[Histiocytic sarcoma derived from the same origin as splenic marginal zone lymphoma revealed by exome analysis].

Histiocytic sarcoma (HS) is a rare aggressive hematological malignancy reported to occur secondary to B cell lymphoma. We report a case of HS secondary to splenic marginal zone lymphoma (SMZL) complicated by autoimmune hemolytic anemia (AIHA) in a 64-year-old man. He was referred to our department with anemia and was diagnosed as having AIHA. After starting treatment with prednisolone, atypical lymphocytes appeared in his blood tests, and a bone marrow biopsy revealed invasion by B cell lymphoma. A CT scan showed splenomegaly and a pancreatic mass, which confirmed the diagnosis of SMZL. The patient received bendamustine and rituximab as chemotherapy, which rapidly improved the anemia and splenomegaly and reduced atypical lymphocytes. However, left lumbar back pain appeared along with an increase in the pancreatic mass, and he died suddenly of acute renal failure. An autopsy revealed that the tumor had invaded several organs including the pancreas, and immunohistochemistry was positive for CD163, leading to the diagnosis of HS. Furthermore, the specimens of SMZL and HS were positive for IgH gene reconstitution, and exome analysis showed genetic abnormalities in 226 genes including CARD11, suggesting that the SMZL and HS had the same origin.

Circulating nucleosomes as a potential cancer biomarker in dogs with splenic nodular lesions.

Splenic nodular lesions in dogs can be either benign or malignant. They might be discovered incidentally or, in case of rupture, they may lead to hemoabdomen. Nevertheless, splenectomy followed by histopathology is essential for diagnosis and to prevent rupture. Yet, this invasive procedure might be postponed for dogs with benign splenic nodular lesions. Conversely, owners may opt for euthanasia over surgery for malignancies with poor prognosis like hemangiosarcoma. Thus, anticipating diagnosis with non-invasive biomarkers is crucial for proper patient management. In this prospective study, plasma samples were collected from 66 dogs with histologically confirmed splenic nodular lesions. A canine-specific ELISA kit was applied to assess nucleosome concentration, with histopathology of the spleen serving as the gold standard. Nucleosome concentration was found to be significantly higher in dogs with malignant splenic nodular lesions, particularly in those with hemangiosarcoma and other malignancies. The presence of hemoabdomen, more prevalent in dogs with splenic malignancy, also resulted in increased plasmatic nucleosome concentrations. Plasma nucleosomes could serve as a biomarker for detecting malignant splenic nodular lesions in dogs. More research is needed to understand how nucleosome concentration relate to disease stage and prognosis in dogs with hemangiosarcoma.

[Surgical treatment of littoral cell angioma of the spleen]. / Opyt khirurgicheskogo lecheniya «pribrezhnokletochnoi¼ (littoral cell) angiomy selezenki.

Littoral cell angioma is a very rare benign splenic neoplasm with typical morphological features. It is difficult to differentiate this angioma from other benign or malignant splenic tumors. It is a unique vascular tumor followed by abdominal pain or detected incidentally during examination. Incidence of this tumor is small, as only few appropriate cases were described in the literature. We present successful surgical treatment of littoral cell angioma of the spleen. The issues of clinical course, diagnosis, therapeutic tactics, morphologic and histochemical features are discussed.

[Solitary splenic neoplasm as an unusual presentation in an adolescent with sporadic Burkitt lymphoma]. / Neoplasia esplénica solitaria como presentación inusual en una adolescente con linfoma de Burkitt esporádico.

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.

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.

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.

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.

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.

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.

Splenic marginal zone lymphoma with monoclonal IgG: A case report.

RATIONALE: Splenic marginal zone lymphoma (SMZL), an indolent small B-cell lymphoma, is uncommon, and part of the patients exist plasmocytic differentiation and secrete monoclonal paraproteins including IgM predominantly. SMZL with monoclonal IgG is rarer. PATIENT CONCERNS: We report a case of SMZL (49-year-old, male) with monoclonal IgG, MYD88L265P mutation and hepatitis B virus infection. DIAGNOSES: The patient was presented to our hospital with aggravating complaints of dizziness, fatigue, postprandial abdominal distension, and night sweats. The diagnosis was confirmed by clinical manifestations, immunophenotype, bone marrow pathology. INTERVENTIONS: The patient received rituximab-based chemotherapy and sequential ibrutinib in combination with entecavir. OUTCOMES: After 1 year of follow-up, his blood routine examination had returned to normal with normal level of albumin and significantly lower globulin than before, and the spleen was of normal size. LESSONS: We conclude that rituximab-based chemotherapy is the main treatment option for the patients with SMZL, and Bruton's tyrosine kinase inhibitor has also shown beneficial efficacy.

Sclerosing angiomatoid nodular transformation of spleen: A rare case report.

ABSTRACT: Sclerosing angiomatoid nodular transformation (SANT) is a reactive non-neoplastic, rare vascular lesion of the spleen. The histology shows multiple angiomatoid nodules surrounded by proliferative stroma. A 31-year-old lady presented with an abdominal mass for 6 months. Contrast-enhanced computed tomography (CECT) abdomen was suggestive of hemangiopericytoma/hemangioendothelioma. An open splenectomy was performed, and the resected specimen was sent for histopathology examination. The gross examination showed a bosselated mass present at the lower pole of the spleen measuring 8 × 8 cm with peripherally located coalescing red-brown nodules embedded in a dense fibrous stroma on the cut surface. On microscopy, multiple circumscribed angiomatoid nodules comprising irregular slit-like vascular channels lined by plump endothelial cells were seen embedded in dense sclerotic stroma. Because of the lack of specific diagnostic features, it is difficult to diagnose SANT clinically and radiologically. However, the typical histopathological findings are a clue in clinching the diagnosis.

Clinicopathological analysis of sclerosing angiomatoid nodular transformation in the spleen.

Background: Splenic sclerosing angiomatoid nodular transformation (SANT) is a rare benign nodular lesion in the red medulla of the spleen. In the past, SANT has not been consistently recognized as the name for this condition and was often misdiagnosed for other conditions. In recent years, SANT has been acknowledged by most scholars as multiple reports have been published. Aim: To assess the clinicopathological features of SANT to identify the histological characteristics of SANT to improve diagnosis and clinical treatment. Materials and Methods: We assessed 25 cases of SANT diagnosed at Zhongshan Hospital affiliated with Fudan University from September 2014 to October 2021, including 14 men and 11 women, aged 24-62 years old. Results: Fourteen cases were complicated with benign tumors of the liver, pancreas, kidney, uterus, and prostate. One case was complicated with renal clear cell carcinoma, and one was complicated with hepatocellular carcinoma. The gross neoplasm is multinodular and well defined. Histologically, angiomatoid nodules are composed of fattened, round, or irregular blood vessels, with or without red blood cells in the lumen, with unequal red blood cell extravasation, and fibrocytes around the nodules. The hemangiomatous nodules were positive for CD31 and CD34, while the vascular wall smooth muscle cells and fibrocytes around the nodules were positive for SMA. Conclusion: The diagnosis of SANT requires a combination of immunohistochemical and histological features, and early splenectomy is crucial for treatment.

Prognostic impact of Ki-67 in canine splenic hemangiosarcoma: A preliminary study.

Canine splenic hemangiosarcoma has a high metastatic rate and short survival time. Currently, the main prognostic parameters are tumor stage and therapy, while data on histologic parameters, such as grade and Ki-67 expression, are scarce. The aims of this study were to compare two methods of assessment of Ki-67, verify their prognostic impact, and define a threshold value based on survival. Thirty-one cases of histologically diagnosed canine splenic hemangiosarcoma, which were treated with splenectomy and had full staging and follow-up information, were collected. Three were stage I, 17 stage II, and 11 stage III. The mean mitotic count (MC) was 23.9 (standard deviation [SD]: 22.1) and the median was 15 (range, 1-93). Immunohistochemistry for Ki-67 was performed, the Ki-67 labeling index (Ki-67LI) was assessed as a percentage of positive neoplastic nuclei per ≥500 cell, and the Ki-67 count (KI-67C) was defined as the average number of positive nuclei using a 1 cm2 optical grid performed in 5, 40× fields. The mean Ki-67LI and Ki-67C were 56.4% (SD: 38.7) and 27.2 (SD: 12.9) and medians were 51% (range, 8.2-55.2) and 26 (range, 5.5-148), respectively. Using a cut-off of 56% and 9, respectively, Kaplan-Meier survival curves showed an association of overall survival with Ki-67LI and MC. In addition to clinical stage, Ki-67LI maintained its prognostic value on multivariate analysis, supporting the role of Ki-67LI as an independent prognostic parameter. Based on these results, we propose a diagnostically applicable cut-off value of 56% for Ki-67LI as a prognostic parameter for canine splenic hemangiosarcoma.

Unusual Case of Splenic Metastasis in Adenosquamous Carcinoma of the Cervix Uteri: Diagnosis and Treatment Considerations.

BACKGROUND Due to several factors such as its specific cellular and biochemical microenvironment, the spleen is not a predestined organ of frequent metastatic colonization in the case of primary solid carcinoma. Hence, the mode of diagnosis and the preferred treatment of a lesion highly suspicious of splenic metastasis must be decided on a case-by-case basis, considering not only the biological tumor entity but also the stage of the primary disease. CASE REPORT In the present case, we demonstrate the clinical course of a 37-year-old female patient who initially presented to our clinic with irregular vaginal bleeding. A consecutive gynecological examination revealed a 3×3-cm large mass of the cervix uteri, and the subsequent histomorphological workup led to the diagnosis of an adenosquamous carcinoma of the cervix uteri. Therapeutically, the patient received multimodal treatment, namely radical hysterectomy with adjuvant radio-chemotherapy. After 1.5 years, the patient presented to our Emergency Department with intermittent left-sided abdominal pain. Subsequent abdominal imaging (computed tomography scan, magnetic resonance imaging, positron emission tomography) determined a metabolically active splenic lesion with a central necrosis - signs of malignancy in line with a splenic metastasis. Presentation and discussion of the case within our interdisciplinary tumor board led to the decision of splenectomy followed by chemotherapy, a procedure that could be considered as therapeutic treatment in such exceptional cases. CONCLUSIONS The collection and reporting of atypical clinical courses remains a key factor in precision medicine to enable the most evidence-based decision making in such cases.

Littoral cell angioma of the spleen associated with two primary cancers.

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.

Controversies in the Spleen: Histiocytic, Dendritic, and Stromal Cell Lesions.

Histiocytic, dendritic, and stromal cell lesions that occur in the spleen are challenging diagnostically, not well studied due to their rarity, and therefore somewhat controversial. New techniques for obtaining tissue samples also create challenges as splenectomy is no longer common and needle biopsy does not afford the same opportunity for examination of tissue. Characteristic primary splenic histiocytic, dendritic, and stromal cell lesions are presented in this paper with new molecular genetic findings in some entities that help differentiate these lesions from those occurring in non-splenic sites, such as soft tissue, and identify possible molecular markers for diagnosis.

Littoral cell angioma discovered after splenectomy in a patient with non-Hodgkin lymphoma and melanoma. Case report and review.

BACKGROUND: Littoral cell angioma (LCA) is a rare vascular primary tumor of the spleen with no more than 440 cases described so far. Although often seen as benign, it is described to have malignant potential and to be associated with other immunologic disorders or malignancies. CASE PRESENTATION: We present the case of LCA in a 75-year old man with a concomitant non-Hodgkin lymphoma and medical history of malign melanoma. The tumor was discovered incidentally after splenectomy for splenomegaly and refractory thrombocytopenia. The post-operative period was uneventful. CONCLUSION: Our case is the first to report an association of LCA with both lymphoma and melanoma thus far. It emphasizes the need for a thorough total body examination for synchronous diseases and close follow-up to reveal associated malignancies or immunologic disorders. Further research is required to identify etiologic and pathogenetic mechanisms behind this tumor and a common basis between the three diseases. KEY WORDS: Littoral Cell Angioma, Neoplasm, Splenectomy, Solid Spleen Tumor.

Unraveling the genetics of transformed splenic marginal zone lymphoma.

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.