Abdominal Lymphadenopathy Research Articles

P50 Systemic lupus erythematous presenting as Kikuchi-Fujimoto disease complicated with haemophagocytic lymphohistiocytosis: a case report and review of the literature

Abstract Introduction Kikuchi-Fujimoto disease (KFD) or histiocytic necrotising lymphadenitis is a rare, self-limiting inflammatory condition that can be associated with autoimmune connective tissue disorders (CTD) especially systemic lupus erythematosus (SLE). The aetiopathogenesis remains unclear but it is thought to be triggered by an infection in individuals with autoimmune predisposition leading to T-cells mediated apoptosis and inflammatory cascade. This condition most commonly affects children and young adults of Asian descent with a slight female preponderance although it can affect any age and ethnic groups. Cervical lymphadenopathy and fever are the salient manifestations, and it can be fatal if complicated with haemophagocytic lymphohistiocytosis (HLH). Case description A 16 year old girl presented with a 2-month history of persistent fever, progressive bilateral cervical lymphadenopathy, generalised maculopapular rash, angioedema-like facial swelling, oral ulcers and B symptoms. Laboratory investigations revealed pancytopaenia, profound acute phase response and abnormalities consistent with HLH with an HScore of 200 points. A thorough infectious workup was unrevealing apart from serological evidence of past Epstein-Barr virus (EBV) infection. ANA, anti-Sm, anti-RNP and anti-B2GP1 IgG were positive. An ultrasound of her neck showed bilateral multilevel cervical lymphadenopathy and the excision biopsy of her lymph node confirmed features of histiocytic necrotising lymphadenitis and ruled out lymphoma. Notably, there were scattered EBV-positive blasts and the special staining for mycobacteria was negative. A CT scan has also demonstrated bilateral axillary and abdominal lymphadenopathy with no hepatosplenomegaly. There was thrombosis in her right internal jugular vein with no intracranial or cardiac extension of the thrombus. The patient was admitted to ITU for close monitoring due to concerns about possible airway compromise but she did not require any respiratory support. She received intravenous (IV) methylprednisolone pulses for three consecutive days, mycophenolate mofetil, hydroxychloroquine and treatment dose of enoxaparin with good response and did not require IV immunoglobulins (IVIG) and/or interleukin-1 receptor antagonists. However, she subsequently developed an abscess in her neck which was complicated by Staphylococcus aureus bacteraemia and underwent an emergency incision and drainage of the abscess. She recovered well with IV antibiotics and was followed up in the CTD clinic with continuous psychological support from the rheumatology clinical nurse specialists. Discussion KFD most commonly presents with acute to subacute tender cervical lymphadenopathy and fevers which can be associated with other systemic features such as arthralgia, various cutaneous manifestations with great histologic heterogeneity, and neurological manifestations including aseptic meningitis, meningoencephalitis, acute cerebellar syndrome and optic neuritis. Generalised lymphadenopathy has been reported in 22% of cases but hepato- and/or splenomegaly is uncommon (3-5% of cases). Various infectious agents including the EBV have been implicated as the potential trigger of this condition but the evidence remains inconclusive. Our patient’s lymph node biopsy has demonstrated EBV-positive blasts with serological evidence of past EBV infection but a causal relationship between these findings and KFD cannot be established with certainty. It remains a diagnostic challenge to differentiate KFD from the other more sinister pathologies such as lymphoma, various infectious lymphadenitis and autoimmune conditions such as sarcoidosis and Kawasaki disease. Therefore, early excisional biopsy of the affected lymph nodes is essential for establishing the correct diagnosis and may obviate the need for inappropriate treatments. Both KFD and SLE share a complex clinicopathological relationship as SLE may precede, occur concurrently as in this case or develop later following the diagnosis of KFD. Moreover, lupus lymphadenitis can mimic KFD clinically and these two entities can only be distinguished histologically by the demonstration of haematoxylin bodies in lupus lymphadenitis. Our patient developed thrombosis in her right internal jugular vein which was thought to be secondary to the compressive effect from her cervical lymphadenopathy rather than due the antiphospholipid syndrome as her repeat antiphospholipid screen was negative. Key learning points • Clinicians should be aware of this rare clinical entity to make an early diagnosis and avoid unnecessary investigations. Differentiating KFD from the other sinister conditions such as lymphoma remains a diagnostic challenge therefore an early excisional biopsy of the affected lymph nodes would avoid delayed diagnosis and obviate the need for potentially risky treatments. Patients with KFD should be carefully screened and monitored for SLE as it can precede, occur in conjunction with or follow the diagnosis of SLE. Most cases of KFD resolve within 1-6 months although recurrence has been reported in 3-4% and the mortality rate is estimated to be 2.1%. It is crucial to follow up these patients given the increased risk of recurrence which can continue for many years. Despite having a good response to treatments, our patient suffered from significant alopecia and post-inflammatory hyperpigmentation in her skin which has had a great impact on her self-esteem and mental well-being. We would like to highlight in this case the biopsychosocial ramifications of this condition and demonstrate the importance of a multidisciplinary approach in the management of young people with CTD.

OA13 Trust your gut: it has the answer

Abstract Introduction We report the case of a 51-year-old man who presented over seven years to multiple specialities, including rheumatology on several occasions, with arthralgia, fevers and weight loss. He was found to have raised inflammatory markers, lymphadenopathy and pericardial and pleural effusions but despite extensive investigation over this time, the cause for his presentation remained elusive. After many years, he developed gastrointestinal symptoms, which led us to the unifying diagnosis of Whipple’s disease. Case description This patient initially presented to rheumatology in 2017 with left ankle pain. His only medical history was renal calculi. He was diagnosed with gout and osteoarthritis and was discharged. Two years later, he presented with fevers. Investigations revealed raised inflammatory markers, small pericardial and pleural effusions and small-volume upper abdominal lymphadenopathy. He was treated with colchicine for pericarditis with some improvement. He presented again in 2021 with chest and flank pain and breathlessness. CTPA revealed an ongoing small pericardial effusion and small-volume intrathoracic and intra-abdominal lymphadenopathy. Inflammatory markers remained raised (CRP 30-50, ESR 20-30). He had now begun to lose weight. He was referred to the cancer of unknown primary service, who monitored his lymphadenopathy. In January 2022, he underwent an open biopsy of a mesenteric lymph node, which revealed non-caseating granulomas and no evidence of malignancy. He was referred again to rheumatology due to ongoing migratory joint pains in his wrists, ankles and knees. Rheumatoid factor, anti-CCP, antinuclear antibody and serum ACE had been negative on several occasions. He underwent a PET scan that revealed persistent lymphadenopathy with low to moderate avidity, borderline splenomegaly and a slightly prominent marrow signal. He then had a bone marrow biopsy that showed no significant abnormality. In December 2023, we settled on a probable diagnosis of sarcoidosis. He was treated with a trial of steroid without much symptomatic improvement and there was a plan to initiate Methotrexate. However, he then developed nausea and vomiting and was noted to have a new iron deficiency anaemia. He was referred to gastroenterology and underwent an upper endoscopy, which was macroscopically normal, but microscopically showed findings in keeping with Whipple’s disease (WD) with PAS-positive staining. Duodenal biopsy PCR confirmed this. He is now awaiting input from the infectious diseases team and long-term antibiotic treatment. Discussion WD is caused by the bacterium Tropheryma whipplei. There are four commonly recognised manifestations of T. whipplei infections: 1) classic WD, 2) localised chronic infections, 3) acute infections and 4) asymptomatic carriage. Our patient has classic WD, and his case exemplifies the disease course and diagnostic challenges. Classic WD is a rare, chronic, multisystem infectious disease. It is reported as most often affecting middle-aged white males of European ancestry. It can be divided into an early phase with intermittent arthralgias or arthritis and fever, a middle phase with gastrointestinal symptoms and weight loss, and a late phase with predominant neurological symptoms. Arthritis is the presenting symptom in 60-70% of cases and occurs in 90% of all cases. Given this, around half of patients are initially misdiagnosed as having an inflammatory rheumatic disease. As in the case of our patient, joint manifestations do not correlate with intestinal symptoms and can precede intestinal manifestations by a mean interval of six years. Joint involvement in WD is typically a migratory oligo- or polyarthritis or arthralgia primarily involving large and medium joints, most commonly the knees, ankles and wrists. Less commonly affected joints are elbows, hips and shoulders. WD should be considered in all patients with the four cardinal symptoms of arthralgias, diarrhoea, abdominal pain, and weight loss after more common differentials have been excluded. In patients without gastrointestinal symptoms, having suspicion of the disease is more difficult; however, consideration should be made in patients with seronegative arthritis, who do not respond to immunosuppressive treatment. Other clinical syndromes in which WD should be considered include fever of unknown origin, chronic serositis, generalised lymphadenopathy, progressive central nervous system disease or early onset cognitive deficits. Although histology revealing non-caseating granulomas is the hallmark of sarcoidosis, they are non-specific and can be found in many other diseases. Key learning points • WD has a reputation as a great mimicker of many diseases and there is still often a considerable delay to diagnosis, even in typical cases, given the natural history of the disease and its rarity. WD should be considered in patients with seronegative arthritis not following a typical disease course, particularly if they develop gastrointestinal symptoms. WD is also a pertinent differential in patients with persistent constitutional upset with unexplained lymphadenopathy or serositis. It should be remembered that non-caseating granulomas can be found in many diseases and that sarcoidosis remains a diagnosis of exclusion among granulomatous disorders.

6402 Unsuspected Bladder Mass Turns Out To Be A Paraganglioma

Abstract Disclosure: S. Lamichhane: None. A. Flowers: None. D.T. Price: None. A. Kaur: None. Background: Urinary bladder (UB) paragangliomas are extremely rare tumors comprising of less than 0.05% of all bladder neoplasms and 10% of all paragangliomas. The diagnosis is usually suspected based on clinical features of painless hematuria, episodic headaches, hypertension, palpitations associated with micturition or bladder and confirmed biochemically by elevated urinary and/or plasma fractionated metanephrines (MN). Here, we present a case of a UB mass that was initially diagnosed on biopsy and subsequent biochemical workup revealed elevated MN. Case Report: A 64-year-old man with type 2 diabetes and hypertension, admitted for revision surgery of subdural hematoma was incidentally found to have a 3.3 x 3.4 x 3.9 cm polypoid heterogenous hyper enhancing UB mass attached to the anterior wall on CT abdomen done for abdominal pain. Cystoscopy and TURBT were complicated by hypertensive urgency. Pathology turned out to be paraganglioma. Detailed history at this time revealed a long-standing history of frequency, urgency, nocturia 4 times nightly, recurrent urinary tract infections, an episode of gross hematuria 1-2 years ago, and difficulty controlling blood pressure on 4 anti-hypertensives. Subsequently, labs showed plasma free MN of 97 pg/ml (ref range <=57), free normetanephrine (NMN) of 2392 pg/ml (ref range <=148), and total MN of 2489 pg/ml (ref range <=205). Genetic panel was negative for SDHA, SHD, AF2, SDHB, SHDC, SDHD, VHL, RET, TMEM127. He received 6 doses of Lantreotide due to suspicion of metastasis based on abdominal, pelvic, and inguinal lymphadenopathy seen on DOTATATE scan. After preparation with alpha and beta blockers, he underwent uneventful robot-assisted cystectomy with lymph node resection that resulted in a well-differentiated paraganglioma negative for margins, lymph node involvement, and extra bladder extension. A week later, MN reduced, plasma free MN 37 pg/ml, free NMN 178 pg/ml, and total plasma MN 215 pg/ml. His BP improved, and micturition-induced palpitations resolved. Conclusion: Due to the rarity of UB paraganglioma, the diagnosis can be missed and delayed. This has a serious implication due to the risk of hypertensive crisis during the surgery and increased risk of cardiovascular complications. This case highlights the need for awareness among physicians of the clinical presentation associated with UB paraganglioma, its early recognition and management. Presentation: 6/2/2024

EP110 - Chyloperitoneum in Follicular lymphoma – presenting as an inguinal hernia

Abstract Introduction Follicular lymphoma is one of the subtypes of slow growing Non-Hodgkin Lymphoma. Rarely, follicular lymphoma can present with chyloperitoneum or chylous ascites which is characterized by a milky-white coloured fluid with raised triglyceride levels. Case Report We present a case of a 60-year-old diabetic male, complaining of a right sided inguinal swelling for 1 year. Examination revealed a reducible right inguinal swelling with a positive cough impulse. The laboratory workup was unremarkable. Ultrasound scan of anterior abdominal wall showed a defect of 2.1 cm in right inguinal region with protrusion of abdominal contents and a positive cough impulse. A diagnosis of right sided reducible direct inguinal hernia was made. Patient was scheduled for Lichtenstein repair. Per-operatively, the hernial sac contained healthy omentum with milky-white ascitic fluid. Omentum was reduced and suture repair was performed. A drain was placed intra-peritoneally. The postoperative course of the patient was uneventful. Ascitic fluid analysis showed high triglyceride levels and no evidence of malignant cells, suggesting chylous ascites. Contrast enhanced CT scan of abdomen and pelvis revealed a large homogenous minimally enhancing soft tissue density retroperitoneal mass in peri aortic and paravertebral location with abdominal lymphadenopathy suggesting retroperitoneal lymphoma. Excisional biopsy of an enlarged inguinal lymph node confirmed follicular lymphoma with positive BCL2 and CD10 markers. Conclusion Follicular lymphoma presents in a variety of ways including chylous ascites in an inguinal hernia and a high index of suspicion is required to diagnose it.

Imaging Features and Recent Advances in Lymph Node Tuberculosis

AbstractThe burden of tuberculosis in our country persists, with diverse presentations affecting various organ systems, most commonly manifesting as pulmonary tuberculosis followed by nodal tuberculosis. While pulmonary involvement is frequently observed, nodal tuberculosis can present in isolation, commonly affecting cervical lymph nodes. Various imaging modalities play pivotal roles in diagnosis and assessment. Radiographs serve as initial screening modality in thoracic lymphadenopathy. Ultrasound is mostly used in cervical lymphadenopathy. Cases of thoracic and abdominal lymphadenopathy are usually evaluated with computed tomography but magnetic resonance imaging can be a radiation-free alternative. The role of fluorodeoxyglucose-positron emission tomography and newer modalities like shear-wave elastography and contrast-enhanced ultrasound are briefly discussed.

A diagnostic dilemma: distinguishing a sulfasalazine induced DRESS hypersensitivity syndrome from a CD30 + lymphoma in a young patient

Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe hypersensitivity reaction characterized by cutaneous rash, lymphadenopathy, fever, eosinophilia, leukocytosis, and life-threatening organ dysfunctions. We describe the case of a 26 year old patient admitted to the Emergency Department for DRESS syndrome after sulfasalazine treatment for rheumatoid arthritis in the right knee. Whole body computer tomography showed multiple neck, chest, and abdominal lymphadenopathy with splenomegaly, massive ascites and severe hepatic cytolysis. Serology results for Epstein-Barr Virus (EBV), influenza, measles, rubella, hepatitis A and B were negative. The histologic analysis of skin, lymph node and bone marrow biopsies could not indicate a classical Hodgkin’s Disease or iatrogenic immunodeficiency/EBV-associated lymphoproliferative disorder (LPD), Hodgkin type. The relatively small caliber of the CD30 + immunoreactive blastoid cells in the lymph nodes suggested reactive immunoblasts rather than Hodgkin cells. The morphologic aspects of the lymph node biopsies with predominance of T-cells were compatible with the diagnosis of a sulfasalazine-induced DRESS syndrome as the patient had a high RegiSCAR score for DRESS. [DRESS Syndrome Foundation: Diagnosis and Treatment. (2023)] The patient’s complex clinical course, marked by two hospital admissions, highlights the challenges in diagnosing and managing DRESS. This case underscores the need for individualized care, close patient monitoring, and further research to better understand DRESS’s underlying mechanisms and optimal therapeutic strategies.

Lymphadenopathy Tissue Sampling by EUS-Guided Fine-Needle Biopsy Contributes to Meeting the Conditions for Genomic Profiling.

EUS-guided fine-needle biopsy (EUS-FNB) performed with a Franseen needle or Fork-tip needle enables greater tissue acquisition. However, it is unknown whether EUS-FNB could contribute to lymphadenopathy genomic profiling. The aim of this study was to determine the efficacy of EUS-FNB using a Franseen or Fork-tip needle for tissue acquisition and genomic profiling in patients with lymphadenopathy. Patients with abdominal lymphadenopathy who underwent EUS-guided fine needle aspiration (FNA)/EUS-FNB were included in this study. The amount of acquired tissue and its suitability for genomic profiling were compared between FNA and FNB. Specimen quality was evaluated by a widely used pathologic adequacy scoring system (0: insufficient; 1 to 2: cytologic; 3: limited histologic; 4 to 5: sufficient histologic). The criteria of FoundationOne CDx (F1CDx) and NCC Oncopanel (NOP) were used to assess the suitability for genomic profiling. In total, 72 patients underwent EUS-FNA, and the other 20 patients underwent EUS-FNB. The pathologic adequacy score and suitability for genomic profiling based on the criteria were significantly higher for FNB than for FNA [histologic adequacy score: 5 (4 to 5) versus 3 (0 to 5), P<0.01; F1CDx: 16.7% vs. 0%, P=0.01; NOP: 66.7% vs. 7.5%, P<0.01]. In multivariate analysis, EUS-FNB was identified as the only factor that influenced the suitability for genomic profiling based on the above-mentioned criteria (odds ratio 19.5, 95% CI: 3.74-102, P<0.01). EUS-FNB performed using Franseen or Fork-tip needles may result in greater lymphadenopathy tissue acquisition and thus enhanced suitability for genomic profiling compared with EUS-FNA.

Endoscopic Ultrasound-Guided Liver Biopsy (EUS-LB): An Endoscopic Solution to the Unmet Needs of Liver Tissue Acquisition and Beyond

Abstract Aim and Objective Endoscopic ultrasound-guided liver biopsy (EUS-LB) is now increasingly being used across the globe as a method of liver tissue acquisition. This method is widely accepted by many professionals as it can overcome many shortcomings of percutaneous liver biopsy and transjugular liver biopsy. The aim of the study is to obtain the adequate and optimal biopsy rate associated with EUS-LB. Materials and Methods This is a retrospective observational study. Consecutive patients undergoing EUS-LB during the study period who were willing to consent were taken up for the study. Results Total 91 patients were taken up for the study. Median age of study population was 44 years out of which 39 patients were males and 52 were females (42.9 and 57.1%). Adequate biopsy rate (according to European Association for the Study of Liver Disease criteria) and optimal biopsy rate (according to American Association for the Study of Liver Diseases criteria) were 89 (81/91) and 60.4% (55/91), respectively. Rate of conclusive diagnosis was 95.6% (86/91). The commonest diagnosis encountered was nonalcoholic steatohepatitis) (23, 25.3%), followed by autoimmune hepatitis (17, 18.7%). Additional diagnostic information was obtained by endosonography during EUS-LB in 21 patients (23.1%). Gallstone disease was found in four (4.8%) patients, chronic calcific pancreatitis in two (1.9%) patients, significant abdominal lymphadenopathy defined as lymph node more than 1.5 cm in five (5.8%) patients, and esophageal or gastric varices in ten (10.6%) patients. One case of self-limiting biopsy site ooze was seen in EUS-LB and the patient was having cirrhosis. Conclusion This study showed a high diagnostic outcome and safety profile with EUS-LB technique. EUS-LB can achieve excellent histological yield when performed with optimal technique. Moreover, it is possible to obtain additional information during the procedure from diagnostic endosonography that is done as a part of EUS-LB.

Pseudokidney Sign in Gastric Cancer.

Pseudokidney sign (PKS) is a characteristic sonographic finding of an abnormal mass with a reniform appearance, and a hyperechoic central region surrounded by a hypoechoic area. It has been seldom documented in gastric cancer. A 75-year-old male patient presented with a palpable abdominal resistance in the left upper abdominal quadrant and ultrasound evaluation revealed a well-vascularized mass presenting with PKS. Regional lymphadenopathy was also found, and the working diagnosis of gastric cancer was established. The suspected diagnosis was later verified endoscopically and on pathohistological examinations as gastric adenocarcinoma. Computed tomography staging also revealed distant metastases to the lungs, liver, and adrenal glands and abdominal lymphadenopathy. The PKS often indicates gastrointestinal pathology, and it may be seen in benign and malignant conditions due to gastrointestinal wall thickening. Therefore, additional diagnostic examinations are advised for a more definite diagnosis.

Primary appendiceal lymphoma managed through videolaparoscopic appendectomy in an Amazonian center: Case report and review of literature

Introduction: Primary gastrointestinal lymphoma is rare and appendix involvement is rarer. Most cases of appendiceal lymphoma present as clinical acute appendicitis and are generally diagnosed intra-operatory. Some computed tomography findings in preoperative evaluation may suggest appendix neoplasm rather than appendicitis, such as appendix diameter larger than 15 mm, abdominal lymphadenopathy and aneurysmal dilatation of the lumen. Case Report: We report a case of a 26-year-old Brazilian man first diagnosed with acute appendicitis and submitted to videolaparoscopic approach with appendectomy and posterior chemotherapy after histopathological reveal of primary appendiceal lymphoma. Conclusion: Surgical resection followed by chemotherapy remains as standard management choice when possible. Adequate investigation and mandatory histopathological analysis in appendectomies are fundamental for diagnosis and proper treatment.

High-grade B-cell lymphoma with a quadruple-hit genetic profile including concurrent MYC, BCL2, BCL6, and CCND1 gene rearrangements.

Several reports of concurrent MYC, BCL2, BCL6, and CCND1 rearrangements in high-grade B-cell lymphoma (HGBL) have been recently described. Herein, we aimed to delineate the scope of this entity through a review of HGBL with a "quadruple-hit" genetic profile identified at our institution. We performed a retrospective review (2015-2023) at our institution of B-cell lymphoma (BCL) cases that were evaluated with concurrent MYC, BCL2, and BCL6 break-apart and IGH::MYC and IGH::CCND1 dual-color dual-fusion fluorescence in situ hybridization studies. Of 203 cases meeting inclusion criteria, 2 (1%) with a quadruple-hit genetic profile were identified. Case 1 represented a 59-year-old female with widespread lymphadenopathy and a diagnosis of HGBL who exhibited primary refractoriness to dose-adjusted etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin, and rituximab (DA-EPOCH-R) chemotherapy. Case 2 represented a 58-year-old male with mediastinal and abdominal lymphadenopathy and a diagnosis of large BCL who died from disease after 1 cycle of DA-EPOCH-R chemotherapy. Similarly, a literature review of 7 previously reported cases of HGBL with a quadruple-hit profile also demonstrated aggressive disease behavior. Our study adds 2 new cases to the rarely encountered quadruple-hit HGBL, and a brief meta-analysis of the 9 available cases indicates aggressive disease behavior conferred by this constellation of genetic events.

Radiomics-based quantitative contrast-enhanced CT analysis of abdominal lymphadenopathy to differentiate tuberculosis from lymphoma

Radiomics-based quantitative contrast-enhanced CT analysis of abdominal lymphadenopathy to differentiate tuberculosis from lymphoma

Concurrent metastatic melanoma and follicular lymphoma diagnosis on liver fine needle aspiration and biopsy

Abstract Introduction/Objective The liver is commonly involved in metastatic neoplasms, such as colorectal, breast, esophageal, stomach, pancreatic, lung, and kidney. Fine needle aspiration (FNA) and core needle biopsy (CNB) are frequently used to establish an accurate diagnosis of liver masses. Here we report the coexistence of metastatic malignant melanoma (MM) and follicular lymphoma (FL) involving the liver. To our knowledge, This is the first reported case, and we hope to add to the literature. Methods/Case Report The patient is a 75 -year-old woman diagnosed with stage I skin melanoma in 2004, and systemic low-grade FL in 2009. She presented with fatigue, weight loss, and difficulty swallowing. CT scan showed new pulmonary, hepatic, and splenic nodules with stable abdominal lymphadenopathy. Smears from the liver nodule FNA showed numerous single tumor cells with moderate cytoplasm and eccentrically located hyperchromatic nuclei. CNB showed fragments of liver parenchyma infiltrated by metastatic melanoma and a focal portal area with expansile lymphoid aggregates of small lymphocytes. The lymphoid cells were positive for CD10, CD20, PAX5, and BCL2 and negative for CD3, CD5, BCL6 and BCL1, with a low Ki-67 proliferative index (&amp;lt;5%). Although not characteristic of an FL, this immunophenotype was most consistent with FL given her clinical history. The metastatic MM was positive for SOX10 and Melan-A, while negative for CD45 and CK AE1/AE3. Despite neoadjuvant therapy, the patient died just two months after diagnosis. Results (if a Case Study enter NA) NA Conclusion A tremendous variety of neoplasms can occur in the liver. Different morphologies under a microscope should broaden the list of differential diagnoses. Familiarizing with morphological features and using ancillary studies are essential for accurate diagnosis and proper patient management. FNA and CNB combined with ancillary studies can successfully establish the diagnosis of malignancies involving the liver.

A Curious Case of IgG4-Related Disease Masquerading As an Occult Abdominal Malignancy

Introduction IgG4-related disease is an autoimmune, fibroinflammatory disorder involving multiple organ systems in the body. Diagnosis is often suggested by strong IgG4-positive stain on tissue biopsy. Patients respond well to steroids; and in cases of relapse, steroid-sparing agents like rituximab, azathioprine or cyclosporine can be used. Case Presentation Here we present the case of a 77 year-old African-American female with a past medical history significant for cerebrovascular accident, chronic hepatitis C infection status post treatment with antivirals, left chronic dacryoadenitis and chronic iron-deficiency anemia. She presented to the hematology office for a follow-up visit for chronic anemia and complained of new onset right upper quadrant pain and unexplained weight loss. Subsequent abdominal and pelvic imaging showed hypoechoic lesion in pancreatic head along with multiple epigastric, omental and gastro-hepatic nodules/lymph nodes suspicious for malignancy. This was followed by esophagogastroduodenoscopy (EGD) with endoscopic ultrasound (EUS) which revealed gastric body/antral erosions along with multiple enlarged abdominal lymph nodes and heterogeneous, hypoechoic irregular mass in the pancreatic head/neck, all of which were biopsied. While awaiting biopsy results, due to high suspicion for malignancy, PET-FDG was performed which showed hypermetabolic lesions corresponding to the EUS findings and a FDG-avid right paravertebral mid thoracic soft tissue mass favored to represent a neurogenic tumor, unrelated to abdominal disease. Laboratory work-up was significant for elevated IgG levels with particularly high titres of IgG subclass 4. Biopsy results from EUS showed no evidence of malignant cells, no clonal proliferation on flow cytometry in either the lymph nodes or pancreatic tissue. Cytology of pancreatic tissue biopsy revealed lymphoid infiltration, extensive edema and fibrosis with no malignant cells. Immunohistochemistry studies revealed strong positive stain for IgG4 suggestive of IgG4-related disease. Of note, the patient had been following up with ophthalmology for a few years for chronic inflammatory dacryoadenitis of the left eye, which can likely be attributed to IgG4-related disease. After shared-decision making with the patient regarding treatment options, treatment was initiated with a plan of rituximab for 4 cycles along with corticosteroid. As of this abstract submission, the patient received one dose of rituximab, pending assessment of response to treatment. Discussion Heterogeneous pancreatic masses on abdominal imaging should be considered malignant until otherwise proven. In cases where these masses are associated with diffuse abdominal and pelvic lymphadenopathy, definitive diagnosis is made through obtaining biopsies from the easily accessible sites. A benign, auto-immune, fibroinflammatory condition which mimics this presentation is IgG4-related disease, whose treatment is far less complicated than a metastatic carcinoma. It often involves lacrimal glands. A high index of suspicion backed by the biopsy results showing strong stain affinity to IgG4 and elevated blood IgG4 levels are required. Treatment involves initiation of steroids followed by steroid-sparing agents like azathioprine, cyclosporine or rituximab in cases of relapse after steroid induction. However, as in our case, combination therapy of simultaneous use of steroids with rituximab can be used after shared decision making with patients.

Computer-Aided System Based on Deep Learning for Lymph Node Lesions Diagnosis in CT Images from Abdominal Lymphadenopathy Patients

Computer-Aided System Based on Deep Learning for Lymph Node Lesions Diagnosis in CT Images from Abdominal Lymphadenopathy Patients

Diagnostic role of endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA) in abdominal lymphadenopathy of unknown etiology.

Endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) is an established method for the evaluation of abdominal organ lesions. However, there are few studies on EUS-FNA for abdominal lymph node (LN) lesions. The purpose of this study was to evaluate the diagnostic role of EUS-FNA in isolated abdominal lymphadenopathy (LAP). A retrospective analysis was performed on patients with isolated abdominal LAP who underwent a EUS-FNA examination. The diagnosis was made based on cytology, histology, and immunohistochemical (IHC) studies. The area under curve (AUC) value, sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), and accuracy were calculated. A total of 99 patients were included in this study. The final diagnoses were metastatic tumor (n = 32), lymphoma (n = 32), tuberculosis (n = 17), sarcoidosis (n = 5), castleman's disease (n = 1), and reactive LAP (n = 12). The AUC value, sensitivity, specificity, PPV, NPV, and accuracy of EUS-FNA in the diagnosis of malignant LAP were 0.9531, 90.6, 100, 100, 85.4, and 93.9%, respectively. For the diagnosis of lymphoma, the accuracy of EUS-FNA combined with IHC staining was 94.9%. Retroperitoneal LN enlargement is more commonly associated with lymphoma, while hepatic hilar LN enlargement predominantly suggests benign conditions or metastatic tumors. Malignant lymph nodes are more likely to be regular border, circular/quasi-circular, and fusion. Lymphomas are more likely to present with fusion and heterogeneous echogenicity than metastatic tumors. EUS-FNA is a safe and effective method to diagnose isolated abdominal LAP.

Diagnostic Accuracy and Safety of Endoscopic Ultrasound-Guided End-cutting Fine-needle Biopsy needles for tissue sampling of Abdominal and Mediastinal Lymphadenopathies: a prospective multicenter series.

The role of the newer EUS-fine needle biopsy (FNB) needles in lymphadenopathies (LA) is still under evaluation. We aimed to evaluate the diagnostic accuracy and the adverse event rate of EUS-FNB in diagnosing LA. From June 2015 to 2022, all patients referred to 4 institutions for EUS-FNB of mediastinal and abdominal LA were enrolled. 22G Franseen tip or 25G Fork tip needles were used. The gold standard for positive results was surgery or imaging and clinical evolution over a follow-up of at least one year. A total of 100 consecutive patients were enrolled, consisting of those with a new diagnosis of LA (40%), presence of LA with a previous history of neoplasia (51%), or suspected lymphoproliferative disease (9%). EUS-FNB was technically feasible in all LA patients with 2 to 3 passes (mean 2.62±0.93). The overall EUS-FNB sensitivity, positive predictive value (PPV), specificity, negative predictive value (NPV), and accuracy were 96.20%, 100%, 100%, 87.50%, and 97.00%, respectively. Histological analysis was feasible in 89% of cases. Cytological evaluation was performed in 67% of specimens. There was no statistical difference between the accuracy of the 22G or 25G needle (p=0.63). A sub-analysis on lymphoproliferative disease revealed a sensitivity and accuracy of 89.29% and 90.0%. No complications were recorded. EUS-FNB with new end-cutting needles is a valuable and safe method to diagnose LA. The high quality of histological cores and the good amount of tissue allowed a complete immunohistochemical analysis of metastatic LA and precise subtyping of the lymphomas.

Scrub Typhus in Children: A Prospective Observational Study in a Tertiary Care Hospital in Eastern India.

Background Scrub typhus is a reemerging, acute, undifferentiating febrile illness and one of the most neglected tropical diseases, calling for an in-depth investigation into its clinical diversity, complications, and mortality, which drives us to carry out this research work. Methods Over a year, prospective observational research was carried out after gaining parental consent and institutional ethical clearance, 206 children of either gender aged between one month and 12 years who had been hospitalized with a fever for at least five days and subsequently tested positive forOrientia​​​​tsutsugamushiwere included in the study. Basic demographic information, clinical characteristics, laboratory findings, complications, related coinfections, and results were gathered and analyzed.A P-value of 0.05 was set as the statistical benchmark. Results The current study found that boys outnumbered girls. The ratio of boys to girls was 1.22:1, and the average age was 5.18 years. All had a fever (100%), and the other most frequently occurring clinical signs and symptoms were abdominal pain (16.99%), vomiting (22.33%), hepatosplenomegaly (49.51%), facial puffiness (39.32%), edema (27.18%), lymphadenopathy (19.90%), eschar (19.90%), macular-erythematous rash (17.96%), cough (21.84%), conjunctival congestion (25.24%), and headache (13.59%). Anemia (81.55%), leucocytosis (20.39%), leucopenia (6.8%), thrombocytopenia (49.51%), thrombocytosis (2.43%), and elevated serum levels of alanine aminotransferase (ALT, 57.28%) and aspartate aminotransferase (AST, 63.59%) were characteristic laboratory results. The coinfections were dengue, enteric fever, urinary tract infections, and malaria.Children who also had dengue were more likely to develop thrombocytopenia, which was statistically significant (P-value = 0.008). With doxycycline medication, early defervescence of fever occurred earlier than with azithromycin, and it was statistically significant (P-value = 0.000). The complications were hepatitis (63.59%), lower respiratory tract infections (LRTIs, 22.82%),scrub typhus meningoencephalitis (STME, 3.88%), acute kidney injury (AKI, 2.91%), myocarditis (1.46%), and acute disseminated encephalomyelitis (ADEM, 0.49%). Except for one who had ADEM, everyone was sent back home after receiving the best care possible. The average duration of hospital stay was 6.89 days. Conclusions Even in the absence of eschar, scrub typhus should be suspected in any febrile child who experiences clinical signs of meningoencephalitis syndrome, capillary leakage, skin rash, conjunctival congestion, LRTI, AKI, lymphadenopathy, hepatosplenomegaly, thrombocytopenia, and liver dysfunction in the post-monsoon season. Strong clinical suspicion and prompt anti-scrub drug administration go a long way in preventing or decreasing the morbidity and mortality of the same.

A convenient scoring system to distinguish intrahepatic mass-forming cholangiocarcinoma from solitary colorectal liver metastasis based on magnetic resonance imaging features

ObjectivesTo develop and validate a diagnostic scoring system to differentiate intrahepatic mass-forming cholangiocarcinoma (IMCC) from solitary colorectal liver metastasis (CRLM).MethodsA total of 366 patients (263 in the training cohort, 103 in the validation cohort) who underwent MRI examination with pathologically proven either IMCC or CRLM from two centers were included. Twenty-eight MRI features were collected. Univariate analyses and multivariate logistic regression analyses were performed to identify independent predictors for distinguishing IMCC from solitary CRLM. The independent predictors were weighted over based on regression coefficients to build a scoring system. The overall score distribution was divided into three groups to show the diagnostic probability of CRLM.ResultsSix independent predictors, including hepatic capsular retraction, peripheral hepatic enhancement, vessel penetrating the tumor, upper abdominal lymphadenopathy, peripheral washout at the portal venous phase, and rim enhancement at the portal venous phase were included in the system. All predictors were assigned 1 point. At a cutoff of 3 points, AUCs for this score model were 0.948 and 0.903 with sensitivities of 96.5% and 92.0%, specificities of 84.4% and 71.7%, positive predictive values of 87.7% and 75.4%, negative predictive values of 95.4% and 90.5%, and accuracies of 90.9% and 81.6% for the training and validation cohorts, respectively. An increasing trend was shown in the diagnostic probability of CRLM among the three groups based on the score.ConclusionsThe established scoring system is reliable and convenient for distinguishing IMCC from solitary CRLM using six MRI features.Clinical relevance statementA reliable and convenient scoring system was developed to differentiate between intrahepatic mass-forming cholangiocarcinoma from solitary colorectal liver metastasis using six MRI features.Key Points• Characteristic MRI features were identified to distinguish intrahepatic mass-forming cholangiocarcinoma (IMCC) from solitary colorectal liver metastasis (CRLM).• A model to distinguish IMCC from solitary CRLM was created based on 6 features, including hepatic capsular retraction, upper abdominal lymphadenopathy, peripheral washout at the portal venous phase, rim enhancement at the portal venous phase, peripheral hepatic enhancement, and vessel penetrating the tumor.

#5430 ANTI-PLA2R ASSOCIATED MEMBRANOUS NEPHROPATHY AND CHRONIC LYMPHOCYTIC LEUKAEMIA, IS THERE A LINK?

Abstract Background and Aims The spectrum of glomerular lesion that occurs in the context of plasma cell dyscrasias (PCD) is wide, including type I and type II membranoproliferative glomerulonephritis (MPGN), focal proliferative GN, membranous nephropathy (MN), minimal change disease and amyloidosis. A clear-cut link between hematological dyscrasias and glomerulopathy is often offered by the finding of paraproteins in the kidney or by kidney leucocytic infiltration and, in these scenarios, malignancy treatment is often found to be also effective towards the glomerulopathy. In the context of PCD in which proliferation of the B-cell clone does not lead to plasma-cell terminal differentiation such as chronic lymphocytic leukemia (LLC) the physiopathological link with glomerulonephritis is not well established and it's hard to determine whether the combination of these two disorders is fortuitous. With regarding to MN, it's now well established that this histopathological pattern must be related to the presence of circulating autoantibodies, where anti-PLA2R auto-antibodies usually been associated with primary MN. To our knowledge association between LLC and anti-PLA2R associated MN has not been described. Case report We report the case of a 76-year-old man who was admitted in February 2022 with anasarca and acute kidney injury. He had recently been evaluated by a hematologist in the context of suspected chronic lymphocytic leukemia (CLL). Laboratory findings, along with typical CLL white blood cell count, showed decreased kidney function from baseline and were compatible with nephrotic syndrome, with proteinuria progressively increasing up to 27 gr/24 h in the following weeks. CLL was confirmed by lymphocyte typing. A total body CT scan showed mediastinal, abdominal and inguinal lymphadenopathies, but no suspected neoplastic lesions. Screening for autoimmunity tested completely negative, as well as infectious screening for common hepatic viruses (HBV, HAV, HCV), EBV, CMV, HIV, Parvovirus B19. Lymph node biopsy showed no aspects of aggressive lymphoproliferative disease and hematological consultation only indicated clinical and laboratory follow-up of LLC, with no further indication to specific therapies. A kidney biopsy was performed showing diffuse thickening of the basement membrane (Fig. 1) with granular deposition of IgG (+++) and C3 (++) along the glomerular capillary walls at immunofluorescence. Electron microscopy revealed subepithelial immune deposits (Fig. 2) with initial incorporation from the glomerular basement membrane (GBM). Anti PLA2R antibodies, found negative at first determination, subsequently tested positive at high titre (ELISA 1356.4 U/ml). A diagnosis of stage two membranous nephropathy was made, and the patient was treated with Rituximab 375 mg/m2 every week for four weeks. Clinical stability was obtained with repeated intravenous administration of diuretics (furosemide 60 mg + canrenone 200 mg) along with albumin. Nevertheless, nephrotic syndrome persisted, and kidney function progressively decreased with serum creatinine up to 2.8 mg/dL. Immunophenotype, which showed reduction but no suppression of CD19+ lymphocytes, was again repeated on November 2022 and showed typical LLC findings (CD 19+, CD 20+, CD 23+, CD 5+) with repopulation of CD19+ lymphocytes. Anti PLA2R antibodies gradually decreased instead, reaching a value of ELISA 1.8 U/ml in November 2022 and persisted negative until present. The patient recovered from nephrotic syndrome in Autumn 2022 and is currently on stage G3B chronic kidney disease with persistent sub-nephrotic proteinuria in supportive treatment. Conclusion Simultaneous diagnosis of primary MN and LLC led us to ask ourselves whether this patient could benefit from LLC-specific chemotherapy, even though there were no signs of aggressive hematological disease and no clear link with the nephropathy. We think that further investigations are required to deeply understand the connection between hematological and renal disorders, in order to identify whether there are two diseases to treat separately or two sides of the same disease to treat as one.