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World Journal of Surgical Oncology Apr 2024The objective of this paper is to offer a thorough examination of the clinical presentations, etiology, and treatment strategies associated with perivascular epithelioid... (Review)
Review
PURPOSE
The objective of this paper is to offer a thorough examination of the clinical presentations, etiology, and treatment strategies associated with perivascular epithelioid cell tumors (PEComas).
METHODS
This retrospective study examined the comprehensive archival data of PEComa cases diagnosed at Beijing Hospital from 2015 to 2023. The pathology slides of all patients were thoroughly reassessed by two experienced pathologists. A thorough retrospective analysis was undertaken, incorporating clinicopathological data including gender, age at diagnosis, initial clinical manifestations, signs, disease onset site, tumor markers, imaging findings, therapeutic modalities, pathological features, immunohistochemical profiles, treatment responses, and prognostic indicators. Patients were evaluated for disease severity according to established pathological classification criteria and were followed up until the designated analysis cut-off date. In instances where patients were unable to be monitored on-site, they were contacted via telephone for postoperative follow-up inquiries.
RESULTS
This study included 11 patients with ages ranging from 17 to 66 years old, presenting with the disease in multiple anatomical sites, including the retroperitoneum (2/11), liver (4/11), kidney (4/11), lung (1/11), and broad ligament of the uterus (1/11). Most patients presented with non-specific clinical symptoms and were subsequently diagnosed with space-occupying lesions upon physical examination. The tumor demonstrated progressive growth and enlargement, which could result in compression of neighboring organs. Preoperative imaging alone is insufficient for a definitive diagnosis of PEComa, but MRI can provide an initial evaluation of the tumor's potential malignancy. Molecular marker testing specific to PEComa, such as HMB-45 (90.0%), SMA (81.8%), Melan-A (90.9%), vimentin (90.9%), and Desmin (36.3%), was conducted on all patients. No adjuvant therapies were administered postoperatively. Upon analysis, no instances of relapse at the primary site or the development of new tumors at other sites were observed. Regular imaging reviews of three patients with malignant PEComa post-surgery showed no evidence of recurrence.
CONCLUSIONS
The clinical presentation, tumor biomarkers, and imaging characteristics of PEComa lack specificity, necessitating dependence on pathology and immunohistochemistry for precise diagnosis. The mainstay of treatment consists of surgical resection, with patients typically experiencing a favorable prognosis.
Topics: Humans; Perivascular Epithelioid Cell Neoplasms; Female; Retrospective Studies; Middle Aged; Male; Adult; Aged; Adolescent; Young Adult; Prognosis; Follow-Up Studies; Biomarkers, Tumor; Lung Neoplasms; Kidney Neoplasms; Liver Neoplasms; Retroperitoneal Neoplasms
PubMed: 38689335
DOI: 10.1186/s12957-024-03349-x -
Case Reports in Women's Health Jun 2024Cotyledonoid dissecting leiomyoma (CDL) is a rare uterine tumor with unique clinical and histological features. We present a case of a 46-year-old woman with a 3-month...
Cotyledonoid dissecting leiomyoma (CDL) is a rare uterine tumor with unique clinical and histological features. We present a case of a 46-year-old woman with a 3-month history of left-flank pain radiating to the back. The patient had a history of infertility and a previous miscarriage. Ultrasound revealed a solid tissue mass suggestive of a degenerated fibroid. Laparoscopy identified subserosal leiomyoma and leiomyoma in the broad ligament. Histologically, CDL is characterized by disorganized smooth muscle with hyaline degeneration and no evidence of malignancy. Clinically, CDL can present with a variety of symptoms, including heavy menstrual bleeding, pelvic pain, and infertility. The coexistence of CDL and adenomyosis is exceedingly rare. This case highlights the importance of considering CDL in the differential diagnosis of pelvic mass, malignant neoplasms, and infertility, even with atypical symptoms. It also emphasizes the value of cooperation between clinicians and pathologists for accurate diagnosis and management of CDL. Adenomyosis in this case further complicated the diagnosis and highlighted the need for an index of suspicion for this rare condition.
PubMed: 38646502
DOI: 10.1016/j.crwh.2024.e00604 -
Medicine Apr 2024Adnexal torsion (AT) is one of a gynecological condition characterized by an acute abdomen. Clinically, a giant ovarian cyst torsion with a diameter of 30 cm is rare....
INTRODUCTION
Adnexal torsion (AT) is one of a gynecological condition characterized by an acute abdomen. Clinically, a giant ovarian cyst torsion with a diameter of 30 cm is rare. Therefore, an accurate and timely diagnosis and treatment are important.
PATIENT CONCERNS
A 25-year-old unmarried female, presented to the emergency department with intermittent abdominal cramps after a sudden change in position. Considering her symptoms and examination, ultrasound, and magnetic resonance imaging (MRI) results, ovarian cyst torsion was suspected.
DIAGNOSIS
Giant ovarian cyst torsion.
INTERVENTIONS
Surgical intervention with exploratory laparotomy was performed immediately.
OUTCOMES
Intraoperatively, we found a 30-cm left ovarian cyst with a clear root. The left fallopian tube, infundibulopelvic ligament, and ovarian ligament were twisted 900 degrees. Finally, the pathological report revealed mucinous cystadenoma.
CONCLUSION
Giant ovarian cyst torsion with a diameter of 30 cm is rare. Considering her symptoms and examination, ultrasound, and MRI results, ovarian cyst torsion was suspected. The patient was successfully treated using emergency surgery.
Topics: Humans; Female; Adult; Abdomen, Acute; Broad Ligament; Cystadenoma, Mucinous; Ovarian Cysts
PubMed: 38608053
DOI: 10.1097/MD.0000000000033283 -
Bone Research Apr 2024Ossification of the Posterior Longitudinal Ligament (OPLL) is a degenerative hyperostosis disease characterized by the transformation of the soft and elastic vertebral...
Ossification of the Posterior Longitudinal Ligament (OPLL) is a degenerative hyperostosis disease characterized by the transformation of the soft and elastic vertebral ligament into bone, resulting in limited spinal mobility and nerve compression. Employing both bulk and single-cell RNA sequencing, we elucidate the molecular characteristics, cellular components, and their evolution during the OPLL process at a single-cell resolution, and validate these findings in clinical samples. This study also uncovers the capability of ligament stem cells to exhibit endothelial cell-like phenotypes in vitro and in vivo. Notably, our study identifies LOXL2 as a key regulator in this process. Through gain-and loss-of-function studies, we elucidate the role of LOXL2 in the endothelial-like differentiation of ligament cells. It acts via the HIF1A pathway, promoting the secretion of downstream VEGFA and PDGF-BB. This function is not related to the enzymatic activity of LOXL2. Furthermore, we identify sorafenib, a broad-spectrum tyrosine kinase inhibitor, as an effective suppressor of LOXL2-mediated vascular morphogenesis. By disrupting the coupling between vascularization and osteogenesis, sorafenib demonstrates significant inhibition of OPLL progression in both BMP-induced and enpp1 deficiency-induced animal models while having no discernible effect on normal bone mass. These findings underscore the potential of sorafenib as a therapeutic intervention for OPLL.
Topics: Animals; Longitudinal Ligaments; Osteogenesis; Sorafenib; Ossification of Posterior Longitudinal Ligament; Cell Differentiation
PubMed: 38594260
DOI: 10.1038/s41413-024-00327-7 -
Perioperative Medicine (London, England) Apr 2024Unanticipated symptoms of peripheral nerve damage following surgery are distressing to both the patient and their clinical team, including surgeons, anesthesiologists,...
BACKGROUND
Unanticipated symptoms of peripheral nerve damage following surgery are distressing to both the patient and their clinical team, including surgeons, anesthesiologists, and neurologists. The causes that are commonly considered for perioperative neuropathy can include surgical trauma, positioning-related injury, or injury related to a regional anesthetic technique. However, these cases often do not have a clear etiology and can occur without any apparent periprocedural anomalies. Postoperative inflammatory neuropathy is a more recently described, and potentially underrecognized cause of perioperative neuropathy which may improve with corticosteroid therapy. Therefore, it is an important etiology to consider early in the evaluation of perioperative neuropathy.
CASE PRESENTATION
An otherwise healthy patient presented for left anterior cruciate ligament reconstruction. He underwent femoral and sciatic ultrasound-guided single-injection peripheral nerve blocks preoperatively, followed by a general anesthetic for the surgical procedure. He developed postoperative neuropathy in the sciatic distribution with both sensory and motor deficits. The patient received multi-disciplinary consultations, including neurology and pain management, and a broad differential diagnosis was considered. Based on neurological evaluation and imaging studies, a final diagnosis of post-surgical inflammatory neuropathy was made. The patient's course improved with conservative management, but immunosuppressive treatment may have been considered for a more severe or worsening clinical course.
CONCLUSIONS
There are limited publications describing postoperative inflammatory neuropathy, and this case serves to illustrate a potentially under-recognized and multifactorial cause of postoperative neuropathy. Perioperative neuropathies are a complication that surgeons and anesthesiologists strive to avoid; however, prevention and treatment of this condition have been elusive. Increased reporting and investigation of postoperative inflammatory neuropathy as one cause for this complication will help to further our understanding of this potentially devastating complication.
PubMed: 38566245
DOI: 10.1186/s13741-024-00384-w -
Frontiers in Sports and Active Living 2024Adolescent athletes involved in sports that involve cutting and landing maneuvers have an increased risk of anterior cruciate ligament (ACL) tears, highlighting the...
INTRODUCTION
Adolescent athletes involved in sports that involve cutting and landing maneuvers have an increased risk of anterior cruciate ligament (ACL) tears, highlighting the importance of identifying risky movement patterns such as dynamic knee valgus (DKV). Qualitative movement screenings have explored two-dimensional (2D) scoring criteria for DKV, however, there remains limited data on the validity of these screening tools. Determining a 2D scoring criterion for DKV that closely aligns with three-dimensional (3D) biomechanical measures will allow for the identification of poor knee position in adolescent athletes on a broad scale. The purpose of this study was to establish a 2D scoring criterion that corresponds to 3D biomechanical measures of DKV.
METHODS
A total of 41 adolescent female club volleyball athletes performed a three-task movement screen consisting of a single-leg squat (SLS), single-leg drop landing (SLDL), and double-leg vertical jump (DLVJ). A single rater scored 2D videos of each task using four criteria for poor knee position. A motion capture system was used to calculate 3D joint angles, including pelvic obliquity, hip adduction, knee abduction, ankle eversion, and foot progression angle. Receiver operating characteristic curves were created for each 2D scoring criterion to determine cut points for the presence of movement faults, and areas under the curve (AUC) were computed to describe the accuracy of each 2D criterion compared to 3D biomechanical data.
RESULTS
3D measures indicated knee abduction angles between 2.4°-4.6° (SD 4.1°-4.3°) at the time point when the center of the knee joint was most medial during the three tasks. AUCs were between 0.62 and 0.93 across scoring items. The MEDIAL scoring item, defined as the knee joint positioned inside the medial border of the shoe, demonstrated the greatest association to components of DKV, with AUCs ranging from 0.67 to 0.93.
CONCLUSION
The MEDIAL scoring criterion demonstrated the best performance in distinguishing components of DKV, specifically pelvic obliquity, hip adduction, ankle eversion, and foot progression. Along with the previously published scoring definitions for trunk-specific risk factors, the authors suggest that the MEDIAL criterion may be the most indicative of DKV, given an association with 3D biomechanical risk factors.
PubMed: 38558858
DOI: 10.3389/fspor.2024.1352286 -
Diagnostics (Basel, Switzerland) Mar 2024A 12-year-old adolescent was diagnosed with a right-sided solid mass in the round ligament of the uterus. The chief complaints were abdominal pain and pelvic discomfort....
A 12-year-old adolescent was diagnosed with a right-sided solid mass in the round ligament of the uterus. The chief complaints were abdominal pain and pelvic discomfort. She underwent laparoscopic tumor resection. Histological examination demonstrated a trabecular growth pattern of epithelioid cells with mitotic activity (3 per 50 HPF), which expressed melanocytic and myoid markers. Due to aforementioned findings, a final diagnosis of perivascular epithelioid cell tumor (PEComa) with uncertain malignant potential was made. To the best of our knowledge, this localization of PEComa is considered to be infrequent with only occasionally reported cases.
PubMed: 38535035
DOI: 10.3390/diagnostics14060616 -
Contraception: X 2024Document the clinical outcomes of an outpatient medical management with procedural evacuation backup procedure for abortions between 18 weeks zero days to 23 weeks six...
OBJECTIVE
Document the clinical outcomes of an outpatient medical management with procedural evacuation backup procedure for abortions between 18 weeks zero days to 23 weeks six days gestation.
STUDY DESIGN
We conducted a retrospective medical records review of adult patients who received mifepristone and repeated misoprostol for second trimester abortion with procedural evacuation backup at an Arizona clinic between October 2017 and November 2021. We extracted patient demographics; pregnancy and medical history; and preoperative, intraoperative, and postoperative data. We assessed abortion outcomes, including procedure timing, mode of completion (medication alone or medications and procedural evacuation), and safety.
RESULTS
All 359 patients had a complete abortion with 63.5% of patients completing with medication alone and 36.5% with procedural evacuation backup. The median time from first dose of misoprostol to fetal expulsion was six hours, among those who completed the abortion with medications alone. Of those who received procedural evacuation as backup, the median time for procedural evacuation was 10 minutes. The vast majority of patients (99.4%) did not have any adverse events. Two safety incidents (0.6%) occurred, a broad right ligament tear and a uterine rupture.
CONCLUSION
Patients in one outpatient setting safely and effectively received medical management of second trimester abortion with procedural evacuation backup, and two thirds completed with medications alone.
IMPLICATIONS
Outpatient settings may consider medical management of abortion between 18 and 24 weeks with procedural evacuation back-up as a safe, effective, and manageable second trimester abortion option. Additional research is needed on patient experience and satisfaction.
PubMed: 38515629
DOI: 10.1016/j.conx.2024.100104