could be seen in high signals named "freckle sign" in MRI scans. On contrast-enhanced CT scans, the enhancement of the lesions was not obvious in the arterial phase, and some of the lesions showed edged ring-like enhancements and "filling lake" progressive enhancement during the venous phase and delayed phase. In multiple lesions, the number of enhanced scan lesions showed a variable changing pattern "less-more-less." MRI-enhanced scan showed the characteristics of "fast in and slow out." Microscopic examinations identified tumor tissue actually composed of sinus-like lacunae that anastomosed with each other in the form of a network. Furthermore, cystic expansion and pseudopapillary protrusions were also seen in the dilated sinus cavity which was lined with single-layer endothelial cells having conspicuous cytoplasmic hemosiderin. High immunophenotypic expressions of vascular endothelial cell phenotype (CD31, CD34, FVIII) and tissue cell phenotype (CD68) were also seen. Total and partial splenectomy were performed in 8 and 2 patients, respectively, and follow-up examinations showed survival in all patients with no recurrence. CONCLUSION: LCA is a rare splenic benign lesion with atypical clinical manifestations. CT and MRI imaging are important tools in preoperative diagnosis based on pathomorphological and immunohistochemical examinations. Splenectomy is a superior therapeutic choice with significant impacts and prognosis.

Atypically located accessory splenic hemangiosarcoma in a Dog: Diagnostic value of triple-phase computed tomography.

A 10-year-old Cocker spaniel presented with lethargy. Triple-phase computed tomography was obtained with a contrast test bolus at the level of porta hepatis, which revealed a right lower abdominal mass. The mass was not connected to other abdominal organs; however, a linear structure was observed connecting the splenic hilum to the mass, which was suspected to be the feeding vessel. The arterial phase image was obtained again with a contrast bolus at the level of the celiac artery. A prominent contrast-enhanced feeding artery originating from the splenic artery to the mass was observed. Histopathology confirmed an accessory splenic hemangiosarcoma.