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Radiographics : a Review Publication of... 2018Von Hippel-Lindau syndrome (VHL) is an autosomal-dominant hereditary tumor disease that arises owing to germline mutations in the VHL gene, located on the short arm of... (Review)
Review
Von Hippel-Lindau syndrome (VHL) is an autosomal-dominant hereditary tumor disease that arises owing to germline mutations in the VHL gene, located on the short arm of chromosome 3. Patients with VHL may develop multiple benign and malignant tumors involving various organ systems, including retinal hemangioblastomas (HBs), central nervous system (CNS) HBs, endolymphatic sac tumors, pancreatic neuroendocrine tumors, pancreatic cystadenomas, pancreatic cysts, clear cell renal cell carcinomas, renal cysts, pheochromocytomas, paragangliomas, and epididymal and broad ligament cystadenomas. The VHL/hypoxia-inducible factor pathway is believed to play a key role in the pathogenesis of VHL-related tumors. The diagnosis of VHL can be made clinically when the characteristic clinical history and findings have manifested, such as the presence of two or more CNS HBs. Genetic testing for heterozygous germline VHL mutation may also be used to confirm the diagnosis of VHL. Imaging plays an important role in the diagnosis and surveillance of patients with VHL. Familiarity with the clinical and imaging manifestations of the various VHL-related tumors is important for early detection and guiding appropriate management. The purpose of this article is to discuss the molecular cytogenetics and clinical manifestations of VHL, review the characteristic multimodality imaging features of the various VHL-related tumors affecting multiple organ systems, and discuss the latest advances in management of VHL, including current recommendations for surveillance and screening. RSNA, 2018 An earlier incorrect version of this article appeared online. This article was corrected on April 9, 2018.
Topics: Diagnosis, Differential; Humans; Mass Screening; Multimodal Imaging; Population Surveillance; von Hippel-Lindau Disease
PubMed: 29601266
DOI: 10.1148/rg.2018170156 -
AJP Reports Jan 2021Broad ligament hematomas are rare in the setting of vaginal delivery. When they do occur, patients typically present with acute hemodynamic instability. No cases of...
Broad ligament hematomas are rare in the setting of vaginal delivery. When they do occur, patients typically present with acute hemodynamic instability. No cases of infected broad ligament hematomas have been reported. A 22-year-old G2 P1011 status post vaginal delivery complicated by chorioamnionitis and pre-eclampsia presented 5 days postpartum with subjective complaints of fever, vomiting, and increased vaginal bleeding. She was treated with antibiotics and uterine evacuation was planned for presumed retained products. After dilation and curettage, the patient was transferred to our facility, as her clinical status did not improve, and was later found to have an infected broad ligament hematoma requiring hysterectomy. Though uncommon, broad ligament hematomas should be considered in postpartum women presenting with anemia and vaginal bleeding, even without hemodynamic instability. Recent intrauterine infections may predispose to hematoma infection.
PubMed: 33614197
DOI: 10.1055/s-0040-1722727 -
Medicine Sep 2018Solitary fibrous tumors (SFTs) are uncommon mesenchymal neoplasms and are particularly rare in the female genital tract. Doege-Potter syndrome is a paraneoplastic... (Review)
Review
INTRODUCTION
Solitary fibrous tumors (SFTs) are uncommon mesenchymal neoplasms and are particularly rare in the female genital tract. Doege-Potter syndrome is a paraneoplastic syndrome involving SFT-associated hypoglycemia. We report, for the first time, on a broad ligament SFT with Doege-Potter syndrome; additionally, we review 30 cases of women with SFTs reported in the literature.
PATIENT CONCERNS
A 37-year-old woman who presented with life-threatening hypoglycemia and a pelvic mass (16 × 15 × 15 cm).
DIAGNOSES
The patient was diagnosed with broad ligament SFT with Doege-Potter syndrome.
INTERVENTIONS
Tumor resection, sub-extensive hysterectomy, bilateral salpingo-oophorectomy, and pelvic lymphadenectomy were performed, and 6 cycles of adjuvant chemotherapy were administered.
OUTCOMES
Serum glucose levels returned to normal as soon as the tumor was resected. Forty-3 months after operation, there was recurrence in the posterior peritoneal tissues. She underwent tumor resection and has remained tumor-free 28 months after this excision.
CONCLUSION
Even though it is extremely rare, SFT should be quickly identified to prevent undue treatment delay and avoid unnecessary examination; surgery and long-term follow-up are recommended. SFT can be considered a highly invasive cancer, and intraoperative bleeding may occur. Although no correlation between adjuvant therapy and improved prognosis was found, further studies are required because of the small number of cases reported to date.
Topics: Adnexal Diseases; Adult; Broad Ligament; Chemotherapy, Adjuvant; Female; Humans; Hypoglycemia; Hysterectomy; Lymph Node Excision; Ovariectomy; Paraneoplastic Syndromes; Salpingectomy; Solitary Fibrous Tumors
PubMed: 30278559
DOI: 10.1097/MD.0000000000012564 -
Orthopaedics & Traumatology, Surgery &... Apr 2021Abundant anatomic descriptions exist of the posterior cruciate ligament (PCL) and menisco-femoral ligaments (MFLs). There is broad agreement that the PCL is composed of...
BACKGROUND
Abundant anatomic descriptions exist of the posterior cruciate ligament (PCL) and menisco-femoral ligaments (MFLs). There is broad agreement that the PCL is composed of two bundles and inserts on the femur near the distal cartilage. However, a different configuration with a single bundle and a complex femoral insertion has been reported. The main objective of our cadaver study was to determine the number of anatomical bundles forming the PCL. We also described the insertion of the PCL and its relationships with the anterior menisco-femoral ligament (AMFL).
HYPOTHESIS
The PCL consists of a single ribbon-like bundle and inserts on the femur separately from the AMFL.
MATERIAL AND METHODS
We used 23 knees of fresh unembalmed cadavers, which we dissected under a microsurgery loupe. Inclusion criteria for the knees were freedom from trauma and scars. No age limits were set. The features of the PCL were studied using the clock method. Measurements were taken using callipers with a precision of 0.01mm.
RESULTS
No knees were excluded from the final analysis. Macroscopically, the PCL appeared as a single bundle forming a slender flat ribbon. With the knee flexed at 90°, the ligament footprint extended from 58min to 25min on the right and from 48min to 2min on the left. The femoral insertion of the AMFL started 2.07mm from the distal cartilage and masked the distal insertion of the PCL. After removal of the AMFL, the middle of the femoral insertion of the PCL started on average 5.99mm from the distal cartilaginous rim when the knee was flexed at 90°.
DISCUSSION
Our study confirms descriptions of the PCL as a single bundle shaped as a flat ribbon. The presence of the AMFL gives the appearance of a double bundle and partially masks the femoral insertion of the PCL, which is located further back relative to the distal joint cartilage.
LEVEL OF EVIDENCE IV
Experimental cadaver study.
Topics: Cadaver; Femur; Humans; Knee; Knee Joint; Posterior Cruciate Ligament
PubMed: 33333270
DOI: 10.1016/j.otsr.2020.102788 -
Cureus Mar 2022Leiomyomas are a common gynaecological finding affecting 20-30% of women over the age of 35, with prevalence decreasing following menopause. Around 25% of women present...
Leiomyomas are a common gynaecological finding affecting 20-30% of women over the age of 35, with prevalence decreasing following menopause. Around 25% of women present clinically with a leiomyoma, which are most commonly found within the uterus. Rarer extra-uterine locations include the broad ligament, cervix, and vagina. We present a rare case of an extra-uterine leiomyoma located in the perineum of a 59-year-old female. Our case highlights the importance for extra-uterine leiomyoma to be considered as a differential diagnosis in patients presenting with a pelvic mass following hysterectomy.
PubMed: 35464590
DOI: 10.7759/cureus.23116 -
Clinical Case Reports Sep 2022Extra-uterine leiomyoma (EUL) is extremely rare and due to its unusual location, results into diagnostic dilemma and due to its unusual location may lead to surgical...
Extra-uterine leiomyoma (EUL) is extremely rare and due to its unusual location, results into diagnostic dilemma and due to its unusual location may lead to surgical complication as in this case. We present a case of large broad ligament leiomyoma in a 40-year-old premenauposal that was excised with challenges.
PubMed: 36177066
DOI: 10.1002/ccr3.6350 -
Orthopaedic Journal of Sports Medicine Dec 2021Although most anterior cruciate ligament (ACL) injuries occur in male athletes, female athletes are consistently observed to be at a higher risk for sports-specific ACL... (Review)
Review
BACKGROUND
Although most anterior cruciate ligament (ACL) injuries occur in male athletes, female athletes are consistently observed to be at a higher risk for sports-specific ACL injury.
PURPOSE
To provide a thorough review of what is known about the sexual dimorphisms in ACL injury to guide treatment and prevention strategies and future research.
STUDY DESIGN
Narrative review.
METHODS
We conducted a comprehensive literature search for ACL-related studies published between January 1982 and September 2017 to identify pertinent studies regarding ACL injury epidemiology, prevention strategies, treatment outcomes, and dimorphisms. By performing a broad ACL injury search, we initially identified 11,453 articles. After applying additional qualifiers, we retained articles if they were published in English after 1980 and focused on sex-specific differences in any of 8 different topics: sex-specific reporting, difference in sports, selective training, hormonal effects, genetics, neuromuscular and kinematic control, anatomic differences, and outcomes.
RESULTS
A total of 122 articles met the inclusion criteria. In sum, the literature review indicated that female athletes are at significantly higher risk for ACL injuries than are their male counterparts, but the exact reasons for this were not clear. Initial studies focused on intrinsic differences between the sexes, whereas recent studies have shifted to focus on extrinsic factors to explain the increased risk. It is likely both intrinsic and extrinsic factors contribute to this increased risk, but further study is needed. In addition to female patients having an increased risk for ACL injuries, they are less likely than are male patients to undergo reconstructive surgery, and they experience worse postsurgical outcomes. Despite this, reconstructive surgery remains the gold standard when knee stability, return to sports, and high functional outcome scores are the goal, but further research is needed to determine why there is disparity in surgical rates and what surgical techniques optimize postsurgical outcomes for female patients.
CONCLUSION
Male athletes often predominated the research concerning ACL injury and treatment, and although sex-specific reporting is progressing, it has historically been deficient. ACL injuries, prevention techniques, and ACL reconstruction require further research to maximize the health potential of at-risk female athletes.
PubMed: 34993256
DOI: 10.1177/23259671211025304 -
Case Reports in Women's Health Oct 2022Leiomyomas are the most common benign tumours of the female genital tract, and almost always arise from the uterine myometrium. Although extrauterine leiomyomas are...
Leiomyomas are the most common benign tumours of the female genital tract, and almost always arise from the uterine myometrium. Although extrauterine leiomyomas are rare, they usually develop in sites such as the ovary, broad ligament, round ligament, cervix or abdominal wall. The broad ligament is the most common site of extrauterine leiomyoma involvement, and this unique clinical entity may prove to be a diagnostic or therapeutic challenge, particularly in patients with advanced endometriosis and distorted pelvic anatomy. Herein, we report the case of a large true broad ligament leiomyoma that was discovered during a total laparoscopic hysterectomy and bilateral salpingo-oophorectomy in a 47-year-old patient with stage IV endometriosis and a congenital left kidney and left ureter. This case highlights a rare occurrence of a true broad ligament leiomyoma, the challenges associated with preoperative diagnosis, and the laparoscopic approach to its management.
PubMed: 35991520
DOI: 10.1016/j.crwh.2022.e00436 -
BMC Surgery Nov 2020Leiomyosarcoma usually develops in the myometrium and is characterized by a high recurrence rate, frequent hematogenous dissemination, and poor prognosis. Metastasis is... (Review)
Review
BACKGROUND
Leiomyosarcoma usually develops in the myometrium and is characterized by a high recurrence rate, frequent hematogenous dissemination, and poor prognosis. Metastasis is usually to lungs, liver, and bone, and occasionally to the brain, but seldom to the head and neck region. Primary leiomyosarcoma very rarely arises in the broad ligament.
CASE PRESENTATION
A 54-year old woman presented to the otolaryngology department with a mass in the right posterior region of the neck 4 years after surgery for a primary leiomyosarcoma of the right broad ligament. The neck mass was removed and found to be a metastatic leiomyosarcoma. Leiomyosarcoma localizations in lungs and liver were absent. Morphological examination showed both the primary and the secondary leiomyosarcomas to have features of low-grade tumors. One year after excision of the neck mass, the patient presented with tachycardia. Echocardiography detected two intracardiac nodules suggestive of metastatic tumors. Chemotherapy was administered; the disease has been stable since then.
CONCLUSIONS
We report the first case of broad ligament leiomyosarcoma with the neck subcutaneous region being the first site of secondary involvement. We speculate that the Batson venous plexus might have been the pathway of dissemination.
Topics: Adnexal Diseases; Antineoplastic Agents; Broad Ligament; Echocardiography; Female; Genital Neoplasms, Female; Head and Neck Neoplasms; Heart Neoplasms; Humans; Leiomyosarcoma; Middle Aged; Tachycardia
PubMed: 33238975
DOI: 10.1186/s12893-020-00951-0 -
Frontiers in Cell and Developmental... 2023Curcumin has broad application prospects in the prevention and treatment of periodontal diseases. Periodontal ligament stem cell-derived extracellular vesicles...
Curcumin has broad application prospects in the prevention and treatment of periodontal diseases. Periodontal ligament stem cell-derived extracellular vesicles (PDLSC-EV) can effectively promote periodontal tissue regeneration and possess good drug delivery capability. Superior pharmacological effects can be exerted using PDLSC-EV as a curcumin carrier. In the present study, we constructed curcumin-primed PDLSCs-derived extracellular vesicles (Cur-PDLSC-EV) from cell culture supernatants of curcumin-pretreated PDLSCs by ultracentrifugation and investigated their effects on the proliferation, migration, and osteogenic ability of PDLSCs and the corresponding downstream molecular pathways. Both Cur-PDLSC-EV and PDLSC-EV promoted osteoblast proliferation and migration. Compared with PDLSC-EV, Cur-PDLSC-EV possessed a more potent pro-osteogenic ability. Moreover, the improved osteogenesis of Cur-PDLSC-EV was related to the activation of the Wnt/β-catenin signaling pathway. This study suggests that Cur-PDLSC-EV can promote osteogenic differentiation by activating Wnt/β-catenin, providing reference bases for the treatment of periodontal diseases.
PubMed: 37842095
DOI: 10.3389/fcell.2023.1225449