Abdominal Mass Research Articles

Association of change in fat and lean mass with incident cardiovascular events for women in midlife and beyond: A prospective study using dual-energy x-ray absorptiometry (DXA).

To determine whether changes in fat and lean mass over time, quantified using dual-energy x-ray absorptiometry (DXA), are related to incident cardiovascular events. Previous studies using surrogate anthropometric methods have had inconsistent findings. Prospective, longitudinal observational study of women aged 40 to 80 randomly selected from the electoral roll and stratified into decades: 40-49, 50-59, 60-69 and 70-79years. Changes in anthropometric measurements (body mass index and waist-to-hip ratio) and DXA-quantified fat mass and lean mass between the first and fifth years of the study. Incident cardiovascular events recorded from the sixth to the 12th year. In total 449 participants (87.9%) were analyzed. A 10% or greater decrease in total fat mass index was associated with a 67% lower likelihood of any cardiovascular event (OR=0.33, 95%CI 0.15-0.71); no association was observed for an increase. A 10% or greater decrease in abdominal fat mass index was associated with a 62% lower likelihood of incident stroke (OR=0.38, 95%CI 0.16-0.91); no association was observed for an increase. A 10% or greater decrease in appendicular lean mass index resulted in increased odds ratio of 2.91 for incident peripheral artery events (OR=2.91, 95%CI 1.18-7.20). Reducing fat mass for women in midlife and beyond may decrease the risk of cardiovascular events. An increase in fat mass may not contribute to additional cardiovascular events. A reduction in limb muscle mass may provide an independent marker for cardiometabolic risk and peripheral artery disease. No independent association was found using anthropometric measurements and incident cardiovascular events.

Biliary cysts in adults: Cerrahpaşa experience.

Biliary cysts are biliary duct dilatations, with 20% of the cysts being diagnosed in adulthood. Abdominal pain, jaundice and palpable abdominal mass are defined as the classical triad. However, nausea, vomiting, fever, itching and weight loss are frequent complaints. There are several treatment options depending on the type of the cyst. This study aimed to share our experience with biliary cysts and contribute to the literature on this subject. Thirty patients, who received treatment for biliary cyst from January 1981 to December 2018 at our clinic, were studied retrospectively. The patients were analyzed based on age, sex, type of the cyst, diagnosis and treatment methods, post-op follow up and complications. Twenty-seven of the patients were females, and three were males. The patients were aged between 16 and 76 years, and the median age was 41.9 years. All patients presented with abdominal pain, which was accompanied by cholangitis in nine patients, nausea and vomiting in four patients, dyspepsia in three patients and palpable mass in one patient. According to the Todani classification, biliary cyst findings were consistent with Type I in 23 patients, Type V in three patients, Type IV in two patients, Type II in one patient and Type III in one patient. Diagnosis and treatment are complex in biliary cysts due to anatomical proximity and variations. Therefore, it would be beneficial to refer them to referral centers. Choice of treatment should be based on the type of the cyst.

'Testicular masquerade': a case report of testicular malignancy with persistent Müllerian duct syndrome and transverse testicular ectopia.

Persistent Müllerian duct syndrome (PMDS) is a rare sexual development disorder. It is even more rarely associated with transverse testicular ectopia (TTE), a rare form of testicular ectopia, in which both testes descend through a single inguinal canal and are present in the same hemiscrotum. PMDS with TTE is associated with 18%-33% malignant transformation. Here we report the case of a 48-year-old man who presented with a large right inguinoscrotal swelling and on evaluation was found to have a large right testicular mass with complete right inguinal hernia, undescended left testis and a central abdominal mass. On evaluation with contrast-enhanced computed tomography abdomen and pelvis and image-guided biopsy he was diagnosed with mixed germ cell tumour of the right testis (predominantly a seminoma) with a retroperitoneal nodal mass and absent left testis, for which he received chemotherapy. Post-chemotherapy he underwent surgery and was diagnosed intraoperatively with PMDS along with TTE and testicular malignancy arising from the ectopic left testis. Postoperative recovery and follow-up were uneventful. Most cases of PMDS are diagnosed early in life. They present clinically with unilateral or bilateral undescended testis with inguinal hernia. In adults, PMDS is usually associated with male infertility. However, TTE is associated with an increased risk of testicular tumours if undiagnosed until adulthood. In adults PMDS with TTE is usually an intraoperative finding and is commonly associated with malignancy in the ectopic/undescended testis.

Abstract A009: CD103+CD56+ innate lymphoid cells are associated with an immunosuppressive microenvironment in ovarian cancer

Abstract Background: Regulatory innate lymphoid cells (ILCregs) are an emerging heterogenous subset of innate lymphoid cells that suppress immunity in several healthy and diseased contexts, including cancer. Previously, we identified CD56+ ILCregs from in vitro expanded tumour-infiltrating lymphocyte (TIL) cultures in patients with epithelial ovarian carcinoma (EOC). However, lineage-defining surface markers or transcription factors that distinguishes these regulatory populations from other conventional NK cells have not been identified. The objective of this study is to characterize markers that define ILCregs directly from primary tumours and investigate its role within the tumour microenvironment (TME). Methods: Women with suspected abdominal mass were recruited and consented at Gynecology Cancer Clinic at Princess Margaret Hospital. Surgically resected EOC tumours were processed and analyzed by flow cytometry and single-cell RNA-sequencing. Suppression assays were performed by co-culture of tumour-infiltrating CD103+CD56+ ILCregs with autologous TILs for 4 days in vitro with soluble αCD3. Results: We found that CD103 expressing CD56+ ILCregs were associated with poor clinical prognosis in patients with ovarian carcinoma. CD103+CD56+ ILCregs displayed reduced expression of cytolytic related markers (i.e., GZMB, CD107a, CD16) compared to CD103-CD56+ NK cells and had a distinct transcriptomic profile from NK cells. Interestingly, CD103+CD56+ ILCregs displayed distinct surface markers (i.e., CD49a, CD69, GITR) and transcription factor networks that were associated with Treg development, differentiation, and suppressive function. Intratumoural CD103+CD56+ ILCregs suppressed autologous CD8+ T cells by downregulation of granzyme B in vitro. Concordantly, the abundance of CD103+CD56+ ILCregs in patient tumours were associated with reduced activated CD8+ T cells within the TME. Conclusion: This study identified CD103+CD56+ ILCregs in the TME of patients with EOC that are associated with poor clinical prognosis and plays a key role in regulating T cells. Further investigations into underlying immunoregulatory networks, including ILCregs, within the TME may provide novel targets to improve prognosis for patients with EOC. Citation Format: Douglas C Chung, Jehan Vakharia, Kathrin Warner, Nicolas Jacquelot, Azin Sayad, SeongJun Han, Maryam Ghaedi, Carlos R Garcia-Batres, Alisha Elford, Ben X Wang, Linh T Nguyen, Patricia A Shaw, Blaise A Clarke, Marcus Q Bernardini, Sarah E Ferguson, Pamela S Ohashi. CD103+CD56+ innate lymphoid cells are associated with an immunosuppressive microenvironment in ovarian cancer [abstract]. In: Proceedings of the AACR Special Conference in Cancer Research: Tumor Immunology and Immunotherapy; 2023 Oct 1-4; Toronto, Ontario, Canada. Philadelphia (PA): AACR; Cancer Immunol Res 2023;11(12 Suppl):Abstract nr A009.

Ileocecal Intussusception of the Adult Induced by the Gastrointestinal Stromal Tumor of the Ileocecal Valve - A Case Report

Abstract Adult intussusception is a rare entity which is distinct frompaediatric cases in incidence, aetiology, and management. It represents 5% of all intussusceptions and is the cause of 1% of allintestinal obstructions, 0,08% of all abdominal surgeries and 0,003-0,02% of all hospital admissions. Ileocolic intussusceptionin adults is a unique variant in which nearly 100% of cases havea malignant lead point. In our report, we described a case of apatient with ileocecal intussusception caused by a rare type of the gastro intestinal tumor. The female patient was admitted to hospital for occasional pain in the lower right quadrant of the abdomen followed by abdominal discomfort and appearance of blood in thestool. The result of CT scan of the abdomen and pelvis showed atumor mass and intussusception at the ileocecal junction, whichwas confirmed peroperatively. Open right hemicolectomy wasperformed adhering to oncological principles. The final pathologic diagnosis indicated the gastrointestinal stromal tumor of theileocecal valvе. The diagnosis of intussusception in adults is delicate, and timely surgical treatment can be vital. Patients with thepalpable abdominal mass, digestive tract obstruction, gastroin-testinal bleeding, or lead point computed tomography must undergo a surgical examination. Given a high risk of malignancy,primary surgical resection using oncologic principles presents the best option for the treatment of ileocecal intussusception inadults.

A Patient with a Thoracic Tumefation

Tumors of the chest wall are uncommon lesions that comprise a heterogeneous group of neoplasms. These tumors may arise from osseous structures or soft tissues and may be malignant or benign. We describe the case of a proliferative swelling of uncertain origin that led us to suspect a neuroendocrine origin for the mass we observed and studied. Neuroendocrine tumors arise from neuroendocrine cells, which are peptideand amine-producing cells dispersed throughout the body. Usually, these tumors occur in various organs as intrathoracic or abdominal masses that become manifest after a variable time, usually after inducing a heterogeneous symptomatology secondary to the secretion of various molecules. The case we describe, on the other hand, develops both exophytically and endophytically, first appearing as an externally developing thoracic mass. Subsequent appropriate investigations revealed bone and muscle involvement in the patient’s thoracic cavity. Therefore, we describe our management of a paucisymptomatic patient who presented with a rapidly developing swelling in the intermammary site that progressed equally rapidly.

The Carney Triad

Abstract Introduction/Objective Carney triad is a multi-tumor syndrome comprising of gastrointestinal stromal tumor (GIST), extra-adrenal paraganglioma and pulmonary chondroma that was first described in 1977. The purpose of this report of this to remind the pathologists about this triad and about the fact that pathology is far beyond just giving a diagnosis on a surgical specimen. Methods/Case Report A 41-year-old female presented with dizziness, nausea, vomiting, weakness, early satiety, and palpitations. She was found to be pregnant, and an ultrasound showed an intrauterine pregnancy along with a large abdominal mass. A subsequent computed tomography (CT) scan demonstrated a left adrenal heterogeneous mass. CT chest was also performed which demonstrated a 0.3 cm right middle lobe pulmonary nodule, compatible with a benign tumor. Lab work showed cortisol, dehydroepiandrosterone sulfate (DHEAS), and adrenocorticotropic hormone (ACTH) to be within normal limits, but both metanephrine and normetanephrine were elevated. The patient was treated with an alpha and beta blockade prior to undergoing an adrenalectomy. During adrenalectomy an incidental mass was found at the greater curvature of the stomach. On gross examination, the adrenal mass was a large, encapsulated, brown-black, multi-lobulated, weighing 1,427 grams and measuring 19.8 x 13.7 x 11.6 cm. Microscopically, the mass was consistent with an extra-adrenal paraganglioma, compressing the adjacent normal adrenal gland and not invading or arising from it. The stomach mass was grossly tan-yellow, multi-lobulated, and firm. The cut sections were variegated tan, white. Microscopically, the mass was consistent with a GIST. Results (if a Case Study enter NA) NA Conclusion Individual diagnoses were rendered for the adrenalectomy and gastric mass specimens. Additionally, possibility of carney’s triad was suggested based on patient’s chest imaging findings.

Severe bronchiectasis resulting from chronic bacterial bronchitis and bronchopneumonia in a jungle cat.

Bronchiectasis is irreversible bronchial dilation that can be congenital or acquired secondary to chronic airway obstruction. Feline bronchiectasis is rare and, to our knowledge, has not been reported previously in a non-domestic felid. An ~10-y-old female jungle cat (Felis chaus) was presented for evaluation of an abdominal mass and suspected pulmonary metastasis. The animal died during exploratory laparotomy and was submitted for postmortem examination. Gross examination revealed consolidation of the left caudal lung lobe and hila of the cranial lung lobes. Elsewhere in the lungs were several pale-yellow pleural foci of endogenous lipid pneumonia. On cut section, there was severe distension of bronchi with abundant white mucoid fluid. The remaining lung lobes were multifocally expanded by marginal emphysema. Histologically, ectatic bronchi, bronchioles, and fewer alveoli contained degenerate neutrophils, fibrin, and mucin (suppurative bronchopneumonia) with rare gram-negative bacteria. Aerobic culture yielded low growth of Proteus mirabilis and Escherichia coli. There was chronic bronchitis, marked by moderate bronchial gland hyperplasia, lymphoplasmacytic inflammation, and lymphoid hyperplasia. The palpated abdominal mass was a uterine endometrial polyp, which was considered an incidental, but novel, finding. Chronic bronchitis and bronchopneumonia should be considered as a cause of bronchiectasis and a differential diagnosis for respiratory disease in non-domestic felids.

Targeting BCMA in Plasmablastic Lymphoma: Two Cases of Therapeutic Response to Teclistamab

Plasmablastic lymphoma (PBL) is an aggressive subtype of B-cell Non-Hodgkin lymphoma (B-NHL) with poor outcomes. PBL occurs more commonly in HIV-positive or immunocompromised patients, but it can also occur in immunocompetent patients. Despite anthracycline-based induction therapy, PBL is associated with chemotherapy resistance and early disease relapse, yielding a median overall survival of 7 to 15 months (Castillo JJ, et al. Blood. 2015). The advent of novel therapies altered the therapeutic landscape for many common subtypes of B-NHL, but progress in PBL is lacking due to the rarity of this disease and the lack of effective therapeutic targets. While PBL does not express many common B-NHL markers (e.g. CD19 or CD20), it does highly express B-cell maturation antigen (BCMA). However, data regarding the efficacy of therapies targeting BCMA in PBL is limited. Herein, we report two cases of relapsed/refractory PBL treated with the BCMA-targeting bispecific antibody teclistamab. Patient 1 was a 33 year old male without significant comorbidities when he was diagnosed with Stage IIE PBL after presenting with a chest wall mass. He was treated with six cycles of dose-adjusted EPOCH-V, followed by radiation to the involved region and a BEAM autologous hematopoietic stem cell transplant. Post-transplant imaging showed him to be in a CR on day 30 PET, but he developed recurrent PBL on his day 100 PET exam with osseus lesions. He was then treated with daratumumab and ICE chemotherapy for four cycles, with an initial resolution of osseus lesions. However, he developed relapsed disease with perilymphatic lung nodules. His PBL was confirmed to express BCMA and he was started on teclistamab monotherapy. Following one cycle of weekly teclistamab (1.5mg/kg), his post-treatment PET imaging demonstrated a complete response. He then underwent a consolidative allogeneic hematopoietic stem cell transplant from a matched unrelated donor. As of this report, he remains in a complete response based on his 30 day PET restaging imaging after transplant and without clinical evidence of disease more than 90 days after transplant. Patient 2 was a 60 year old male with medical history of comorbid CAD, stage IV chronic kidney disease, chronic back pain requiring opioid treatment, and peripheral arterial disease when he was diagnosed with Stage IV PBL after he was found to have mass-like thickening of his descending colon on a CT scan. He underwent partial colectomy, with pathology revealing PBL and his post-operative staging PET/CT scan noting osseus, splenic, and hepatic lesions consistent with advanced stage disease. He was initially treated with DA-EPOCH for six cycles with intrathecal methotrexate, which yielded an initial complete response, though he relapsed four months after completing therapy. He was then treated with gemcitabine, oxaliplatin, and daratumumab but progressed after eight cycles on treatment with abdominal and perisplenic masses confirmed on biopsy to be PBL He was then initiated on teclistamab, which was continued weekly. His treatment course was complicated by a viral pneumonia from which he recovered with IVIG and supportive measures. Following 2 total cycles of therapy, his repeat PET/CT imaging was consistent with a complete response (Figure 1), and he remains on weekly teclistamab therapy with no evidence of disease to date. The encouraging responses noted in our two cases illustrate the therapeutic potential of targeting BCMA using teclistamab in PBL. In our cases, teclistamab was a well tolerated therapy, even in our patient with significant comorbidities. This report provides a foundation for future prospective, multicenter clinical trials to evaluate the efficacy and safety of teclistamab in the treatment of relapsed and refractory PBL.

Rapidly growing massive abdominal sarcomatoid carcinoma on F18-FDG PET-CT scan

Sarcomatoid carcinoma is a rare type of tumour and most commonly arises in the lungs. However, rarely can it also be found in the abdomen. Sarcomatoid tumours are aggressive with large tumoural volume showing cancerous epithelial cells mixed with sarcomatous (nerve, muscle, fat etc.) features on histopathology. Most of the carcinosarcomas arise in the background of pleomorphic adenoma, originating from a myoepithelial precursor. These tumours are resistant to treatment and rapidly metastasize. We present a unique case of hepatocellular sarcomatoid sarcoma, evaluated through F18-FDG PET/CT. Keywords: Sarcomatoid sarcoma, 18F FDG PET-CT, massive abdominal mass.

A painless growing abdominal mass in a patient with systemic lupus erythematosus

A painless growing abdominal mass in a patient with systemic lupus erythematosus

Clinical, imaging and pathological features of hepatic carcinosarcoma in a sugar glider (Petaurus breviceps)

AbstractA 5‐year 6‐month‐old, neutered, male sugar glider (Petaurus breviceps) presented for lethargy. Plasma biochemistry revealed severely elevated alkaline phosphatase and moderate hypercholesterolemia. A heterogenous liver and a right abdominal mass were noted on abdominal ultrasonography. Computed tomography revealed an enlarged liver with ill‐defined hypoattenuating regions and a heterogenous mass arising from the caudate lobe. Cytology of the liver was consistent with low‐grade mixed lipid and hydropic vacuolar change and mild to moderate mixed to mature lymphocytic infiltrate. Liver biopsies were declined, and the patient was managed with silymarin and prednisolone. Serial bloodwork and abdominal ultrasonography revealed persistently elevated alkaline phosphatase and progressive heterogenous hepatomegaly and hepatic nodules. The sugar glider died over 2 years after original presentation. On postmortem examination, there was a poorly differentiated hepatic neoplasm, with immunohistochemistry most consistent with a carcinosarcoma. In future cases, hepatic biopsies could be considered for sugar gliders presenting with hepatic masses when cytology is unrewarding.

Clinical Presentation and Histological Types of Ovarian Tumor in Patients Admitted in Tertiary Care Center

Background: Ovarian cancer is the eighth most frequent cancer among women worldwide, accounting for 4% of all cancers in the female population. When compared to other reproductive system cancers, ovarian cancer has a high death and morbidity rate. Objectives: The aim of the study was to assess the clinical presentation and histological types of ovarian tumor in patients admitted in tertiary care center. Methods: A descriptive cross-sectional study was conducted to determine the clinical presentation and histopathological types of ovarian tumour diagnosed at Medical college for women, Uttara and Nightingale medical college, Ashulia. The study was approved by the Institutional Review Board (IRB) and 600 patients diagnosed with ovarian tumors were consecutively included in the study from July 2009 to August 2016. The cases were diagnosed and subclassified in accordance with the WHO Classification of Female Genital Tumours, Fourth Edition. Statistical Package for Social Sciences (SPSS) application, version 25, was used for data analysis. Results: Out of Total Gynaecological admission of 4800 patients during the study period, 600 patients were admitted with diagnosis of ovarian Tumor, So occurance was 12.5%. the mean age of the patients was 35 ± 2.09. About 8.6% of the patients had less than 20 years old. 56.6% were within the age group of 21-40, 29.8% were within the age group of 41-60 and only 5% had more than 60 years. The mean Menarche in years was 14.6 ± 1.02 and Menopause in years was 48.7 ± 2.46. Regarding clinical presentation 37.40% had abdominal pain, 55.50% had abdominal mass, 3% had abdominal distension,2% had ascites, 0.50% had menstrual irregularities and 2.50% were asymptomatic. Regarding surgical procedure, 35% undergone Total abdominal hysterectomy and bilateral salpingo-oophorectomy, 18.33% Bilateral salpingo-oophorectomy, 15% Right salpingo-oophorectomy and Left salpingo-oophorectomy recommendation, 13.33% Right cystectomy, 3.33% Left cystectomy. Regarding consistency of tumour 57.33% were cystic, 37% were solid, 5.33% were complex and 0.33% were Diffuse pattern (metastatic tumor). Benign tumors were 77%, borderline 16.67% and malignant 6.33% cases. Out of 462 benign tumors, Serous cystadenoma (230/49.78%), Mucinous cystadenoma (60/12.98%), benign Brenner tumor (10/2.16%), Mature cystic teratoma (110/23.80%), stroma ovarii (10/2.16%), Fibroma (10/2.16%), Fibro-thecoma (12/ 2.59%), leiomyoma (20/4.34%). Out of 38 malignant tumour, Serous cystadenocarcinoma (11/28.94%), mucinous cystadenocarcinoma (6/15.78%), clear cell carcinoma (1/2.63%), Malignant Brenner tumor (1/2.63%), endometroid adenocarcinoma (4/10.52%), squamous cell carcinoma arising in mature teratoma (1/2.63%), Dysgerminoma (7/18.4%), Immature teratoma (1/2.63%), malignant mixed germ cell tumor (1/2.63%), Adult granulosa cell tumor (4/10.52%), Krukenburg tumor (1/2.63%). Survival status was satisfactory; 99.67% patients was alive. Conclusion: The study found that ovarian neoplasms had ambiguous signs and symptoms, were mostly seen in reproductive age groups, and were mostly benign. The proportion of malignant ovarian neoplasms was significantly lower than that of benign ovarian neoplasms. Although incidence of malignant tumor is less common but gynocologists should be more careful in diagnosis of malignancy as ovaries are pelvic organs and definite screening methods are not available and malignancy can occur in any age.

Obstrucción intestinal por fecaloma gigante en adolescente con enfermedad de Hirschsprung

Hirschsprung’s disease (HD) is uncommon, and most of the cases occur in neonates. It is rarely diagnosed during adolescence, and even fewer cases are documented. We present the case of a 16-year-old male who had 15 days of constipation, anorexia, postprandial vomiting and abdominal distension. Imaging examinations showed a massive fecaloma obstructing the colon, and histopathological examinations showed HD. Given the failure of conservative evacuation measures, an extended left hemicolectomy and colostomy were performed, plus appendectomy for bowel obstruction. The patient was discharged with antibiotic therapy. This case highlights the importance of considering HD in adolescents with chronic constipation and abdominal masses. Although patients may experience postoperative problems, surgery is the most effective treatment for HD.

“Duplex sans duplex: a cryptic cause”: a case report

BackgroundDuplex kidneys represent an embryologic maldevelopment at time of renogenesis resulting in a spectrum of bifurcation anomalies of the reno-ureteric system. Though most are antenatally detected, recurrent urinary tract infections (UTIs), abdominal mass due to obstruction and incontinence are other common manifestations. Upper moiety ureter is usually obstructed and the lower moiety is refluxing. Management is guided by the percentage function of each of the moieties. A non-functioning system warrants a heminephrectomy. We report a toddler with right flank mass and a provisional diagnosis of right duplex system following investigations but met with a cryptic cause at surgery thereby altering the management.Case presentationA 2 ½ years girl with progressively increasing right abdominal mass for 6 weeks was found to have 12 × 10 cm right non-tender flank mass. Ultrasonography, contrast tomography and nuclear scans showed a right duplex system with obstructed, poorly functioning lower moiety. A lower moiety heminephrectomy was planned but at surgery, a densely adherent cystic structure displacing the right kidney superiorly was noted. On decompressing, the ureter was found to enter the cyst with discontinuation for a length of 6cms before being traced distally to its entry into the bladder. Retrograde pyelogram confirmed mid-ureteral transection and cystic urinoma. The cyst was excised and the ureter reconstructed with an appendicular interposition graft. Child recovered uneventfully and at 8 months follow up is well with good drainage across the conduit.ConclusionThe case highlights a rare presentation of mid-ureteral transection with urinoma masquerading as a duplex system and its satisfactory management.

A gist on an obscure neoplasm in Ghana: gastrointestinal stromal tumours

BackgroundGastrointestinal Stromal Tumour is a rare but potentially curable tumour of the gastrointestinal tract accounting for up to 1% of all gastrointestinal tumours. The discovery of Imatinib mesylate, a novel tyrosine kinase inhibitor has improved the chances even for unresectable, recurrent, or metastatic diseases.MethodsThis study sought to document the clinical and pathological characteristics of GISTs from two tertiary hospitals in Ghana that have undergone immunohistochemistry confirmation between 2014 and 2021.ResultsThe median age of the subjects was 50 years with most of them (28.0%) being above 61 years. There were more females than males (64.0% vs. 36.0%). Abdominal mass and abdominal pain made up the majority of the clinical presentations. The majority of the subjects had partial gastrectomy (32.0%) which was followed by wedge resection (28.0%). Appendectomy and sleeve gastrectomy were the least performed procedures (8% each). Four of the 25 patients (16.0%) had resections of involved contiguous organs done with splenectomy being the most common procedure. The majority of GISTs were found in the stomach (68.0%) followed by the appendix (12.0%) and small bowel (12.0%). Gastrointestinal bleeding (55.8%) and abdominal pain (38.5%) were the most reported symptoms. Free resection margins were observed in 84.0% of the subjects and only 3/25 (12.0%) experienced tumour recurrence.ConclusionGIST is a potentially curable tumour that once was obscure but currently gaining popularity. Surgical resection offers the hope of a cure for localized disease while targeted therapies is a viable option for recurrent, metastatic, or unresectable tumours.

Safe and Effective Escalation of Evidence-Based Chemotherapy for Mature B Cell Non-Hodgkin's Lymphoma in a Low Resource Setting

BACKGROUND Although high intensity chemotherapy regimens result in >90% long term survival of children with Mature B-cell Non-Hodgkin Lymphoma (MB-NHL), low intensity chemotherapy regimens are used in most Sub-Sahara African (SSA) setting resulting in decades of less than 20% long term survival. This is in part because dose-intense chemotherapy strategies that are standard of care in high income countries (HIC) have not been widely or effectively implemented in low- and middle income countries (LMIC). We investigated the toxicity and outcomes of children with MB-NHL who were treated with a treatment regimen that includes 1g/M2 of methotrexate per dose (mini COPADM) at Mulago National Referral Hospital in Uganda. The objective of this study was to evaluate the safety and effectiveness of an incremental chemotherapy escalation strategy to control treatment-related toxicities and increase response and survival of children treated for MB-NHL at a pediatric oncology center in the SSA setting. METHODS We prospectively collected clinical outcomes on children under 18 years with MB-NHL at Mulago National Referral Hospital (MNRH) in Uganda between January 2020 and July 2023. Overall (OS) and event-free survival (EFS) were calculated using the Kaplan-Meier method. Events in the EFS analysis were: 1) Death; 2) Relapse; or 3) Persistent or progressive disease at the end of Cycle 3. Patients were censored at the earlier of two years from presentation or their last day of follow-up. Descriptive statistics were presented. Toxicities were graded according to Common Terminology Criteria for Adverse Events (CTCAE) version 5. RESULTS We identified 77 patients, among whom 16 (21%) died prior to commencement of the first cycle of chemotherapy and 6 (8%) treated with alternative therapies. The 55 patients who were treated with mini COPADM with 1g/M 2 of methotrexate and are included in this analysis had similar demographics and clinical phenotypes to the 22 that were excluded. The median age was 9.6 years (interquartile range ((IQR) 6.6 - 11.7 years) with 38 (69%) males. Patients were Stage III (53%, 29/55), stage IV (38%, 21/55); and 55%, (30/55) were high-risk. The most common presentations were abdominal mass 43 (78%) and jaw mass 14 (25%). With a median follow-up time of 9 months (IQR 6 - 24 months), the 1-year overall survival (OS) and event free survival (EFS) were 56%; 95% CI (41 - 76) and 41%; 95%CI (27 - 61) respectively. For children with high-risk disease, 1-year OS was 44%; 95%CI (28 - 72) and EFS 25%; 95% CI (12 - 51). Toxicities included febrile neutropenia (83%) and CTCAE grade ≥3 mucositis (44%). For the 12 patients whose cause of mortality was recorded, 1 case (8.3%) was treatment-related and 11 (91.7%) were due to cancer. CONCLUSION: This study establishes baseline response rates, survival, and toxicity for patients with pediatric MB-NHL in Uganda treated with 1g/M2 of methotrexate. Toxicities were consistent with treatment received, perhaps exacerbated by synergy of anthracycline (50 mg/m2) along with intermediate dose MTX. These findings suggest that mini-COPADM with 1g/M 2 of methotrexate can be safely supported with the current clinical resources at MNRH and this low dose regimen accounts for the poor survival of children with MB-NHL in our center. We plan to escalate our therapy to mini-COAPDM with 3g/M2 and addition of Rituximab, with contingent intensification of supportive care to curb treatment-related toxicities.

Association of body fat distribution with all-cause and cardiovascular mortality in US adults: a secondary analysis using NHANES

ObjectiveTo investigate the association of fat and lean mass in specific regions with all-cause and cardiovascular-related mortality.DesignPopulation based cohort study.SettingUS National Health and Nutrition Examination Survey (2003–2006 and 2011–2018).Participants22 652...

Rare giant renal artery aneurysm in neurofibromatosis type 1 patient: a case report

Introduction and importance: Neurofibromatosis type 1 (NF1) is a genetic disorder characterised by multiple neurofibromas, café-au-lait spots, and iris hamartomas. The variety of vasculopathies that can occur in NF1 make it difficult for clinicians to accurately follow-up patients. Most cases of vasculopathies are stenotic, and, in few cases, aneurysms may form. Case presentation: A 35-year-old male presented with extreme left flank pain for the past 2 days. His physical examination revealed whole-body several café-au-lait skin macules, a subcutaneous lesion, and a palpable abdominal mass in the left flank. His laboratory workup was within normal ranges. A multi-slice computed tomography and computed tomography angiogram with contrast outlined a giant left renal artery aneurysm (RAA). A kidney salvage surgery was planned. However, due to ectatic dilatation and large extension of the aneurysm, the affected renal artery branches and renal vein were found unfit for auto-transplantation during the surgical procedure and a total nephrectomy was necessary. Symptoms improved significantly postoperatively and no complications developed. Clinical discussion: RAA is an uncommon finding in NF1 patients. Diagnosis is often dependent on computed tomography angiogram. Management techniques are conservative, endovascular, or surgical. In few surgical cases, a total nephrectomy may be necessary if auto-transplantation is not feasible. Conclusion: Despite its rarity, the diagnosis of RAA should be considered in patients with NF1 presenting with flank pain. Moreover, early screening for renal vasculopathies can evade critical surgical outcomes including a total nephrectomy. Hence, the authors recommend a total vascular workup for these patients, consisting of doppler ultrasound and, if necessary, a multi-slice computed tomography with contrast.

Hepatic Activin E mediates liver-adipose inter-organ communication, suppressing adipose lipolysis in response to elevated serum fatty acids

ObjectiveThe liver is a central regulator of energy metabolism exerting its influence both through intrinsic processing of substrates such as glucose and fatty acid as well as by secreting endocrine factors, known as hepatokines, which influence metabolism in peripheral tissues. Human genome wide association studies indicate that a predicted loss-of-function variant in the Inhibin βE gene (INHBE), encoding the putative hepatokine Activin E, is associated with reduced abdominal fat mass and cardiometabolic disease risk. However, the regulation of hepatic Activin E and the influence of Activin E on adiposity and metabolic disease are not well understood. Here, we examine the relationship between hepatic Activin E and adipose metabolism, testing the hypothesis that Activin E functions as part of a liver-adipose, inter-organ feedback loop to suppress adipose tissue lipolysis in response to elevated serum fatty acids and hepatic fatty acid exposure. MethodsThe relationship between hepatic Activin E and non-esterified fatty acids (NEFA) released from adipose lipolysis was assessed in vivo using fasted CL 316,243 treated mice and in vitro using Huh7 hepatocytes treated with fatty acids. The influence of Activin E on adipose lipolysis was examined using a combination of Inhbe knockout mice, a mouse model of hepatocyte-specific overexpression of Activin E, and mouse brown adipocytes treated with Activin E enriched media. ResultsIncreasing hepatocyte NEFA exposure in vivo by inducing adipose lipolysis through fasting or CL 316,243 treatment increased hepatic Inhbe expression. Similarly, incubation of Huh7 human hepatocytes with fatty acids increased expression of INHBE. Genetic ablation of Inhbe in mice increased fasting circulating NEFA and hepatic triglyceride accumulation. Treatment of mouse brown adipocytes with Activin E conditioned media and overexpression of Activin E in mice suppressed adipose lipolysis and reduced serum FFA levels, respectively. The suppressive effects of Activin E on lipolysis were lost in CRISPR-mediated ALK7 deficient cells and ALK7 kinase deficient mice. Disruption of the Activin E-ALK7 signaling axis in Inhbe KO mice reduced adiposity upon HFD feeding, but caused hepatic steatosis and insulin resistance. ConclusionsTaken together, our data suggest that Activin E functions as part of a liver-adipose feedback loop, such that in response to increased serum free fatty acids and elevated hepatic triglyceride, Activin E is released from hepatocytes and signals in adipose through ALK7 to suppress lipolysis, thereby reducing free fatty acid efflux to the liver and preventing excessive hepatic lipid accumulation. We find that disrupting this Activin E-ALK7 inter-organ communication network by ablation of Inhbe in mice increases lipolysis and reduces adiposity, but results in elevated hepatic triglyceride and impaired insulin sensitivity. These results highlight the liver-adipose, Activin E-ALK7 signaling axis as a critical regulator of metabolic homeostasis.