Randomized Controlled Trial to Compare Stapled Hemorrhoidopexy Plus Ligation Anopexy With Stapled Hemorrhoidopexy for Managing Grade III and IV Hemorrhoidal Disease.

BACKGROUND: Despite the benefits of the stapled hemorrhoidopexy in the short term, management of prolapsing hemorrhoids, the long-term results are still insufficient regarding recurrent prolapse and patient satisfaction. The current study investigates the addition of ligation anopexy to stapled hemorrhoidopexy. OBJECTIVE: Valuation of adding ligation anopexy to stapled hemorrhoidopexy in improving short-term and long-term results in the treatment of grade III and IV hemorrhoids. DATA SOURCES: Between January 2018 and January 2020, we recruited 124 patients with grade III and IV hemorrhoids at Alexandria Main University Hospital. STUDY SELECTION: Randomized controlled trial. INTERVENTIONS: One hundred twenty-four patients were blindly randomly assigned to 2 equal groups: stapled hemorrhoidopexy (group I) and stapled hemorrhoidopexy plus ligation anopexy (group II). MAIN OUTCOME MEASURES: Recurrence of hemorrhoids and patient satisfaction after a follow-up period of at least 2 years. RESULTS: The average operating time was noticeably less in the stapled hemorrhoidopexy group. Postoperative pain, analgesia requirement, hemorrhoid symptoms score, return to work, complications rate, and quality of life 1 month after surgery were similar between groups. Following a mean follow-up of 36 months (interval, 24-47), in group I, 10 patients (16%) reported recurrent external swelling and/or prolapse compared to 3 patients (5%) in group II ( p = 0.0368). Five patients in group I required redo surgery, whereas no patients required redo surgery in group II. Long-term patient satisfaction was significantly better in group II. LIMITATIONS: It was a single-center experience, so longer follow-up was needed. CONCLUSIONS: Stapled hemorrhoidopexy and stapled hemorrhoidopexy plus ligation anopexy were similar in short-term results with regard to complications rate, hemorrhoids symptoms score, return to work, and quality of life. Long-term results were significantly better with regard to recurrence of external swelling and/or prolapse and patient satisfaction after stapled hemorrhoidopexy plus ligation anopexy. See Video Abstract . TRIAL REGISTRATION NUMBER: Pan African Clinical Trials Registry identifier PACTR20180100293130. ECA PARA COMPARAR LA HEMORROIDOPEXIA CON GRAPAS MS ANOPEXIA POR LIGADURA CON LA HEMORROIDOPEXIA CON GRAPAS PARA EL TRATAMIENTO DE LA ENFERMEDAD HEMORROIDAL DE GRADO III Y IV: ANTECEDENTES:A pesar de los beneficios de la hemorroidopexia con grapas a corto plazo, el manejo de las hemorroides prolapsadas, los resultados a largo plazo aún son insuficientes en cuanto al prolapso recurrente y la satisfacción del paciente, por lo que en nuestro estudio actual agregamos anopexia por ligadura a la hemorroidopexia con grapas.OBJETIVO:Valoración de añadir anopexia por ligadura a la hemorroidopexia con grapas para mejorar los resultados a corto y largo plazo en el tratamiento de las hemorroides grado III-IV.FUENTES DE DATOS:Entre enero de 2018 y enero de 2020 reclutamos a 124 pacientes con hemorroides de grado III-IV en el hospital universitario principal de Alexandria.SELECCIÓN DEL ESTUDIO:Ensayo controlado aleatorio PACTR201801002931307.INTERVENCIÓN(S):124 pacientes fueron asignados al azar de forma ciega a dos grupos iguales, hemorroidopexia con grapas (grupo I) y hemorroidopexia con grapas más anopexia por ligadura (grupo II).PRINCIPALES MEDIDAS DE RESULTADO:Recurrencia de hemorroides y satisfacción del paciente después de un período de seguimiento de al menos dos años.RESULTADOS:El tiempo operatorio promedio fue notablemente menor en el grupo de hemorroidopexia con grapas. Mientras tanto, el dolor posoperatorio, la necesidad de analgesia, la puntuación de los síntomas de hemorroides, el regreso al trabajo, la tasa de complicaciones y la calidad de vida un mes después de la cirugía fueron similares. Después de un seguimiento medio de 36 meses (intervalo: 24-47), el Grupo I, 10 pacientes (16%) se quejaron de inflamación externa recurrente y/o prolapso en comparación con 3 pacientes (5%) en el Grupo II ( p = 0,0368) que requiere rehacer la cirugía. No fue necesaria una nueva cirugía en el grupo II; además, la satisfacción del paciente a largo plazo fue significativamente mejor en el grupo II.LIMITACIONES:Se necesita un seguimiento más prolongado y experiencia en un solo centro.CONCLUSIONES:La hemorroidopexia con grapas comparada con la hemorroidopexia con grapas más anopexia por ligadura fue similar en resultados a corto plazo en cuanto a tasa de complicaciones, puntuación de síntomas de hemorroides, regreso al trabajo y calidad de vida. Los resultados a largo plazo fueron significativamente mejores en cuanto a la recurrencia de la inflamación externa y/o el prolapso y la satisfacción del paciente después de la hemorroidopexia con grapas más anopexia por ligadura. (Traducción-Dr. Mauricio Santamaria ).

Massive Localized Lymphedema: Two Case Studies and Diagnostic Challenges.

Massive localized lymphedema (MLL) is an emerging clinical phenomenon predominantly observed in morbidly obese individuals. It presents both diagnostic and therapeutic challenges to clinicians due to its characterization by large, pendulous masses in the abdomen or thigh. MLL may resemble malignant conditions, such as liposarcoma, leading to unnecessary invasive interventions. This study presents two case studies: a 74-year-old male who succumbed to postoperative complications and a 56-year-old female who experienced successful recovery. These cases highlight the urgent need for robust diagnostic criteria and evidence-based management approaches for MLL. In addition, further research exploring the pathogenesis, risk factors, and potential connections among MLL, hypothyroidism, and angiosarcoma is essential.

Paracetamol Versus Ondansetron for Prevention of Postoperative Shivering in Liposuction Surgeries Under Combined General Epidural Anaesthesia: A Randomized Controlled Trial.

Objective: Postoperative shivering (POS) is considered one of the most common complications that is encountered by the anaesthetists worldwide. Despite using several treatment options, there has not been a clear consensus regarding this issue. This trial was conducted to investigate the efficacy and safety of paracetamol and ondansetron in preventing POS in patients undergoing liposuction procedures under combined general epidural anaesthesia. Methods: One hundred twenty patients scheduled for liposuction were randomly allocated to one of three groups: group P (paracetamol group) which received 1 g paracetamol intravenously, group O (ondansetron group) which received 8 mg of ondansetron intravenously, and group S (saline group), which received 100 mL normal saline intravenously; all medications were given postoperatively. The primary outcome was the incidence of POS, and the secondary outcomes included shivering score, tympanic temperature, and the occurrence of side effects. Results: The incidence of occurrence of POS was found to be lower in groups P and O compared to group S with values of 25% and 37.50% vs. 77.50%, respectively, with a P value <0.001. Additionally, the severity of POS was found to be lower in groups P and O compared to group S (P <0.001). Tympanic temperature and complications were comparable between the groups with no significant differences. Conclusion: Prophylactic use of paracetamol or ondansetron at the end of the procedure was shown to be of great value in reducing the incidence and severity of POS, with no statistically significant difference between the paracetamol and ondansetron groups. Moreover, no significant drawbacks were reported as a result of using these medications.

Outcomes Following Type C Distal Humeral Fracture Fixation Using Two Different Osteosynthesis Techniques.

BACKGROUND: Distal humeral fractures are notorious injuries, and they require surgical fixation. The reliability of reconstruction devices has been a subject of debate. Our primary outcome was to detect differences, if any, between two different groups of patients using the patient-reported outcomes measurement information system (PROMIS) Global-10 form and to compare it with the Mayo elbow performance score (MEPS) results. Other secondary outcomes included the difference in union time between patients who received a reconstruction plate vs those who received a pre-contoured anatomical plate. MATERIAL AND METHODS: It is a prospective randomised study which included a total of 30 cases equally distributed into two groups. RESULTS: The mean PROMIS and MEPS scores for group A were 31.5 SD 6.6 and 77.7, respectively, compared to 33.7 SD 6.66 and 73, respectively, for the other group. Time to union was 13.4 weeks for group A and 12.6 weeks for the other group. There was no statistically significant difference between the groups regarding union and function. However, reconstruction plates were more cost effective. The correlation between the MEPS and PROMIS G scores in both groups was statistically significant. CONCLUSION: 1. Recon plates continue to be a cost-effective method of treatment in simple intra-articular fractures. 2.PROMIS is a valuable tool to be used along with other scores in future studies.

Perivascular Epithelioid Cell Tumor (PEComa) of the Pancreas in a Patient with Ulcerative Colitis: A Case Report and Review of the Literature.

Perivascular epithelioid cell tumors (PEComas) are mesenchymal tumors of peculiar cells that are focally associated with blood vessels, and generally have a distinctive bi-phenotypic expression of both smooth muscle and melanocytic markers. There are several entities in the PEComa family, including tumors that arise in the soft tissues and viscera. Frequently affected organs include the lungs (sugar tumors), uterus, broad ligament, colon, small bowel, liver, and pancreas. Ulcerative colitis (UC) has been associated with the development of tumors, especially colorectal and hepatobiliary carcinomas. Rare cases of UC have been reported in the PEComa family of tumors, but none in the pancreas. Here, we present a case study of a 27-year-old female patient with a history of UC who developed PEComa of the pancreas, a unique association that has not been previously reported. We also review reported cases of PEComas in the pancreas, as well as PEComas at all anatomic sites associated with UC.

Giant Ectopic Parathyroid Adenoma Arising in the Posterior Mediastinum. Report of Case and a Review.

We present a case of a greatly enlarged giant ectopic parathyroid adenoma that weighed 43 grams, which was located in the posterior mediastinum of a 74-year-old man. The patient presented with generalized weakness and decreased level of consciousness. He was found to have elevated level of serum calcium (19.9 mg/dl), and a subsequent assay of parathyroid hormone (PTH) was greatly elevated (2234 pg/ml). We report the course of management and outcome of the patient and present a review of the literature on giant ectopic parathyroid adenomas in the posterior mediastinum.

Evaluating the predictive value of fetal Doppler indices and neonatal outcome in late-onset preeclampsia with severe features: a cross-sectional study in a resource-limited setting.

BACKGROUND: Preeclampsia constitutes a major health problem with substantial maternal and perinatal morbidity and mortality. The aim of this study was to detect the diagnostic efficacy of fetal Doppler in predicting adverse outcomes in severe late onset preeclampsia (LOP). METHODS: A prospective study was conducted among childbearing women who presented with severe LOP and matched controls. Umbilical artery (UA) and middle cerebral artery (MCA) Doppler indices including pulsatility index (PI), resistance index (RI), systolic/diastolic ratio (S/D) and cerebroplacental ratio (CPR) were measured. RESULTS: All UA indices were significantly higher in the case group compared to the controls (p < 0.001). UA PI and RI were significantly correlated with all neonatal adverse outcomes except cord pH status (p < 0.05). Abnormal CPR was the most sensitive index that positively correlated with intrauterine growth retardation (IUGR), low 5- minute Apgar score and neonatal intensive care unit admission (79, 72.8 and 73.3%, respectively). In the same context, Abnormal UA PI and RI represented the most specific tool for predicting IUGR, low 1- and 5- minutes Apgar score with positive predictive values were 52, 87 and 57%, respectively. CONCLUSION: In severe LOP, UA Doppler remains the preferential indicator for adverse birth outcomes with CPR is the best index that could be solely used for predicting such outcome.

Blood miRNAs Are Linked to Frequent Asthma Exacerbations in Childhood Asthma and Adult COPD.

MicroRNAs have been independently associated with asthma and COPD; however, it is unclear if microRNA associations will overlap when evaluating retrospective acute exacerbations. Objective: We hypothesized that peripheral blood microRNAs would be associated with retrospective acute asthma exacerbations in a pediatric asthma cohort and that such associations may also be relevant to acute COPD exacerbations. Methods: We conducted small-RNA sequencing on 374 whole-blood samples from children with asthma ages 6-14 years who participated in the Genetics of Asthma in Costa Rica Study (GACRS) and 450 current and former adult smokers with and without COPD who participated in the COPDGene study. Measurements and Main Results: After QC, we had 351 samples and 649 microRNAs for Differential Expression (DE) analysis between the frequent (n = 183) and no or infrequent exacerbation (n = 168) groups in GACRS. Fifteen upregulated miRs had odds ratios (OR) between 1.22 and 1.59 for a doubling of miR counts, while five downregulated miRs had ORs between 0.57 and 0.8. These were assessed for generalization in COPDGene, where three of the upregulated miRs (miR-532-3p, miR-296-5p, and miR-766-3p) and two of the downregulated miRs (miR-7-5p and miR-451b) replicated. Pathway enrichment analysis showed MAPK and PI3K-Akt signaling pathways were strongly enriched for target genes of DE miRNAs and miRNAs generalizing to COPD exacerbations, as well as infection response pathways to various pathogens. Conclusion: miRs (451b; 7-5p; 532-3p; 296-5p and 766-3p) associated with both childhood asthma and adult COPD exacerbations may play a vital role in airflow obstruction and exacerbations and point to shared genomic regulatory machinery underlying exacerbations in both diseases.

Soft Tissue Sarcomas: A 16-Year Experience of a Tertiary Referral Hospital in North Jordan.

Background and Objectives: Sarcomas are rare malignant tumors of mesenchymal origin. Their low prevalence and histological heterogeneity make their diagnosis a challenging task. To the best of our knowledge, the epidemiology of soft tissue sarcomas (STSs) was not well studied in Jordan. This study thus aimed to determine STS epidemiologic trends at King Abdullah University Hospital (KAUH); a tertiary hospital that provides cancer healthcare for 70% of the population in Irbid Governorate, North Jordan. The findings of this study will provide a good reference point of the burden of STSs in Jordan and the Middle East region. Materials and Methods: All cases with confirmed STS diagnoses who attended KAUH from January 2003 until December 2018 were included in the initial analysis. Bone sarcomas, gastrointestinal stromal tumors and uterine sarcomas were not included in the study. Information collected from the pathology reports and electronic medical records was used to determine STS prevalence, incidence rate, age and gender distributions, histological types and anatomic location. Cases were reviewed by three pathologists with interest in soft tissue tumors. The findings were compared with literature. Results: In total, 157 STS cases were reported (1.9% of cancers diagnosed at KAUH during the 16-year study period). Crude annual incidence rate (IR) per 100,000 person-years ranged from 0.48 in 2015 to 1.83 in 2011 (average = 1.04). Age-standardized IR (ASR)(World WHO 2000-2025) was 1.37. Male:female ratio was 1.3:1. Median age was 39 years. Age ranged from <1 year to 90 years. Overall STS rates increased with age. The most common histological types were liposarcoma (19%), rhabdomyosarcoma (17%) and leiomyosarcoma (10%). The most common anatomic location was the extremity (40.1%), followed by the trunk (14.7%), then head and neck (10.8%). Conclusion: STSs are rare in North Jordan. A slight increase in their incidence was identified during the study period similar to global trends. The collection of relevant data on established risk factors along with a broader scale evaluation of the epidemiology of STS in the Middle East region is recommended to better evaluate disease burden and trends.

Snakebites in Jordan: A clinical and epidemiological study.

The epidemiology features and clinical symptoms associated with 121 cases of snakebite requiring admission to 24 hospitals in Jordan, during 2018-2020, are discussed. Ninety-six of them (79%) brought with them to the hospital the snake that bit them or a photograph of it. Echis coloratus was responsible for 68 of the bites and 6 fatalities. Sex ratio was 3.2 males: 1 female, with an overall average age of 27 ± 14.36 years. The highest incidence of bites was reported in September. Bites were most common on hands and legs. The period of hospitalization ranged from 1 to 36 days. Irbid and Karak governorates had the highest number of snakebites, most cases being reported from agricultural areas and among farmers. Clinical symptoms associated with five species of venomous snakes are described along with illustrative case histories. Symptoms associated with Echis coloratus bites included local swelling and necrosis, coagulopathy and bleeding, microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury (AKI) and chronic renal failure. Daboia palaestinae victims exhibited ecchymoses, local swelling and necrosis, with one case of angioedema. Other symptoms included thrombocytopenia, coagulopathy, microangiopathic hemolysis and local and systemic bleeding, as well as AKI. A single case of envenoming by Macrovipera lebetinus developed the following symptoms; swelling, severe pain, extensive ecchymoses, neutrophil leukocytosis, normochromic normocytic anemia and aggregated platelets with thrombocytopenia. Symptoms associated with two cases of Pseudocerastes fieldi included swelling that spread from bitten hands, and mild abnormalities of platelet count and bleeding time. Four cases of envenoming by Atractaspis engaddensis exhibited severe pain, local swelling, erythema, numbness and tissue necrosis. One of them developed acute systemic symptoms. The only antivenom currently available in Jordan, is VINS "Snake venom antitoxin (Biosnake)", manufactured in India using venoms of three African snakes that do not occur in Jordan. It proved clinically ineffective against envenoming by Jordanian Viperidae, failing to correct coagulopathy and life-threatening hemorrhage, and to prevent AKI.

Malignant Glandular Triton Tumor Arising in the Radial Nerve with Prolonged Survival: A Case Report and Review of the Literature.

Divergent differentiation is a well-known phenomenon in malignant peripheral nerve sheath tumors (MPNST) which occurs approximately in 15% of these tumors, usually towards mesenchymal elements. Differentiation towards epithelial components, however, is quite uncommon, and even exceptionally rare is concomitant mesenchymal and glandular differentiation. To our knowledge, only 14 cases of MPNST with both mesenchymal (rhabdomyoblastic) and glandular differentiation had been reported, and only two of these tumors had frankly malignant glandular components. Herein, we report the third such case. A 26-year-old male, without any of the stigmata of NF1, presented with a 2-year history of pain in his left shoulder and an elbow swelling of six-month duration. The tumor was initially diagnosed clinically as a neurofibroma at a local hospital. The patient underwent excision of the mass there, and pathological examination at that hospital showed the tumor to be MPNST. Six months later, the patient was referred to our hospital, a tertiary care medical center, with recurrent swelling at the same location. Histopathological material from the referral hospital was reviewed, and the tumor was diagnosed as MPNST with rhabdomyoblastic differentiation or malignant triton tumor (MTT) that contained in addition foci of malignant glandular epithelium. The patient refused any surgical intervention. He received three cycles of chemotherapy followed by radiotherapy with excellent response and marked reduction in the size of the tumor. The patient had prolonged survival for 10 years following the initial resection of the tumor.

Effects of Low-Dose Ketamine Infusion on Alleviating the Opioid Burden for Patients Undergoing Myomectomy Surgery.

OBJECTIVE: Recent research has focused on the use of N-methyl-D-aspartate (NMDA) receptor antagonists for pain management. Several drugs are known to have this action, including ketamine, which exerts its main analgesic effect through NMDA receptor antagonism. This study aimed to evaluate the effect of low-dose ketamine infusion on opioid exposure for patients undergoing myomectomy surgery under general anaesthesia. METHODS: A total of 70 women were included in this prospective double-blind trial study. The patients included in this study were American Society of Anaesthesiologists physical status I-II, aged between 18 and 50 years and scheduled for laparotomy myomectomy surgery. Patients were randomised to receive either a bolus of 0.2 mg kg-1 of ketamine followed by a continuous infusion of 0.2 mg kg-1 hr-1 during the operation or a placebo of normal saline. Both groups also received morphine as needed for pain relief. The primary outcome was the total amount of morphine used during the intraoperative and postoperative periods. Intraoperative and postoperative mean blood pressure, heart rate and postoperative visual analogue scale for pain were assessed. RESULTS: Total mean morphine consumption was significantly lower in the ketamine group than in the control group (26±3.5 mg vs. 34.7±3.3 mg, respectively, p<0.05). However, there were no statistical differences between the groups regarding haemodynamics, postoperative pain score and complications. CONCLUSION: The use of ketamine in low infusion doses intraoperatively during an elective myomectomy procedure produced an opioid-sparing effect by reducing perioperative morphine consumption without significant side effects.

Endotracheal intubation in patients undergoing open abdominal surgery in the lateral position: a comparison between the intubating video stylet and fiberoptic intubating bronchoscopy.

BACKGROUND: Some situations compel anesthetists to execute endotracheal intubation in the lateral position. We compared elective endotracheal intubation in the lateral decubitus position using the video stylet (VS) device with the fiberoptic (FO) bronchoscope device in patients undergoing abdominal surgery. METHODS: Overall, 50 patients were enrolled in this prospective, randomized study. They were randomly classified into the VS intubation or FO intubating bronchoscope group. After anesthesia induction, patients were placed in the lateral decubitus position, and a single investigator well-versed with the use of the VS and FO bronchoscope performed the intubation. The primary outcome was the time taken for intubation. Secondary outcomes included the intubation success rate, hemodynamic response at specific time points and perioperative complications. RESULTS: The average time taken for intubation was significantly lesser in the VS group than in the FO group, with values of 39.5 ± 10.0 and 75.6 ± 16.2 s, respectively (P < 0.001). Incidences of a successful first attempt of intubation in the VS and FO groups were 88% and 100%, respectively, showing no significant difference. There was a negligible difference in complications between the groups, except sore throat, which showed a higher incidence in the VS group than in the FO group (P = 0.002). CONCLUSIONS: In laterally positioned patients, elective endotracheal intubation with VS provides less intubation time; however, its use is accompanied by a significant increase in the hemodynamic response after intubation and an increased incidence of sore throat.

Malignant teratoid tumor of the thyroid gland: an aggressive primitive multiphenotypic malignancy showing organotypical elements and frequent DICER1 alterations-is the term "thyroblastoma" more appropriate?

Primary thyroid teratomas are exceedingly rare. Mature and immature variants recapitulate their gonadal counterparts (predilection for infants/children, triphasic germ layer differentiation, and favorable outcome). On the other hand, the so-called malignant teratomas affect predominantly adults and elderly, are highly aggressive, and, according to a few published cases, harbor DICER1 mutations. We describe three highly aggressive sporadic malignant teratoid thyroid tumors in 2 females (17 and 45 years) and one male (17 years). Histology showed triphasic neoplasms composed of solid nests of small primitive monomorphic cells embedded in a cellular stroma with primitive immature rhabdomyosarcoma-like (2) or pleomorphic sarcoma-like (1) phenotype. The third component was represented by TTF1+/PAX8+ primitive teratoid epithelial tubules reminiscent of primitive thyroid follicles and/or Wilms tumor, admixed with scattered respiratory- or enteric-type tubules, neuroepithelial rosettes, and fetal-type squamoid nests. Foci of cartilage were seen in two cases, but none contained mature organoid adult-type tissue or skin adnexa. SALL4 was expressed in the small cell (2) and stromal (1) component. Other germ cell markers were negative. Molecular testing revealed a known "hotspot" pathogenic DICER1 mutation in two cases. In addition, case 1 had a missense TP53 variant. This type of thyroid malignancy is distinct from genuine teratomas. The immunoprofile suggests primitive thyroid- or branchial cleft-like differentiation. Given that "blastoma" is a well-accepted terminology in the spectrum of DICER1-associated malignancies, the term "thyroblastoma" might be more convenient for these malignant teratoid tumors of the thyroid gland. Relationship of thyroblastoma to the DICER1 syndrome remains to be addressed.

Low-Dose Ketamine Infusion Versus Morphine Infusion During Abdominoplasty to Change the Postoperative Pain Profile.

BACKGROUND: With the increased number of abdominoplasty all over the world, and the need to manage postoperative pain, it is a must to find proper and effective drugs to decrease opioid consumption in the postoperative period. OBJECTIVES: In this double-blind randomized controlled clinical trial, we assumed that low-dose ketamine infusion will reduce the postoperative pain profile than the conventional method of morphine. METHODS: The scheduled patients for abdominoplasty under general anesthesia were recruited in two groups: group (K) with low-dose ketamine infusion intra-operatively (80 patients) and group (M) with morphine infusion intra-operatively (80 patients). Both groups were monitored intraoperatively and postoperatively for rescue doses of fentanyl, visual analogue scale (VAS), and side effects. RESULTS: There were no statistical differences between both groups regarding the fentanyl rescue doses intra- and postoperative with no remarkable side effects. CONCLUSIONS: Low-dose ketamine has a useful analgesic effect in abdominoplasty similar to morphine without remarkable side effects, such as sedation or hallucinations.

Gastric Plexiform Fibromyxoma Arising in the Cardia in an Adolescent Male: A Rare Tumor with an Unusual Location.

Plexiform fibromyxoma of the stomach, also known as plexiform angiomyxoid myofibroblastic tumor, is a rare benign gastric mesenchymal tumor, first described in 2007, which usually arises in the gastric antrum and affects adults. Few cases have been reported in children and adolescents. It can present with different clinical manifestations including abdominal pain, dyspepsia, hematemesis, and vomiting. Preoperatively, this tumor is usually diagnosed as gastrointestinal stromal tumor (GIST), and the correct diagnosis is made only after histopathological examination following surgical resection. Most cases were reported from East Asia (China, Japan, and Korea), North America, and Europe. We report herein a unique case of plexiform fibromyxoma, the first to be reported from the Middle East, arising in the cardia of the stomach in a 16-year-old adolescent male, with a brief review of the literature.

Immunopathology of Kikuchi-Fujimoto disease: A reappraisal using novel immunohistochemistry markers.

AIMS: Kikuchi-Fujimoto disease (KFD) is a self-limited disease characterised by destruction of the lymph node parenchyma. Few studies have assessed the immunohistological features of KFD, and most employed limited antibody panels that lacked many of the novel immunohistochemistry markers currently available. METHODS AND RESULTS: We used immunohistochemistry to reappraise the microanatomical distribution of plasmacytoid dendritic cells (pDCs), follicular helper T cells and cytotoxic T cells, B cells, follicular dendritic cell (FDC) meshworks, and histiocytes in lymph nodes involved by KFD. The study group consisted of 138 KFD patients (89 women; 64.5%) with a median age of 27 years (range, 3-50 years). Cervical lymph nodes were most commonly involved, in 108 (78.3%) patients. The numbers of pDCs were increased, predominantly around and within apoptotic areas and the paracortex, and tapering off within xanthomatous areas. pDCs formed sizeable tight clusters, most notably around apoptotic/necrotic areas. T cells consisted mostly of CD8-positive cells with predominant expression of T-cell receptor-ß. There were notable increases in the numbers of CD8-positive T cells within lymphoid follicles, and their numbers correlated with alterations in FDC meshworks (P < 0.001). The number of follicular helper T cells was decreased within distorted FDC meshworks. CD21 highlighted frequent distortion of FDC meshworks, even in lymph node tissue that was distant from apoptotic/necrotic areas. Distorted FDC meshworks spanned all morphological patterns, and FDC meshwork characteristics (intact; distorted; remnant/nearly absent) correlated with morphological patterns (P < 0.01). CONCLUSIONS: The immunohistological landscape of KFD is complex and characterised by increased numbers of pDCs that frequently cluster around apoptotic/necrotic foci, increased numbers of cytotoxic T cells, and substantial distortion of FDC meshworks.

Primary Chordoma of the Nasopharynx: A Rare Case Report and Review of the Literatures.

Primary chordoma of the nasopharynx is an extremely rare malignant tumor of notochordal origin in the extra-osseous axial skeleton. It presents as a soft tissue mass without involvement of the skull base bone (clivus) and may mimic other lesions of the nasopharynx. A 26-year-old male patient is presented with nasal obstruction and congestion for the last 3 years. Physical and radiological examination revealed a mass in the naso-oropharyngeal region. It was suspected to be a cystic mass or abscess on radiological imaging. However, histopathological examination revealed a chordoma. We review all 20 cases of primary nasopharyngeal chordoma reported previously in the literature. Nasopharyngeal chordoma should be considered in the differential diagnosis of nasopharyngeal mass due to its unspecific appearance on clinical and radiology examination.

Mediastinal malignant triton tumor: A rare case series and review of literature.

INTRODUCTION: In 1938, the Malignant Triton Tumor (MTT) was first explained by Mason. CASE PRESENTATION: Case 1: A man aged 28 years presented with chest pain and difficulty in breathing since last five months, there was no history of cough fever or night sweats. Clinical examination was unremarkable. His routine hematological tests including tumor markers were within normal range, testicular ultrasound was normal. CT scan of thorax revealed a mass in the anterior mediastinum. CT guided biopsy revealed a malignant triton tumor. Case 2: A 30 years old man, nonsmoker presented with history of chest tightness and feeling pressure while kneeling down since last 3 months, otherwise fit and healthy. His routine hematological investigations including tumor markers were within normal range. A CT scan of thorax revealed a large mass in the right posterior mediastinum. CT guided biopsy showed malignant triton tumor. Case 3: A man aged 28 years presented with chest pain and difficulty in breathing since last five months, there was no history of cough fever or night sweats. Clinical examination was unremarkable. His routine hematological tests including tumor markers were within normal range, testicular ultrasound was normal. CT scan of thorax revealed a mass in the anterior mediastinum. CT guided biopsy revealed a malignant triton tumor. DISCUSSION: Malignant peripheral nerve sheath tumors (MPNST) are uncommon sarcomatous tumors that are believed to be derived from Schwann cell or neighboring cells with perineurial differentiation. MTT is rarely reported in mediastinum, lung and heart (<10%) To the best of our knowledge, only few cases of MTT in the mediastinum have been reported in English literature, including, four were reported in the anterior mediastinum, three in the posterior mediastinum, one in the middle mediastinum and one between the ascending aorta and the main pulmonary artery. Most of the patients were young adults. We report three cases of rare mediastinal malignant triton tumors. They have been treated with palliative surgery/radical surgery +/- adjuvant therapy. The prognosis varied from a 3 month overall survival time to being alive at a 53 month follow-up period. CONCLUSION: In conclusion we report three rare cases of mediastinal malignant triton tumor treated with radical surgical resection and post-operative radiotherapy, one patient developed lung metastasis, and two had late local recurrence. The malignant triton tumor is a lethal neoplasm which carries very poor prognosis particularly when they occur in the mediastinum because it's very difficult to obtain wider tumor free margin due to the nature of location site.

Metastasizing Malignant Granular Cell Tumor (Abrikossoff Tumor) of the Anterior Abdominal Wall, with Prolonged Survival.

Malignant granular cell tumor (MGCT) is a rare high-grade mesenchymal tumor of Schwann cell origin. MGCTs commonly affect thigh, extremity, and trunk; however, involvement of the abdominal wall is quite rare. It has poor prognosis with 39% mortality rate in 3-year interval. We report a 50-year-old female who had MGCT arising in the anterior abdominal wall and developed massive metastatic deposits in both lungs and in the right inguinal lymph nodes, with prolonged survival for 11 years. A brief review of the literature is presented.