Search Results (16)

Background: A wandering accessory spleen (WAS) is defined as an ectopic accessory spleen with a long vascular pedicle characterized by marked anatomical mobility. “Wandering” can be congenital or acquired due to splenomegaly or pregnancy. The study aimed to analyze the clinical course of WAS and its symptoms. Methods: The desk research method and statistical analysis were used to assess the outcome. Results: In total, 12 cases of WAS managed operatively, including 11 women and 1 man, have been found. The correct WAS diagnosis was established before surgery in 3 of the 12 patients. One individual did not exhibit any symptoms and underwent surgery to avoid WAS torsion and infarction. Abdominal pain was the most commonly reported symptom in 11 patients. No mortality has been reported. Conclusions: WAS is a rare anomaly. It can be symptomatic or produce a spectrum of symptoms, such as abdominal pain, and may resemble abdominal neoplasms. Torsion is the most common pathology in the WAS study group and is most common in young people. Full article

Background/Objectives: Chronic idiopathic thrombocytopenic purpura (chITP) is an autoimmune disease which develops in 10–30% of patients with newly diagnosed idiopathic thrombocytopenic purpura (ndITP). It is defined as thrombocytopenia which lasts longer than 12 months, with extremely diverse clinical expressions. The aim is to present the most significant clinical and laboratory characteristics of children with chITP. Methods: This is retrospective, observational research, which included children between 2–18 years with chITP who were treated in the Republic of Serbia for 25 years. We analyzed clinical data from personal and family medical histories and different laboratory analyses. Results: The total number of respondents was 152, with female predominance (F:M = 1.27:1) and mild predominance of adolescents. Of the patients, 15% were asymptomatic, but 15% had periodically life-threatening bleeding. Transfusion was not required for 70% of patients. Thirty-five percent of patients had chITP alone, and 45% had high titer levels of autoantibodies. The most frequent comorbidity was Hashimoto thyroiditis (15%). The same percentage (45%) of family members were reported with and without autoimmune diseases. Twenty-five percent of patients were resistant to initial therapy. Helicobacter pylori was detected in 20%, 70% had higher levels of lactate dehydrogenase (LDH), three patients had sufficient serum vitamin D levels, splenomegaly was found in 25%, and accessory spleen in 14% of patients. Around 50% of patients had a platelet count between 20–50 × 10⁹/L, and 40% below 20 × 10⁹/L. Mean platelet volume (MPV) was 10.6 ± 1.4 fL. No dysplastic changes were noted in bone marrow aspirate. Initial first-line therapy was sufficient for 45% of patients, second-line therapy was administered in 25%, splenectomy was performed in 20%, and 10% received all available treatments. Conclusions: The severe clinical form of pediatric chITP is accompanied by a low platelet count, the presence of autoimmune comorbidities, a positive family medical history, resistance to initial therapy, hypovitaminosis D, and rare megakaryocytes in the bone marrow. Full article

Several solid lesions can be found within the pancreas mainly arising from the exocrine and endocrine pancreatic tissue. Among all pancreatic malignancies, the most common subtype is pancreatic ductal adenocarcinoma (PDAC), to a point that pancreatic cancer and PDAC are used interchangeably. But, in addition to PDAC, and to the other most common and well-known solid lesions, either related to benign conditions, such as pancreatitis, or not so benign, such as pancreatic neuroendocrine neoplasms (pNENs), there are solid pancreatic lesions considered rare due to their low incidence. These lesions may originate from a cell line with a differentiation other than exocrine/endocrine, such as from the nerve sheath as for pancreatic schwannoma or from mesenchymal cells as for solitary fibrous tumour. These rare solid pancreatic lesions may show a behaviour that ranges in a benign to highly aggressive malignant spectrum. This review includes cases of an intrapancreatic accessory spleen, pancreatic tuberculosis, solid serous cystadenoma, solid pseudopapillary tumour, pancreatic schwannoma, purely intraductal neuroendocrine tumour, pancreatic fibrous solitary tumour, acinar cell carcinoma, undifferentiated carcinoma with osteoclastic-like giant cells, adenosquamous carcinoma, colloid carcinoma of the pancreas, primary leiomyosarcoma of the pancreas, primary and secondary pancreatic lymphoma and metastases within the pancreas. Therefore, it is important to determine the correct diagnosis to ensure optimal patient management. Because of their rarity, their existence is less well known and, when depicted, in most cases incidentally, the correct diagnosis remains challenging. However, there are some typical imaging features present on cross-sectional imaging modalities that, taken into account with the clinical and biological context, contribute substantially to achieve the correct diagnosis. Full article

The presence of multiple accessory spleens in the abdominal cavity is typically limited to two, with cases involving a higher number being exceedingly rare. Concurrently, accessory spleen infarction is remarkably uncommon, primarily resulting from torsion of the vascular pedicle. In this report, we present a case of a 19-year-old male who experienced infarction in one of four accessory spleens. Imaging diagnosis proved challenging, with the definitive diagnosis being made through postoperative pathology, revealing no torsion in the affected accessory spleen. Following surgery combined with anti-inflammatory and analgesic treatment, the patient exhibited an uneventful recovery. No complications were observed at the 3-month follow-up. This case indicates the challenge and difficulty of diagnosing accessory splenic infarction without torsion in imaging diagnosis. Employing a multimodality approach and diffusion-weighted imaging may aid in confirming the diagnosis. Full article

Solid pseudopapillary neoplasm (SPN) is rare pancreatic tumor occurring most commonly in young females. The typical imaging appearance of SPN is of well-defined, encapsulated, and large heterogeneous tumors, consisting of solid and cystic components due to various degrees of intralesional hemorrhage and necrosis. However, atypical imaging presentation in the form of small solid tumors or uniformly cystic lesions might also be seen, which can be explained by specific pathological characteristics. Other imaging features such as a round shape, the absence of main pancreatic duct dilatation, and slow growth, in combination with vague symptoms, favor the diagnosis of SPNs. Nevertheless, the radiological findings of SPN might overlap with other solid and cystic pancreatic neoplasms, such as neuroendocrine tumors, serous and mucinous neoplasms, and even small pancreatic adenocarcinomas. In addition, a few benign non-tumorous conditions including walled-of-necrosis, and intrapancreatic accessory spleen may also pose diagnostic dilemmas simulating SPNs on imaging studies. The aim of this manuscript is to provide a comprehensive overview of the typical and atypical imaging features of SPNs and to describe useful tips for differential diagnosis with its potential mimickers. Full article

Background: The incidence of benign diseases among pancreatic resections for suspected malignancy still represents a relevant issue in the surgical practice. This study aims to identify the preoperative pitfalls that led to unnecessary surgeries at a single Austrian center over a twenty-year period. Methods: Patients undergoing surgery for suspected pancreatic/periampullary malignancy between 2000 and 2019 at the Linz Elisabethinen Hospital were included. The rate of “mismatches” between clinical suspicion and histology was considered as primary outcome. All cases that, despite that, fulfilled the indication criteria for surgery were defined as minor mismatches (MIN-M). Conversely, the true avoidable surgeries were identified as major mismatches (MAJ-M). Results: Among the 320 included patients, 13 (4%) presented with benign lesions at definitive pathology. The rate of MAJ-M was 2.8% (n = 9), and the most frequent causes of misdiagnoses were autoimmune pancreatitis (n = 4) and intrapancreatic accessory spleen (n = 2). In all MAJ-M cases, various mistakes within the preoperative workup were detected: lack of multidisciplinary discussion (n = 7, 77.8%); inappropriate imaging (n = 4, 44.4%); lack of specific blood markers (n = 7, 77.8%). The morbidity and mortality rates for mismatches were 46.7% and 0. Conclusion: All avoidable surgeries were the result of an incomplete pre-operative workup. The adequate identification of the underlying pitfalls could lead to minimize and, potentially, overcome this phenomenon with a concrete optimization of the surgical-care process. Full article

Background: Precise knowledge of the topographic features of the splenic artery and its branches in the hilum region is of practical interest due to the various interventions on the vessels of the spleen. Materials and methods: The anatomy of the spleen was studied by means of macroscopic dissection on 330 organ complexes, which were carefully documented and analyzed statistically. Results: The analysis of the splenic artery trajectory led to identification of four types: straight (43.03%), sinusoidal (27.58%), serpentine (20.91%) and alternating (8.48%). To assess the relation between the trajectory of the splenic artery and its branches we performed a chi square test. Sinuous or serpentine trajectory was associated with the presence of long splenic artery branches (dorsal pancreatic artery or the great pancreatic artery), X2 (2, N = 330) = 12.85, p = 0.001. The artery was located suprapancreatic in 70.30% of cases, anteropancreatic in 4.55%, the vessel had an intrapancreatic course in 14.85% and in 10.00% of cases the artery was located retropancreatic. The presence of inferior polar arteries was associated with a longer pancreas (Spearman’s correlation; r = 0.37; p = 0.037). In a multiple regression analysis, inferior polar arteries predicted the length of the pancreas although only a small number of cases could be explained by this model (R2 = 0.127, Adjusted R2 = 0.098; Betta = 0.357; t(330) = 2.091; p = 0.045). There were 30 (9.09%) cases of accessory spleens. Conclusions: The arterial supply of the spleen is highly variable in its trajectory, terminal branches, and relation to other organs. The splenic artery tends to be sinuous or serpentine in zones when a large artery branches off (e.g., the dorsal pancreatic or greater pancreatic artery). Multiple short branches tend to stabilize the trajectory of the splenic artery. Inferior polar arteries and accessory spleens contribute to the length of the pancreas, most likely due to increased vascular supply to the tail of the gland. Full article

No data exist for the association between the presence of accessory spleen after splenectomy and response to rituximab in immune thrombocytopenia (ITP). We investigated the relationship between accessory spleen presence and rituximab response in splenectomized ITP patients. Fifteen chronic refractory ITP patients were included. Four weekly doses of rituximab 375 mg/m² were administered. All patients had undergone splenectomy before rituximab administration. Accessory spleen was detected in 5 of 15 patients (33.3%). Median age at diagnosis was significantly higher in patients with accessory spleen than those without accessory spleen (40 (range 25–68 years) and 26 (range 7–40 years), respectively; p = 0.049). There was a trend for older age at time of rituximab initiation in patients with accessory spleen compared to the other group (median 51 (range 43–75 years) and 42.5 (range 30–60 years), respectively; p = 0.066). Median follow-up duration was 96 months (range 40–98). We demonstrated a significant correlation between accessory spleen presence and older age. Accessory spleen presence correlated with higher platelet and WBC counts. We showed good inverse correlation between presence of accessory spleen and time to early response (ER) to rituximab while the rate of early response (ER), late response (LR), sustained response (SR) and overall response (OR) did not differ with respect to the presence of acessory spleen. Full article

Background: Red blood cells (RBC) scintigraphy can be used not only for detection of bleeding sites, but also of spleen tissue. However, there is no established quantitative readout. Therefore, we investigated uptake in suspected splenic lesions in direct quantitative correlation to sites of physiologic uptake in order to objectify the readout. Methods: 20 patients with Tc-99m-labelled RBC scintigraphy and SPECT/low-dose CT for assessment of suspected splenic tissue were included. Lesions were rated as vital splenic or non-splenic tissue, and uptake and physiologic uptake of bone marrow, pancreas, and spleen were then quantified using a volume-of-interest based approach. Hepatic uptake served as a reference. Results: The median uptake ratio was significantly higher in splenic (2.82 (range, 0.58–24.10), n = 47) compared to other lesions (0.49 (0.01–0.83), n = 7), p < 0.001, and 5 lesions were newly discovered. The median pancreatic uptake was 0.09 (range 0.03–0.67), bone marrow 0.17 (0.03–0.45), and orthotopic spleen 14.45 (3.04–29.82). Compared to orthotopic spleens, the pancreas showed lowest uptake (0.09 vs. 14.45, p = 0.004). Based on pancreatic uptake we defined a cutoff (0.75) to distinguish splenic from other tissues. Conclusion: As the uptake in extra-splenic regions is invariably low compared to splenules, it can be used as comparator for evaluating suspected splenic tissues. Full article

Endoscopic ultrasonography (EUS) has been applied to the diagnosis of various digestive disorders. Although it has been widely accepted and its diagnostic value is high, the dependence of EUS diagnosis on image interpretation done by the endosonographer has persisted as an important difficulty. Consequently, high interobserver reliability (IOR) in EUS diagnosis is important to demonstrate the reliability of EUS diagnosis. We reviewed the literature on the IOR of EUS diagnosis for various diseases such as chronic pancreatitis, pancreatic solid/cystic mass, lymphadenopathy, and gastrointestinal and subepithelial lesions. The IOR of EUS diagnosis differs depending on the disease; moreover, EUS findings with high IOR and those with IOR that was not necessarily high were used as diagnostic criteria. Therefore, to further increase the value of EUS diagnosis, EUS diagnostic criteria with high diagnostic characteristics based on EUS findings with high IOR must be established. Full article

Chronic lymphocytic leukemia (CLL) is a B-lymphoproliferative disease, which consists of the abnormal proliferation of CD19/CD5/CD20/CD23 positive lymphocytes in blood and lymphoid organs, such as bone marrow, lymph nodes and spleen. The neoplastic transformation and expansion of tumor B cells are commonly recognized as antigen-driven processes, mediated by the interaction of antigens with the B cell receptor (BCR) expressed on the surface of B-lymphocytes. The survival and progression of CLL cells largely depend on the direct interaction of CLL cells with receptors of accessory cells of tumor microenvironment. Recently, much interest has been focused on the role of tumor release of small extracellular vesicles (EVs), named exosomes, which incorporate a wide range of biologically active molecules, particularly microRNAs and proteins, which sustain the tumor growth. Here, we will review the role of CLL-derived exosomes as diagnostic and prognostic biomarkers of the disease. Full article

Tumors of mesenchymal origin are rarely reported in the pancreas. Therefore, this study characterized 17 feline non-epithelial pancreatic tumors, including clinical data, histopathology, and immunohistochemistry. Seventeen feline pancreatic tissue samples were investigated histopathologically and immunohistochemically. Selected pancreatic and inflammatory serum parameters, e.g., feline pancreatic lipase immunoreactivity (fPLI), 1,2-o-dilauryl-rac-glycero-3-glutaric acid-(6′-methylresorufin) ester (DGGR) lipase and serum amyloid A (SAA), were recorded, when available. The neoplasms were characterized as round (n = 13) or spindle (n = 4) cell tumors. Round cell tumors included 12 lymphomas and one mast cell tumor in ectopic splenic tissue within the pancreas. Lymphomas were of T-cell (n = 9) or B-cell (n = 3) origin. These cats showed leukocytosis (3/3) and increased fPLI (5/5), DGGR lipase (3/5) and SAA (4/5) values. Spindle cell tumors included two hemangiosarcomas, one pleomorphic sarcoma and one fibrosarcoma. The cat with pleomorphic sarcoma showed increased SAA value. Overall survival time was two weeks to seven months. These are the first descriptions of a pancreatic pleomorphic sarcoma and a mast cell tumor in accessory spleens within feline pancreas. Although rare, pancreatic tumors should be considered in cats presenting with clinical signs and clinical pathology changes of pancreatitis. Only histopathology can certainly distinguish solitary pancreatitis from a neoplasm with inflammation. Full article

Zika virus (ZIKV) is an arbovirus that causes birth defects, persistent male infection, and sexual transmission in humans. The purpose of this study was to continue the development of an ovine ZIKV infection model; thus, two experiments were undertaken. In the first experiment, we built on previous pregnant sheep experiments by developing a mid-gestation model of ZIKV infection. Four pregnant sheep were challenged with ZIKV at 57–64 days gestation; two animals served as controls. After 13–15 days (corresponding with 70–79 days of gestation), one control and two infected animals were euthanized; the remaining animals were euthanized at 20–22 days post-infection (corresponding with 77–86 days of gestation). In the second experiment, six sexually mature, intact, male sheep were challenged with ZIKV and two animals served as controls. Infected animals were serially euthanized on days 2–6 and day 9 post-infection with the goal of isolating ZIKV from the male reproductive tract. In the mid-gestation study, virus was detected in maternal placenta and spleen, and in fetal organs, including the brains, spleens/liver, and umbilicus of infected fetuses. Fetuses from infected animals had visibly misshapen heads and morphometrics revealed significantly smaller head sizes in infected fetuses when compared to controls. Placental pathology was evident in infected dams. In the male experiment, ZIKV was detected in the spleen, liver, testes/epididymides, and accessory sex glands of infected animals. Results from both experiments indicate that mid-gestation ewes can be infected with ZIKV with subsequent disruption of fetal development and that intact male sheep are susceptible to ZIKV infection and viral dissemination and replication occurs in highly vascular tissues (including those of the male reproductive tract). Full article

The cell type-, organ-, and species-specific expression of the Toll-like receptors (TLRs) are well described, but little is known about the respective expression profiles of their accessory molecules. We therefore determined the mRNA expression levels of LBP, MD2, CD36, CD14, granulin, HMGB1, LL37, GRP94, UNC93b1, TRIL, PRAT4A, AP3B1, AEP and the respective TLRs in human and mouse solid organs. Humans and mice displayed significant differences between their respective mRNA expression patterns of these factors. In addition, the expression profiles in transient tissue inflammation upon renal ischemia-reperfusion injury, in spleens and kidneys from mice with lupus-like systemic autoimmunity, and in progressive tissue fibrosis upon unilateral ureteral obstruction were studied. Several TLR co-factors were specifically regulated during the different phases of these disease entities, suggesting a functional involvement in the disease process. Thus, the organ- and species-specific expression patterns need to be considered in the design and interpretation of studies related to TLR-mediated innate immunity, which seems to be involved in the tissue injury phase, in the phase of tissue regeneration, and in progressive tissue remodelling. Full article