\receivedDD MMMM YYYY \acceptedDD MMMM YYYYKey Clinical Message:Whipple’s disease is a rare, multisystem infection caused byTropheryma whipplei , most commonly seen in European males. We report a rare case of Whipple’s disease in a Mongolian Nepali male presenting with chronic diarrhea but without the classic rheumatologic symptoms. Histopathological analysis of the small intestinal biopsy samples revealed periodic acid-Schiff (PAS)-positive macrophages, confirming the diagnosis. The patient had received empirical corticosteroid treatment before diagnosis, which led to a decline in their clinical condition. He was effectively treated with ongoing antibiotics. This case highlights the risk of mismanagement and the diagnostic difficulty of atypical Whipple’s disease presentations and emphasizes the possible risks of starting immunosuppressive treatment before receiving a diagnosis.Keywords: Chronic diarrhea, Intestinal lipodystrophy, Whipple’s disease

Title:Cotyledonoid leiomyoma: a case report and review of the literature

A Case of Acral Necrosis Caused by Septic Shock and Literature ReviewGuoping Zhou, Jing Li, Heliang Wu, Ping Li, Yujun LouAuthor Affiliation: Department of Critical Care Medicine, Gaochun People’s Hospital, Nanjing, Jiangsu, 211300Corresponding Author: Guoping Zhou, Email: zgp8508@sina.comKey words : septic shock; acral necrosis; disseminated intravascular coagulation; microcirculatory disturbance; continuous renal replacement therapy[Abstract] Acral necrosis in septic shock signals microvascular thrombosis and poor prognosis. Vigilant extremity examination is critical for early detection. Prompt multidisciplinary intervention (anticoagulation + critical care support) may salvage tissue viability and reduce amputation risk.1 | IntroductionSepsis is a common condition in the Intensive Care Unit (ICU), and septic shock is the leading cause of death among critically ill patients in the ICU, with a mortality rate as high as 20∼30%.[1]Acral necrosis is a rare complication of septic shock, thought to be associated with factors such as microcirculation disorders, disseminated intravascular coagulation (DIC), and the administration of high-dose vasoactive drugs.[2, 3]This paper analyzes the clinical characteristics and treatment process of a patient with peripheral necrosis in all four acrals, aiming to enhance clinicians’ understanding of acral necrosis associated with sepsis.2 | Case Presentation/Examination2.1 Admission History and Physical ExaminationThe patient is a 59-year-old female with a history of ’hypertension and type 2 diabetes,’ who has been on long-term oral antihypertensive medication and insulin injections (specific drugs and dosages are not detailed). She was admitted to the Nephrology Department of Gaochun People’s Hospital, Nanjing, at approximately 21:40 on June 21, 2024, due to ’abdominal pain accompanied by fever for one day.’ The patient experienced abdominal pain without an obvious cause a day prior, followed by chills and shivering, without measuring body temperature, chest tightness, shortness of breath, nausea, vomiting, frequent urination, urgency, or dysuria, and did not seek immediate medical attention. Subsequently, her body temperature rose, although not measured, she felt fatigued, with generalized pain unrelieved by rest, prompting her to visit the emergency department. Blood glucose was indicated as ’Hi’ and the patient vomited once during the emergency visit, with the vomitus being gastric contents. Upon admission, physical examination revealed: body temperature: 39.0°C, pulse: 113 beats/min, respiration: 24 breaths/min, blood pressure: 81/53 mmHg, consciousness was clear but in poor spirit, assuming a supine position, and was uncooperative during examination; breath sounds in both lungs were clear without dry or moist rales and wheezing sounds, heart rhythm was regular, no pathological murmurs were heard in the auscultation areas of the valves; the abdomen was flat, percussion of both kidney areas was tender without rebound pain, no shifting dullness was detected, bowel sounds were normal, averaging 4 times/min.2.2 Auxiliary ExaminationsBlood cell analysis + hsCRP (June 21, 2024): White blood cell count: 4.67×10 9/L, Neutrophils: 86.7%, Hemoglobin: 114g/L, Platelet count: 92×10 9/L, hsCRP: 155.30mg/L; Biochemistry (June 21, 2024): Aspartate aminotransferase: 49.8U/L, Urea: 10.54mmol/L, Uric acid: 398.6umol/L, Sodium: 133.5mmol/L, Chloride: 97.8mmol/L, Alanine aminotransferase: 48U/L, Lactate: 5.28mmol/L; Plasma D-dimer (June 21, 2024): 15.33mg/L; Arterial blood gas analysis (June 21, 2024): pH: 7.429, Partial pressure of oxygen: 69.00mmHg, Partial pressure of carbon dioxide: 28.90mmHg, Oxygen saturation: 94%, Standard bicarbonate: -5mmol/L, Actual bicarbonate: -3.1mmol/L, Total carbon dioxide: 20.00mmol/L, Antibodies for epidemic hemorrhagic fever virus (IgG\IgM), 2019-nCoV antigen, and influenza A and B virus antigens (June 21, 2024) all negative. Chest and abdominal CT scan with contrast (June 21, 2024): Multiple small nodules in both lungs, mild fatty liver, calcification in the right liver lobe, a nodular lesion at the gallbladder base, possible polyp; other possibilities include dilation and fluid accumulation in the right renal pelvis and upper ureter, thickened and enhanced ureteral wall, fluid in the right paracolic gutter, roughness and exudation around both kidneys, thickened left adrenal gland, suspected lipomatosis; slightly thickened bladder wall, a small amount of fluid in the pelvic cavity, and varicose expansion of both ovarian veins.2.3 Initial Admission Diagnosis and Treatment MeasuresPreliminary Diagnosis: 1. Acute Pyelonephritis, 2. Septic Shock, 3. Type 2 Diabetes Mellitus with Poor Glycemic Control, 4. Hypertension, 5. Hyperlactatemia. Upon admission, the patient was monitored for vital signs and treated with piperacillin/tazobactam 4.5g IV infusion for infection control, fluid resuscitation, and norepinephrine to raise blood pressure. Further tests were performed: Coagulation profile (Emergency) (June 21, 2024): Prothrombin time: 16.6 sec, Prothrombin time ratio: 1.44, Activated partial thromboplastin time: 40.1 sec, D-dimer: 19.75 mg/L, Antithrombin III: 68.7%. Procalcitonin test (June 21, 2024): >100.0 ng/ml, Lactate: 10.12 mmol/L.2.4 Disease Progression and Diagnosis and TreatmentAfter admission, the patient gradually became drowsy, confused, and experienced difficulty maintaining blood pressure. Following a consultation with the intensive care department, the patient was transferred to the intensive care unit for further treatment at approximately 23:50. Upon entering the unit, the patient was in a drowsy state with poor mental status, with blood pressure maintained at 94/42 mmHg through metaraminol at 5 mg/h, and a heart rate of 136 beats per minute. Auscultation revealed coarse breath sounds in both lungs without noticeable dry or wet rales, a regular heart rhythm, no significant murmurs in the valve auscultation areas, a soft abdomen without tenderness or rebound tenderness, no significant percussion pain in the bilateral renal areas, and no swelling in both lower acrals. The admission diagnosis was: 1. Sepsis (urinary tract), 2. Septic shock, 3. Acute pyelonephritis, 4. Acute kidney injury (KDIGO stage 2), 5. Disseminated intravascular coagulation, 6. Type 2 diabetes with poor blood glucose control, 7. Hypertension, 8. Hyperlactatemia. Treatment included monitoring vital signs, administering meropenem (1.0g every 8 hours) via intravenous drip for anti-infection, fluid resuscitation (2000ml of crystalloids), low-dose metaraminol (3 to 5 mg/h) to maintain blood pressure at 90-110/50-60 mmHg, among other treatments.On the second day of admission (June 22, 2024), further re-examination of blood cell analysis (emergency) was conducted: White blood cell count: 18.24×10^9/L, neutrophil percentage: 93.6%, platelet count (manual): 40.0×10^9/L. Coagulation routine (emergency): Prothrombin time: 25.6 seconds, prothrombin time ratio: 2.28, coagulation time: 23.0 seconds, partial thromboplastin time: 54.6 seconds, fibrinogen: 1.15 g/L, D-dimer: 188.50 mg/L. Blood gas analysis: pH: 7.326, partial pressure of carbon dioxide: 30.5 mmHg, partial pressure of oxygen: 114 mmHg, total hemoglobin: 12.1 g/dl, calcium ion: 1.13 mmol/L, chloride ion: 108 mmol/L, lactate: 4.1 mmol/L, actual base excess: -8.9 mmol/L, standard base excess: -10.1 mmol/L, standard HCO3: 17.3. Biochemical combination: Albumin: 26.7 g/L, aspartate aminotransferase: 73 U/L, lactate dehydrogenase: 852 U/L, urea: 13.68 mmol/L, creatinine: 195.8 μmol/L, glomerular filtration rate: 23.7 ml/min, glucose: 10.54 mmol/L, total carbon dioxide: 14.5 mmol/L, C-reactive protein: 226.12 mg/L. Re-examination of CT showed right renal pelvis dilatation with fluid accumulation and contrast agent retention.On June 22, 2024, at approximately 3:30 PM, the patient experienced a drop in blood pressure and heart rate, and lost consciousness. Epinephrine was administered intravenously, followed by tracheal intubation and mechanical ventilation. The patient regained spontaneous cardiac rhythm after 5 minutes. Vasoactive drugs (Norepinephrine 0.2 µg/kg/min) were then administered to maintain blood pressure above 120/60 mmHg. Considering the patient’s severe sepsis, acute kidney injury, and acidosis, continuous renal replacement therapy (CRRT) was initiated. The presence of disseminated intravascular coagulation (DIC) was noted (changes in coagulation indicators after admission are shown in Figure 1), and component blood transfusion and treatments to improve coagulation were administered. A dynamic re-examination of the urinary system via CT indicated obstruction of the right renal ureter. After repeated consultations with the urology department, a double J stent was placed on the right side on June 25, 2024 (changes in hydronephrosis of the right renal pelvis and ureter before and after stent placement are shown in Figure 2). Through treatment, the patient’s circulation gradually stabilized. On June 26, 2024, vasoactive drugs were discontinued, and enteral nutrition support was initiated. On June 27, 2024, daily awakening began, with the patient showing some agitation. By June 29, 2024, the patient regained consciousness and respiration stabilized. On July 2, 2024, the tracheal tube was removed, and oxygen was administered via double nasal cannula. The patient exhibited weak acral strength, and rehabilitation treatment was sought from the rehabilitation department. On July 16, 2024, the patient began to urinate, producing 250 ml, with gradual increases. On July 18, 2024, regular hemodialysis was started every other day, and the frequency of dialysis was gradually reduced as urine output increased. By July 27, 2024, urine output reached approximately 1500 ml, and hemodialysis was discontinued.On June 23, 2024, the patient began to exhibit cyanosis in the extremities, which progressively worsened to blackening and gangrene. Consultations were held with the orthopedics, vascular surgery, and interventional departments. Comprehensive vascular ultrasound and DSA revealed no arterial or venous embolism. Subsequently, treatments such as papaverine and alprostadil were administered to enhance peripheral circulation, and the local necrotic areas were managed with enhanced dressing changes to prevent infection.On August 2, 2024, the patient was transferred to the nephrology department for ongoing treatment. During this period, the necrosis and gangrene of the fingers and toes gradually stabilized. The patient received treatments such as blood sugar control and polystyrene oxide capsules to improve renal function. The patient experienced recurrent urinary tract infections, which improved with anti-infective treatment. On August 24, 2024, the patient was discharged for recuperation. On September 16, 2024, the patient returned to our hospital’s urology department to have the double-J stent removed. Subsequently, the patient sought treatment at several external hospitals for peripheral necrosis, where amputation of fingers and toes was recommended. On September 28, 2024, the patient underwent fingertip amputation surgery at the orthopedics department of Gaochun People’s Hospital, Nanjing, with satisfactory postoperative recovery. On November 2, 2024, the patient was discharged. Changes in peripheral necrosis are shown in Figure 3-1 to Figure 3-3. The patient continues regular follow-up visits to the orthopedics and nephrology departments at Gaochun People’s Hospital, Nanjing, and is able to manage daily life independently.

Cutaneous Clues to a Silent Malignancy: Tripe Palm and Malignant Acanthosis Nigricans Unveiling Gastric Adenocarcinoma

A document by Jad Kabbara. Click on the document to view its contents.

Background: Angioleiomyoma is a rare benign tumor originating from vascular smooth muscle. This paper aims to describe the clinicopathologic features and management of nasal angioleiomyoma, emphasizing its rarity and diagnostic challenges. Methods: A 73-year-old male presented to the Otolaryngology-Head and Neck Surgery with chronic right nasal obstruction and facial pressure. Physical examination

IntroductionIgG4-related cholangiopathy , also referred to asIgG4-associated cholangitis (AIC) or IgG4-related sclerosing cholangitis (IgG4-RSC) , is an immune-mediated inflammatory disease of unknown etiology. It is characterized by elevated serum IgG4 levels, dense infiltration of IgG4-positive plasma cells, and histopathologic features such as storiform fibrosis and/orobliterative phlebitis affecting the bile ducts.1This condition represents a hepatobiliary manifestation ofIgG4-related disease (IgG4-RD) , a systemic fibroinflammatory disorder with potential multi-organ involvement, including the pancreas, salivary glands, kidneys, and retroperitoneum. The concept of IgG4-RSC as a benign form of biliary stricture was proposed by Kamisawa et al.2Biliary involvement in IgG4-RD, collectively termed IgG4-related cholangiopathy, most commonly presents in association with type 1 autoimmune pancreatitis (AIP) . However, in cases where no concurrent pancreatic disease is present, the condition is referred to asisolated IgG4-RSC . This isolated form is particularly challenging to diagnose, as it often mimics perihilar cholangiocarcinoma both clinically and radiologically, despite requiring varied treatment approaches.Clinically, IgG4-RSC may present with biliary strictures and/or mass-like lesions , making it difficult to distinguish fromprimary sclerosing cholangitis (PSC) or biliary tract malignancies. 3 Timely and accurate diagnosis is essential, as cholangiocarcinoma typically requires surgical intervention, whereas IgG4-RSC responds well tocorticosteroid therapy , which remains the consensus first-line treatment globally.2We report a case that was initially suspected as a case of biliary malignancy, but subsequently diagnosed as IgG4-related cholangiopathy after responding well to the corticosteroid therapy. This case highlights the importance and challenges of considering IgG4-SC in the differential diagnosis of biliary strictures and mass-forming lesions, and the diagnostic dilemma when two mimickers create confusion.

IntroductionCemento-ossifying fibroma (COF), as per the World Health Organization’s (WHO) 2022 classification of head and neck tumours, is a well-demarcated neoplasm which consists of fibrous tissue containing varying amounts of mineralised material resembling bone and/or cementum (1). It is a benign fibro-osseous lesion, and is derived from the multipotential mesenchymal blast cells of the periodontal ligament (2).COF was already defined as a benign mesenchymal odontogenic tumour in the 2017 WHO classification. However, it was described under the category of fibro-osseous lesions (3), but is now an integral part of the benign mesenchymal odontogenic tumours in the 2022 classification, completely isolated from the non-odontogenic juvenile trabecular (JTOF) and psammomatoid types (POF) (1).COFs occur mostly in between the second and fourth decades of life with a definitive female predilection in the ratio of 4:1 (4). However, they may be present in children and adolescents, as well as in older adults (5,6). The mandible – preferentially in the molar region – is more involved than the maxilla (7), albeit other cranial and facial bones such as the frontal, periorbital, sphenoid, ethmoid and temporal bones are also affected (8). The tumour is termed central COF when lesions are located intraosseously, whereas peripheral COF refers to extraosseous lesions, or those appearing on the outer soft tissue (9). The tumour is well demarcated by a teeny sclerotic shell of bone. Unpredictably, it may either show a slow growth or appear to be locally aggressive with cortical damage and involvement of several nearby anatomical structures (10).Histologically, the neoplasm is composed of a proliferating fibrous tissue and osseous particles containing bony strands and cementum-like material (5). This lesion is thought to occur in reaction to low-grade irritations such as trauma, calculus, plaque, masticatory forces, ill-fitting dentures and poor-quality restorations (11).Clinically, OF presents as a spherical or ovoid, generally slow-growing, painless and expansive jaw bone mass that may displace the roots of adjacent teeth, and occasionally causes facial deformity and/or functional discomfort (12). Definitive diagnosis requires correlation of clinical, radiographic, and histopathological findings (13). The treatment consists of surgical excision with enlarged resection depending on the size and location of the lesion (10,14).For an optimal management of COF, an accurate diagnosis is necessary. Clinical presentation is not unique, and the radiological patterns depend on the degree of mineralisation of the tumour. The clinician is then confronted by the overlapping patterns of the broad spectrum of fibro-osseous tumours, and the presence of unusual patterns makes the diagnosis even more challenging. We present a rare case of COF in a 22-year-old female patient in the lateral maxilla, which is an unusual site of occurrence.Case reportA 22-year-old female patient was referred to the Department of Oral and Maxillofacial Surgery with a painless swelling in the upper right region of the jawbone since 10 years. The swelling sprouted as a small painless nodule when the patient was 12, and grew with time. As the swelling was increasing, only the vision was shielded, but the deglutition and breathing were not affected. Mastication on the right side and speech were also affected. No pain, fever, or any comorbidity were reported. There was no history of previous similar swelling, toothache, numbness, trauma, surgery, or radiotherapy. Although the patient was from a low socio-economic status, her parents and siblings were all well. There was no similar swelling in the family.The patient’s general condition was otherwise good with fair nutritional status. Extraoral examination showed a bulky, dome-shaped, and well-defined swelling extending over the right hemi-maxilla with a frontward expansion causing facial disfigurement. The dome of the swelling was budding and ulcerated. The palpation of the swelling revealed a bony hard consistency, with no tenderness elicited. The mass was immobile on mobilisation and measured approximately 40 cm × 12 cm in size (figure 1). On intraoral examination, all the teeth of the right hemi-maxilla were buried under the swelling.There was no palpable lymphadenopathy. Neurological examination, as well as respiratory, digestive, and urogenital explorations did not reveal any abnormality.A maxillofacial computed tomography (CT) was performed, and revealed a well-circumscribed exuberant mass in the right facial region, spanning from the right palatal and maxillary bones and extending into the right malar bone. Detailedly, an osteoforming excrescence with a regular margin, and a mixed density content made of soft tissue and diffuse scattered calcified foci, with teeth 21 to 28 engulfed in the mass (figure 2). Also, CT image showed a mass effect of the tumour causing left deviation of the nasal septum, with a narrowed osteo-meatal system, yet preserving its anatomical structure (figure 3).A provisional diagnosis of osteoblastoma was recorded, with fibrous dysplasia and ossifying fibroma being considered in the differential diagnosis.An excisional biopsy (16 × 10 cm) of the tumour was performed, and the histopathological examination revealed a well-circumscribed fibrous tissue containing dispersed foci of calcification. The mass harboured dense bundles of fibrocollagenous connective tissue in a cellular stroma (figure 4). The matrix was formed by interconnected trabeculae of mature lamellar bone with fibroblastic rimming (figure 5). No atypical cells nor mitosis were observed, and the lesion also showed an ulcerated stratified squamous epithelium. On the basis of histopathological findings, and correlating them with clinical and radiological features, a final diagnosis of (peripheral) cemento-ossifying fibroma was established, excluding JTOF and POF subtypes.As management, a surgical resection of the tumour was performed, followed by curettage. Postoperative period was uneventful, and a clinical follow-up plan was established for review after 3 months, 6 months, and 1 year after surgery.

Long-term survival benefit from first-line immunotherapy followed by BRAF/MEK inhibition in advanced NSCLC withBRAF V600E and PD-L1 high expressionFangfang Gao 1, Zhongkang Li 2, Huanhuan Wang 2, Feng Du 3, Pengfei Cui 1, Yanqiu Zhao 1,*1 Department of Internal Medicine, The Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, Henan, China.2 Geneplus-Beijing, Beijing 102206, China.3 Department of Radiology, The Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, Henan, China.* Corresponding authors: Yanqiu ZhaoDepartment of Internal Medicine, The Affiliated Cancer Hospital of Zhengzhou University, Henan Cancer Hospital, Zhengzhou, Henan, China.mail address: zlyyzhaoyanqiu0366@zzu.edu.cnFangfang Gao, Email: zlyygaofangfang4204@zzu.edu.cnZhongkang Li, Email: lizhk@geneplus.org.cnHuanhuan Wang, Email: wanghh1997@126.comFeng Du, Email: 2564641945@qq.comPengfei Cui, Email: cuipf20@mails.jlu.edu.cnYanqiu Zhao, Email: zlyyzhaoyanqiu0366@zzu.edu.cn

A document by Qing Wang. Click on the document to view its contents.

Title:

A document by DIBYAJYOTI DEKA. Click on the document to view its contents.

Leukemic Arthritis Mimicking Juvenile Idiopathic Arthritis: A Diagnostic Challenge in Pediatric Oncology

AbstractBilateral femoral hernias are exceptionally rare in young males, with few cases documented. They are often overlooked due to their low incidence and non-specific presentation. Accurate diagnosis can be challenging, particularly in younger patients without typical risk factors. A healthy male in his 40s presented with a right groin bulge and intermittent discomfort. Physical examination revealed a right-sided irreducible hernia and a left-sided cough impulse. Initial ultrasound was inconclusive. Dynamic MRI with Valsalva confirmed bilateral femoral hernias, with two hernial sacs on the right and one on the left. The patient underwent elective laparoscopic transabdominal preperitoneal (TAPP) repair of both hernias using mesh. This case underscores the rarity of bilateral femoral hernias in young males and highlights the importance of imaging—particularly dynamic MRI—in evaluating atypical groin symptoms. The TAPP approach enabled direct visualization and simultaneous repair of both defects. To our knowledge, this is the first reported case of a young male with bilateral femoral hernias managed using laparoscopic TAPP. Bilateral femoral hernias should be considered in young males presenting with unexplained groin symptoms. Dynamic MRI is a valuable diagnostic tool, and the TAPP approach offers a safe and effective method for simultaneous bilateral repair with excellent postoperative outcomes.

A document by Amir Khanmirzaei. Click on the document to view its contents.

A document by Zhiqun Lin. Click on the document to view its contents.

IntroductionEwings sarcoma (EWS) is the second most common primary bone cancer, and develops in the axial skeleton or long bones. 1, 2Extraosseous EWS is a lesser known entity affecting the central nervous system. Primary intracranial EWS is commonly intraparenchymal and is located either in the supratentorial location or in the spinal cord. An extra-axial / extradural presentation of EWS is exceptionally rare2 and only a few cases have been previously described 3, 4.Primary intracranial Ewing’s sarcoma is malignant small round cell neoplasm, that arises from bone and soft tissue, prevalent among children and adolescents 5,6 The diagnosis of intracranial extraosseous Ewing’s sarcoma (EES) poses challenges due to the absence of specific clinical and imaging features prior to surgery. It is crucial to differentiate the tumor from other small round cell malignancies postoperativelyThis report presents a rare case of EES, discussing the clinical, neuroimaging, and differential diagnoses, and emphasizing the challenges in diagnosing such atypically located tumors.

A Case Report of Recurrent Well-Differentiated and Myxoid Giant Liposarcoma Following Lipoma Excision and Literature ReviewKuan Wang, Ping Zhang, Xuelian Chen, Min Xie, Jing Lei, Jie ZouDepartment of Plastic and Burns Surgery, The First Affiliated Hospital of Chengdu Medical College, Chengdu, Sichuan, ChinaCorrespondence to: Jie Zou; Email: 947200956@qq.com