Primary hepatic pleomorphic liposarcoma: Case report and literature review / Liposarcoma pleomorfo hepático primario: presentación de un caso y revisión bibliográfica

Primary hepatic liposarcoma is an extremely rare malignant tumour derived from adipocytes and is part of the group of mesenchymal tumours. We present the case of a 43-year-old Hispanic male patient with a pleomorphic hepatic liposarcoma and absence of MDM2 gene amplification. Two years and six months after surgery, the patient is asymptomatic. The present case is the first report of this entity with positive immunohistochemical testing for p16, p53, S100, vimentin and absence of MDM2 gene amplification. (AU)

El liposarcoma hepático primario es un tumor maligno extremadamente raro, derivado de adipocitos, y forma parte del grupo de tumores mesenquimales. Presentamos el caso de un paciente masculino de 43 años con diagnóstico de liposarcoma hepático pleomorfo con ausencia de amplificación del gen MDM2. Dos años y 6 meses después de la cirugía el paciente se encuentra asintomático. El presente caso es el primer informe de esta entidad con estudio inmunohistoquímico positivo para p16, p53, S100, vimentina y ausencia de amplificación del gen MDM2. (AU)

Bilateral multifocal chorioretinitis as the only presentation of acute West Nile virus infection: a case report.

BACKGROUND: To describe a case of bilateral multifocal chorioretinitis as the only presentation of acute West Nile virus (WNV) infection in the absence of neurological involvement. CASE PRESENTATION: A 78-year-old Italian woman was admitted to our emergency department because she noticed blurry vision in both eyes. She did not report fever, fatigue, or neurological symptoms in the last few days. Multimodal imaging showed the presence of bilateral hyperfluorescent lesions with a linear distribution, that corresponded to hypocyanescent spots on indocyanine green angiography. Antibody serology showed the presence of IgM antibodies, IgG antibodies, and ribonucleic acid (RNA) for WNV. Magnetic resonance imaging (MRI) of the brain ruled out central nervous system involvement. Three months later, the patient reported spontaneous resolution of her symptoms and remission of the chorioretinal infiltrates. CONCLUSIONS: In endemic areas, it is important to think of acute WNV infection as an explanatory etiology in cases of multifocal chorioretinitis, even without neurological involvement.

A basaloid carcinoma with multilocular thymic cyst mimicking a mediastinal teratoma.

This case report details a rare thymic basaloid carcinoma initially misinterpreted as a mediastinal teratoma, underscoring the diagnostic challenges posed by such tumors. A 71-year-old female presented with an asymptomatic anterior mediastinal tumor discovered incidentally during a routine health examination. Surgical intervention, followed by pathological and immunohistochemical analysis including CK-pan, p63, p40, and CD117 molecules, led to a definitive diagnosis of basaloid carcinoma of the thymus. This case highlights the critical importance of differential diagnosis in mediastinal lesions, especially those presenting with multilocular thymic cysts on chest CT. The subxiphoid video-assisted thoracoscopic surgery enabled complete tumor resection with minimal trauma and favorable postoperative outcomes. The patient opted against further radiotherapy or chemotherapy and she has survived for over eight months without recurrence. This case report contributes to the growing understanding of thymic basaloid carcinoma, a rare and potentially aggressive thymic carcinoma subtype. It emphasizes the necessity for precise surgical techniques and enhanced diagnostic acumen among cardiothoracic surgeons and oncologists.

Estrangulated obturatory hernia. Case report of a challenging diagnosis of obstructive syndrome.

INTRODUCTION AND IMPORTANCE: Obturator hernia accounts for less than 1 % of all documented cases of pelvic hernias. It most commonly presents as an obstructive syndrome in elderly, multiparous, slim women, characterized by a wider pelvis that facilitates the passage of the hernia sac through the obturator foramen alongside the obturator nerve. In this case, adhering to the SCARE (Updating Consensus Surgical CAse REport) checklist criteria, we present a typical scenario involving an elderly woman who was initially misdiagnosed with a fecaloma, concealing an obturator hernia. CASE REPORT: An 85-year-old patient, displaying prodromal signs of senile disease, presented for medical attention with incapacitating abdominal pain in the right iliac fossa, accompanied by nausea and vomiting. Rectal examination revealed the presence of a fecaloma, and glycerin administration was performed rectally. The patient's condition worsened with the development of mental confusion and hyperactive delirium. Abdominal Computer tomography scan (CT scan) revealed right obturator hernia with enteral segment insinuation and dilation of the proximal bowel. An infraumbilical laparotomy was performed. The herniation of an ileal segment and the right ovary through the obturator foramen was identified. The content proved irreducible to manual maneuvers, leading to obturator muscle section following the dissection of the Retzius space. The right round ligament of the uterus was sectioned, and we manage to preserve the Obturatory branch of the lumbar plexus throughout dissection. A polypropylene mesh was positioned and secured with non-absorbable sutures on the Cooper's ligament, iliac crest, and obturator muscle and segmental enterectomy with primary anastomosis using a linear stapler was performed. CLINICAL DISCUSSION: This demanding case brings to the spotlight the importance of reevaluating even the usual cases. We provide our experience bringing together an unusual diagnosis after the conduction of a once diagnosed fecaloma that almost went down to a perforated acute abdomen. Hence the importance of suspect obturator hernia in unknown obstructive abdomen in elderly women. CONCLUSION: With this report we aim to raise awareness of careful propaedeutic inquiry of acute abdomen. We provide our experience bringing together the diagnosis that agrees with our literature review. Elderly patients commonly challenge the clinical evaluation, especially those with signs of senile disease. Thereby, inkling hidden diagnosis in typical scenarios can improve the patient's care in emergency settings.

Jejunojejunal intussusception induced by a gastrointestinal stromal tumor: a case report and literature review.

Intussusception is defined as the invagination of a proximal segment of the bowel into the adjoining or distal segment. In most adults with intussusception, there is a demonstrable lead point with a definite pathologic abnormality. The clinical features of intussusception include chronic intermittent abdominal pain, nausea and vomiting, constipation, and a palpable abdominal mass. The present case report describes a 62-year-old woman with a 2-week history of abdominal pain and 9-day history of vomiting. Clinical, imaging, and histologic evaluations revealed a jejunojejunal intussusception with a gastrointestinal stromal tumor as the lead point. A gastrointestinal stromal tumor should be considered as a possible lead point in adult patients with intussusception. The implication of reducing the intussusception prior to tumor resection requires further evaluation in view of the risk of venous embolism, including direct spread of malignant cells, in cases involving a large polypoid mass with a necrotic surface that extends to the serosa as shown by intraoperative examination. Accordingly, the rationale for adjuvant therapy with imatinib also requires further evaluation.

Idiopathic gingival fibromatosis and primary analysis of dominant bacteria in subgingival biofilm: a case report.

Idiopathic gingival fibromatosis (IGF), a rare fibroproliferative disease of unknown etiology, affects gingival tissue and has substantial adverse effects on patients. Therefore, the pathogenesis of IGF requires more extensive and in-depth research. In this case, a patient with confirmed IGF underwent initial nonsurgical periodontal therapy and gingivectomy, and the prognosis was good. The patient had no loss of periodontal attachment but had a history of swelling and bleeding of the gingiva prior to fibrous enlargement, which prompted further investigation. We explored the patient's subgingival microbiome and found a high abundance of periodontal pathogens. Gingival tissue biopsy revealed abundant fibrous tissue containing multiple inflammatory cell infiltrates. These results suggest that gingival inflammation secondary to periodontal pathogens can contribute to IGF onset.

Guillain-Barre syndrome following scrub typhus: a case report and literature review.

BACKGROUND: Scrub typhus is an acute infectious disease caused by Orientia tsutsugamushi. Guillain-Barre syndrome (GBS) is an autoimmune-mediated peripheral neuropathy with a frequent history of prodromal infections, but GBS associated with scrub typhus is very rare. CASE PRESENTATION: We report a 51-year-old male patient who developed dysarthria and peripheral facial paralysis following the cure of scfrub typhus. CSF examination and electrophysiological findings suggested a diagnosis of GBS. After treatment with intravenous immunoglobulin, the patient's neurological condition improved rapidly. CONCLUSIONS: Scrub typhus infection is likely to be a potential predisposing factor in GBS, while scrub typhus-associated GBS has a favorable prognosis.

Severe Hepatitis Complicating Olmesartan Enteropathy: A Case Report.

Introduction: Olmesartan, an angiotensin II receptor antagonist, is associated with an uncommon complication of enteropathy that presents insidiously, usually months to years after initial commencement of anti-hypertensive therapy which can be dose-dependent. It has a variable spectrum of clinical presentation but commonly presents as a moderate to severe malabsorptive process with potential severe complications related to poor end-organ perfusion. Lymphocytic gastritis and microscopic colitis are often noted in patients presenting with olmesartan-induced enteropathy; however, hepatic involvement has been less frequently observed. Case Presentation: We illustrate a case of a 43-year-old female presenting with 2 weeks of profuse non-bloody diarrhea in the context of olmesartan enteropathy which was complicated by an acute severe ischemic and enteropathic hepatopathy. Conclusion: Our case prompts clinicians to maintain a high index of suspicion in cases presenting with a seronegative enteropathy and concurrent acute liver injury while on olmesartan therapy. Cessation of olmesartan therapy resulted in prompt resolution of diarrheal symptoms and normalization of the acute transaminitis on subsequent three-week follow-up.

A case report of successful rescue using veno-arterial extracorporeal membrane oxygenation: managing cerebral-cardiac syndrome.

Introduction: The presence of cerebral-cardiac syndrome, wherein brain diseases coincide with heart dysfunction, significantly impacts patient prognosis. In severe instances, circulatory failure may ensue, posing a life-threatening scenario necessitating immediate life support measures, particularly effective circulatory support methods. The application of extracorporeal membrane oxygenation (ECMO) is extensively employed as a valuable modality for delivering circulatory and respiratory support in the care of individuals experiencing life-threatening circulatory and respiratory failure. This approach facilitates a critical temporal window for subsequent interventions. Consequently, ECMO has emerged as a potentially effective life support modality for patients experiencing severe circulatory failure in the context of cerebral-cardiac syndrome. However, the existing literature on this field of study remains limited. Case description: In this paper, we present a case study of a patient experiencing a critical cerebral-cardiac syndrome. The individual successfully underwent veno-arterial-ECMO (VA-ECMO) therapy, and the patient not only survived, but also received rehabilitation treatment, demonstrating its efficacy as a life support intervention. Conclusion: VA-ECMO could potentially serve as an efficacious life support modality for individuals experiencing severe circulatory failure attributable to cerebral-cardiac syndrome.

Case Report: Perianal infection in children caused by ingested jujube pits: a report of two cases.

Background: The ingestion of jujube pits by children is a rare cause of perianal infection.This article aimed to report two cases of perianal infection in children resulting from the ingestion of jujube pits. Methods: We reviewed the clinical records of perianal infection caused by jujube pits at our hospital. Details of the patients' presentation, imaging studies, complications and treatment were recorded. Results: Both pediatric patients presented with perianal swelling and pain. The caregivers of both patients denied a history of jujube consumption. Magnetic resonance imaging (MRI) indicated the presence of jujube pits, which were subsequently removed during surgery. Postoperatively, both patients recovered well, and follow-up showed no recurrence or the formation of anal fistulas. Conclusion: The ingestion of jujube pits leading to perianal infection is rare and inconspicuous. Early diagnosis and treatment are beneficial in preventing the occurrence of serious complications.

A case report of successful primary percutaneous coronary intervention to an occluded anomalous left main coronary artery arising from the right coronary sinus.

Background: Anomalous aortic origin of a coronary artery from the opposite sinus is a rare congenital abnormality that may be encountered during primary percutaneous coronary intervention (PCI) for ST-elevation myocardial infarction (STEMI). Case summary: A 65-year-old man presented with chest pain and signs of heart failure. Electrocardiogram demonstrated atrial fibrillation with ST elevation in the high lateral leads, and he was taken emergently to the cardiac catheterization laboratory for primary PCI. Coronary angiography identified the culprit to be an occluded anomalous left main coronary artery (LMCA) arising from the right coronary cusp, and primary PCI was successfully performed in the LMCA and the left anterior descending artery (LAD). Computed tomography angiography confirmed a benign retroaortic course of the anomalous LMCA with no additional high-risk features, as well as a new left atrial appendage thrombus. He subsequently developed deep venous thrombosis, acute pulmonary embolism, and acute kidney injury secondary to renal artery embolism with associated infarction. Workup for patent foramen ovale and thrombophilia were negative, and he was discharged in a stable condition. At 2-month follow-up, he was asymptomatic with no evidence of myocardial ischaemia on stress cardiac magnetic resonance imaging. Discussion: We present the first reported case of an occluded anomalous LMCA arising from the right coronary sinus in a patient presenting with STEMI. Rapid recognition of this congenital anomaly and selection of an appropriate guide catheter were keys to achieving timely reperfusion and a good outcome in this case.

A Rare Case of Idiopathic Pleuro-Parenchymal Fibroelastosis Presenting With Bilateral Spontaneous Pneumothoraces.

Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD), characterized by predominantly upper lobe pleural and subjacent sub-pleural parenchymal fibrosis. Its name refers to a combination of fibrosis involving the visceral pleura with fibroelastotic changes, predominantly in the subpleural lung parenchyma. We describe the case of a 67-year-old lady who presented to the accident and emergency department of Weston General Hospital with worsening shortness of breath (SOB) and cachexia of six to eight months' duration. The initial imaging studies showed bilateral spontaneous pneumothoraces on a background of pleural-based consolidation and fibrotic changes. A subsequent high-resolution CT (HRCT) chest showed evidence of pleuroparenchymal fibroelastosis in the background. She was not considered for anti-fibrotic medications due to the advanced stage of the disease and was managed with supportive measures, including oxygen support, oral steroids and antibiotics to cover for any infections. After initial management of symptoms and long discussions with the patient, family and the palliative team, she was discharged home with community follow-up.

Genetic Counseling for an Infertile Couple With Premature Chromatid Separation (PCS) Syndrome: A Case Report.

We report the first case of successful genetic counseling for an infertile couple with premature chromatid separation (PCS) syndrome. After our careful genetic counseling, the couple decided to continue infertility treatment. As a result, they gave birth to a baby (girl: 2,930 g) by caesarean section in May 2018. To our knowledge, there have not been any published reports regarding genetic counseling for an infertile couple with PCS after PubMed, EMBASE, and Web of Science searches until March 2024.

Epileptic Seizures in a Pediatric Patient With Vein of Galen Aneurysmal Malformation and Obstructive Hydrocephalus: A Rare Case Report.

The vein of Galen aneurysmal malformation (VGAM) is a rare congenital arteriovenous fistula of the embryonic median prosencephalic vein of Markowski, resulting in its pathological dilation. If left untreated, it can lead to multiple severe complications in the neonatal period, among which obstructive hydrocephalus. We present a case report of a six-year-old male patient with severe status epilepticus and a clinical history of VGAM and obstructive hydrocephalus, diagnosed via an MRI and an MR-angiography. The hydrocephalus was treated via a ventriculostomy at the age of six months, while the VGAM underwent a partial transarterial endovascular embolization when the patient was four years old. The procedures were successful, and there were no significant post-operative complications observed. The epileptic seizures began at a later point and were successfully medicated with valproate. However, they resumed due to a lowering of the medication dosage by the patient's parents. The patient was given a new valproic acid regimen with an appropriate dosage, and his parents reported no further seizures. This case report emphasizes the use of appropriate prenatal and neonatal diagnostic methods for VGAM and explores the nature of the multi-procedural therapy approach towards the pathology and its complications in relation to a possibly idiopathic co-pathology, namely epilepsy.

Hematological investigations in a case of intravascular hemolysis due to Clostridium perfringens infection.

A patient presented with fever, severe pain and edematous tight due to hip trauma and was scheduled for urgent fasciotomy. Following physical examination, laboratory analyses were requested, and results revealed anemia and severe infection. As the patient's condition was serious, a new set of samples was sent to the laboratory four hours later. Following centrifugation, severely hemolyzed dark-colored serum and plasma samples were obtained and in vitro hemolysis was suspected. The collection of samples was repeated, but a new set of samples was also hemolyzed with a significant decrease in the hemoglobin value. At that point, in vivo hemolysis was suspected, and samples were processed according to standard laboratory procedures for hemolytic samples. Following confirmation of the gas gangrene diagnosis by clinicians, the cause of hemolysis was attributed to the cytotoxic activity of α-toxin produced by the anaerobic gram-positive bacterium Clostridium perfringens. An insight into the laboratory procedure that could help to narrow down the causes of hemolysis and single out C. perfringens as a cause of intravascular hemolysis was given.

A case of pancreatic PEComa with prominent inflammatory cell infiltration: the inflammatory subtype is a distinct histologic group of PEComa.

BACKGROUND: PEComa is a mesenchymal tumor that can occur in various organs including the uterus and soft tissues. PEComas are composed of perivascular epithelioid cells, and angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangiomyomatosis (LAM) are considered lesions of the same lineage as tumors of the PEComa family. Histologically, a common PEComa shows solid or sheet-like proliferation of epithelioid cells. This is accompanied by an increase in the number of dilated blood vessels. Here, we report a case of pancreatic PEComa with marked inflammatory cell infiltration. CASE PRESENTATION: A 74-year-old male patient underwent an appendectomy for acute appendicitis. Postoperative computed tomography and magnetic resonance imaging revealed a 30 × 25 mm non-contrast-enhanced circular lesion in the tail of the pancreas. The imaging findings were consistent with a malignant tumor, and distal pancreatectomy was performed. Histologically, most area of the lesion was infiltrated with inflammatory cells. A few epithelioid cells with large, round nuclei, distinct nucleoli, and eosinophilic granular cytoplasm were observed. Spindle-shaped tumor cells were observed. Delicate and dilated blood vessels were observed around the tumor cells. Immunohistochemically, the atypical cells were positive for αSMA, Melan A, HMB-45, and TFE3. The cytological characteristics of the tumor cells and the results of immunohistochemical staining led to a diagnosis of pancreatic PEComa. CONCLUSIONS: A histological variant known as the inflammatory subtype has been defined for hepatic AML. A small number of tumor cells present with marked inflammatory cell infiltration, accounting for more than half of the lesions, and an inflammatory myofibroblastic tumor-like appearance. To our knowledge, this is the first report of pancreatic PEComa with severe inflammation. PEComa is also a generic term for tumors derived from perivascular epithelioid cells, such as AML, CCST, and LAM. Thus, this case is considered an inflammatory subtype of PEComa. It has a distinctive morphology that is not typical of PEComa. This histological phenotype should be widely recognized.

Left Atrial Appendage Aneurysm: A Case Report and Literature Review.

The left atrial appendage aneurysm is an uncommon condition that has garnered attention from the medical community due to its low incidence and varied clinical manifestations. The difficulty in identification is reflected in its incidental detection in imaging studies such as echocardiograms and tomographies, while symptoms range from mild to severe, including heart failure and thromboembolic events. The complex etiology includes congenital and acquired factors, and its management focuses on preventing complications through surgical resection, accompanied by medical strategies such as controlling heart rhythm and anticoagulation. The case of a 67-year-old woman with significant medical history illustrates these challenges. Despite an inconclusive initial diagnosis, a tomography revealed an aneurysm with an intracavitary thrombus, leading to successful surgical resection. However, subsequent infectious complications resulted in her death. The average age of diagnosis is around 30 years, and while it is more common in women, there are no significant gender differences. Surgical management remains the preferred option, especially in severe cases, although in some patients, a watchful waiting approach is chosen. In conclusion, the left atrial appendage aneurysm is a complex entity that requires a multidisciplinary approach to improve clinical outcomes. Early diagnosis and appropriate treatment are crucial to prevent serious complications and improve the quality of life of affected patients.

Giant Cell Tumor in Tarsal Midfoot Bones: A Case Report.

Diffuse tenosynovial giant cell tumor (D-TGCT), previously known as pigmented villonodular synovitis (PVNS), is a benign, aggressive, and distracting proliferative synovial lesion. D-TGCT is commonly seen in large joints such as the knee and hip. We present the case of a 57-year-old female who initially presented with swelling on the left midfoot that increased over four years. Clinically, a ganglion was suspected on the left midfoot and an MRI showed a heterogeneous lobulated soft tissue mass on the superior aspect of the tarsal midfoot measuring 5.8 x 2.4 x 4.2 cm. The mass causing remodeling and bony erosion was more appreciated at the medial aspect of the talus bone and extended to the sinus tarsi and talocalcaneal joint space. Surgical excision of the mass was performed, and pathology reports found lobulated soft tissue lesions composed of mononuclear cells, multinucleated giant cells, sheets of foamy macrophages, inflammatory cells, and hemosiderin-laden macrophages. This case represents D-TGCT without atypia or malignancy based on the findings.

A Novel Physiotherapy Approach for Enhancing Mobility in a 53-Year-Old With Down Syndrome: A Case Report.

Down syndrome (DS) is a genetic condition developing from a supplementary chromosome 21, referred to as trisomy 21. It ranks among the most prevalent developmental disabilities. People with DS often live inactive lifestyles, not meeting the weekly physical activity guidelines. With age, they face increased risks of cardiovascular disease and osteoporosis, as well as neurological and orthopedic concerns. Physiotherapy is especially important for improving balance, coordination, strength, and endurance in adults over the age of 50. Our approach consisted of a three-week regimen that included strengthening exercises based on the DeLorme strength training principle, balance exercises with perturbation and treadmill training, and coordination exercises with equilibrium and non-equilibrium movements. We evaluated outcomes using measures such as the Berg Balance Scale, Timed Up and Go test, and Functional Independence Measure, which were performed before and after the physiotherapy intervention. We present a case study of a 53-year-old woman to demonstrate the importance of physiotherapy in making lifestyle changes and improving strength, balance, and endurance, thereby improving overall quality of life through tailored interventions.

Abdominal Wall Endometriosis: Early Diagnosis of a Rare Iatrogenic Complication Following Cesarean Section.

Abdominal wall endometriosis is a rare form of extrapelvic endometriosis, frequently diagnosed with delay in most cases. It is typically iatrogenic and primarily associated with procedures such as cesarean sections or other gynecological surgeries. In our patient, endometriosis at the laparotomy scar was diagnosed relatively early, approximately two months after the onset of symptoms, which manifested 10 months after the last cesarean section. The patient, who had an obstetric history of three cesarean sections, presented at the Gynecology outpatient clinic of the General Hospital of Trikala, complaining of pain associated with menstruation located in the lower abdomen, near the surgical scar. Based on clinical and imaging findings, abdominal wall endometriosis was suspected, leading to a decision for wide excision of the endometriotic lesion. Histological examination of the surgical specimen confirmed the diagnosis. The postoperative course was uneventful, and three months after the surgery, the patient reported complete resolution of symptoms. This case report emphasizes the importance of integrating advanced diagnostic methods alongside classic clinical findings for the accurate diagnosis of abdominal wall endometriosis. It also highlights the contribution of surgical treatment through wide excision of the endometriotic lesion, in facilitating early diagnosis, achieving cure, and minimizing the risk of disease recurrence in the future.