-
Medicine Mar 2017Lymphangioleiomyomatosis (LAM) is a rare disease affecting young women caused by abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs and... (Review)
Review
BACKGROUND
Lymphangioleiomyomatosis (LAM) is a rare disease affecting young women caused by abnormal proliferation of smooth muscle-like cells (LAM cells) in the lungs and extrapulmonary sites (extrapulmonary LAM). The objective of this case series is to demonstrate marked regression in 2 cases of retroperitoneal LAM after treatment with everolimus, an mTOR inhibitor.
METHODS
We enrolled 2 cases with large volume, extrapulmonary pelvic LAM, and evaluated them with contrast-enhanced abdominal computed tomographic (CT) scans at presentation and serially during treatment with everolimus. Results were objectively quantified using the Response Evaluation Criteria in Solid Tumors, RECIST, Version 1.1.
RESULTS
After 12 to 18 months of treatment with everolimus, both patients showed substantial reduction in the volume of their tumors. The first had about 50% regression of the pelvic LAM and renal angiomyolipoma (AML). The second patient had extensive abdomino-pelvic LAM which after treatment showed complete remission. Both patients have not demonstrated disease progression after nearly 4 and 2 years of follow-up, respectively.
CONCLUSIONS
This case series demonstrates the enormous value of mTOR inhibitors (specifically everolimus) in the management of extrapulmonary pelvic LAM, of which there is no effective treatment currently available.
Topics: Adult; Antineoplastic Agents; Everolimus; Female; Humans; Lymphangioleiomyomatosis; Middle Aged; Pelvic Neoplasms; Pelvis
PubMed: 28272193
DOI: 10.1097/MD.0000000000004562 -
JMIR MHealth and UHealth Jul 2019Mobile health (mHealth) technologies are increasingly used in various medical fields. However, the potential of mHealth to improve patient care in radiotherapy by...
BACKGROUND
Mobile health (mHealth) technologies are increasingly used in various medical fields. However, the potential of mHealth to improve patient care in radiotherapy by acquiring electronic patient reported outcome measures (ePROMs) during treatment has been poorly studied so far.
OBJECTIVE
The aim of this study was to develop and implement a novel Web app (PROMetheus) for patients undergoing radiotherapy. Herein, we have reported our experience with a focus on feasibility, patient acceptance, and a correlation of ePROMs with the clinical course of the patients.
METHODS
In the period between January and June 2018, 21 patients used PROMetheus to score side effects, symptoms, and quality of life-related parameters during and after their treatment. Items of the Patient Reported Outcome version of the Common Terminology Criteria for Adverse Events (PRO-CTCAE) were chosen based on the primary site of disease, 27 items for head and neck tumors, 21 items for thoracic tumors, and 24 items for pelvic tumors.
RESULTS
In total, 17 out of the 21 patients (81%) regularly submitted ePROMs and more than 2500 data points were acquired. An average of 5.2, 3.5, and 3.3 min was required to complete the head and neck, thorax, and pelvis questionnaires, respectively. ePROMS were able to detect the occurrence of both expected and unexpected side effects during the treatment. In addition, a gradual increase in the severity of side effects over the course the treatment and their remission afterward could be observed with ePROMs. In total, 9 out of the 17 patients (53%), mostly those with head and neck and thoracic cancers, reported PRO-CTCAE grade III or IV fatigue with severe impairments of activities of daily life.
CONCLUSIONS
This study shows the successful implementation of an ePROM system and a high patient acceptance. ePROMs have a great potential to improve patient care in radiotherapy by providing a comprehensive documentation of symptoms and side effects, especially of ones that are otherwise underreported.
Topics: Activities of Daily Living; Aged; Big Data; Electronic Data Processing; Fatigue; Feasibility Studies; Female; Head and Neck Neoplasms; Humans; Implementation Science; Male; Middle Aged; Neoplasm Staging; Patient Acceptance of Health Care; Patient Reported Outcome Measures; Pelvic Neoplasms; Quality of Life; Radiation Oncology; Radiotherapy; Surveys and Questionnaires; Telemedicine; Thoracic Neoplasms; Workflow
PubMed: 31342906
DOI: 10.2196/12345 -
International Journal of Radiation... Feb 2023To identify potential clinical prognostic factors associated with a higher risk of local recurrence in patients with localized pelvic Ewing sarcoma treated with...
Analysis of Local Control Outcomes and Clinical Prognostic Factors in Localized Pelvic Ewing Sarcoma Patients Treated With Radiation Therapy: A Report From the Children's Oncology Group.
PURPOSE
To identify potential clinical prognostic factors associated with a higher risk of local recurrence in patients with localized pelvic Ewing sarcoma treated with radiation therapy.
METHODS AND MATERIALS
Data for 101 patients treated with definitive radiation therapy (RT) or both surgery and radiation (S + RT) to primary pelvic tumors on INT-0091, INT-0154, and AEWS0031 were analyzed. Imaging data for patients who did not receive radiation were not available for central review; therefore, patients with surgery alone were not included. Cumulative incidence rates for local failure at 5 years from time of local control were calculated accounting for competing risks.
RESULTS
The most common pelvic subsite was sacrum (44.6%). RT was used in 68% of patients and S + RT in 32%. The local failure rate was 25.0% for RT and 6.3% for S + RT (P = .046). There was no statistically significant difference in local control modality by tumor characteristics. Tumors originating in the ischiopubic-acetabulum region were associated with the highest local failure incidence, 37.5% (P = .02, vs sacrum and iliac/buttock tumors), particularly those treated with RT (50.0%, P = .06). A higher incidence of local failure was seen with each additional 100 mL of tumor at diagnosis (P = .04). Multivariable analysis demonstrated RT alone (hazard ratio [HR], 5.1; P = .04), tumor subsite (particularly ischiopubic-acetabulum tumors; HR 4.6; P = .02), and increasing volume per 100 mL (HR, 1.2; P = .01) were associated with a higher incidence of local recurrence.
CONCLUSIONS
Combination surgery and RT is associated with improved local control in patients with pelvic Ewing sarcoma compared with definitive RT. Tumors involving the ischiopubic-acetabulum region and increasing tumor volume at diagnosis are associated with inferior local control. Tumor characteristics did not correlate with choice of local therapy modality suggesting an opportunity to develop best local therapy practices guidelines for future studies based on tumor features.
Topics: Humans; Child; Sarcoma, Ewing; Bone Neoplasms; Prognosis; Combined Modality Therapy; Sacrum; Pelvic Neoplasms; Retrospective Studies; Neoplasm Recurrence, Local
PubMed: 36302496
DOI: 10.1016/j.ijrobp.2022.07.1840 -
Scientific Reports Sep 2022Computed tomography (CT) has been widely used for the diagnosis of pelvic rhabdomyosarcoma (RMS) in children. However, it is difficult to differentiate pelvic RMS from...
Computed tomography (CT) has been widely used for the diagnosis of pelvic rhabdomyosarcoma (RMS) in children. However, it is difficult to differentiate pelvic RMS from other pelvic malignancies. This study aimed to analyze and select CT features by using least absolute shrinkage and selection operator (LASSO) logistic regression and established a Fisher discriminant analysis (FDA) model for the quantitative diagnosis of pediatric pelvic RMS. A total of 121 pediatric patients who were diagnosed with pelvic neoplasms were included in this study. The patients were assigned to an RMS group (n = 36) and a non-RMS group (n = 85) according to the pathological results. LASSO logistic regression was used to select characteristic features, and an FDA model was constructed for quantitative diagnosis. Leave-one-out cross-validation and receiver operating characteristic (ROC) curve analysis were used to evaluate the diagnostic ability of the FDA model. Six characteristic variables were selected by LASSO logistic regression, all of which were CT morphological features. Using these CT features, the following diagnostic models were established: (RMS group)[Formula: see text]; (Non-RMS group)[Formula: see text], where [Formula: see text], [Formula: see text], … and [Formula: see text] are lower than normal muscle density (1 = yes; 0 = no), multinodular fusion (1 = yes; 0 = no), enhancement at surrounding blood vessels (1 = yes; 0 = no), heterogeneous progressive centripetal enhancement (1 = yes; 0 = no), ring enhancement (1 = yes; 0 = no), and hemorrhage (1 = yes; 0 = no), respectively. The calculated area under the ROC curve (AUC) of the model was 0.992 (0.982-1.000), with a sensitivity of 94.4%, a specificity of 96.5%, and an accuracy of 95.9%. The calculated sensitivity, specificity and accuracy values were consistent with those from cross-validation. An FDA model based on the CT morphological features of pelvic RMS was established and could provide an easy and efficient method for the diagnosis and differential diagnosis of pelvic RMS in children.
Topics: Child; Humans; Logistic Models; Pelvic Neoplasms; ROC Curve; Rhabdomyosarcoma; Tomography, X-Ray Computed
PubMed: 36115914
DOI: 10.1038/s41598-022-20051-8 -
International Journal of Radiation... Apr 2019Radiation-induced gastrointestinal injury or radiation enteropathy is an imminent risk during radiation therapy of abdominal or pelvic tumors. Despite remarkable... (Review)
Review
Radiation-induced gastrointestinal injury or radiation enteropathy is an imminent risk during radiation therapy of abdominal or pelvic tumors. Despite remarkable technological advancements in image-guided radiation delivery techniques, the risk of intestinal injury after radiotherapy for abdominal or pelvic cancers has not been completely eliminated. The irradiated intestine undergoes varying degrees of adverse structural and functional changes, which can result in transient or long-term complications. The risk of development of enteropathy depends on dose, fractionation, and quality of radiation. Moreover, the patients' medical condition, age, inter-individual sensitivity to radiation and size of the treatment area are also risk factors of radiation enteropathy. Therefore, strategies are needed to prevent radiotherapy-induced undesirable alteration in the gastrointestinal tract. Many natural plant products, by virtue of their plethora of biological activities, alleviate the adverse effects of radiation-induced injury. The current review discusses potential roles and possible mechanisms of natural plant products in suppressing radiation enteropathy. Natural plant products have the potential to suppress intestinal radiation toxicity.
Topics: Abdominal Neoplasms; Ascorbic Acid; Biological Products; Curcumin; Garlic; Humans; Intestinal Diseases; Pelvic Neoplasms; Plant Extracts; Radiation Injuries; Vitamin E
PubMed: 30526224
DOI: 10.1080/09553002.2018.1552374 -
World Journal of Surgical Oncology Oct 2015Previous reports about laparoscopic total pelvic exenteration (LTPE) are still limited. In the present study, we described our single-center experience of the initial 11...
BACKGROUND
Previous reports about laparoscopic total pelvic exenteration (LTPE) are still limited. In the present study, we described our single-center experience of the initial 11 cases.
METHODS
Between April 2011 and September 2015, eight males and three females diagnosed as pelvic malignancies underwent LTPE by the same operation team. We retrospectively collected all cases' parameters about surgical technique. Thirty-seven patients who received open surgery were also retrospectively collected. A comparison between LTPE and open surgery was performed to evaluate the feasibility and safety of LTPE.
RESULTS
Eleven cases successfully underwent the LTPE without any intraoperative complication. No open conversion was required. Eight patients underwent Bricker's procedure. Three patients were performed with the cutaneous ureterostomy. Anus preservation operation was performed in three patients. Compared with open surgery, LTPE had longer mean operative time (565.2 vs 468.2 min, p = 0.004) but less mean blood loss (547.3 vs 1033.0 ml, p < 0.001) and shorter postoperative hospitalization time (15.3 vs 22.4 days, p = 0.004). One patient died of pulmonary embolism in the 7th month of follow-up time. One patient died of recurrence in the 12th month of follow-up time. Nine patients are still alive without recurrence and metastasis. The mean follow-up time was 11.1 months.
CONCLUSIONS
The technique of LTPE seems to be feasible and safe in the treatment of carefully selected patients of pelvic malignancies. LTPE can also decrease the blood loss, the recovery time, and the hospital stay. But the oncological safety and long-term outcome of LTPE still need to be explored.
Topics: Adult; Aged; Female; Follow-Up Studies; Humans; Laparoscopy; Length of Stay; Male; Middle Aged; Neoplasm Staging; Pelvic Exenteration; Pelvic Neoplasms; Postoperative Complications; Prognosis; Retrospective Studies; Robotics
PubMed: 26472147
DOI: 10.1186/s12957-015-0715-2 -
Taiwanese Journal of Obstetrics &... Jan 2022Only a few cases of early-stage ovarian granulosa cell tumor (GCT) with lung metastasis have been previously documented in the literature. We present a case of stage IA... (Review)
Review
OBJECTIVE
Only a few cases of early-stage ovarian granulosa cell tumor (GCT) with lung metastasis have been previously documented in the literature. We present a case of stage IA adult-type ovarian GCT with late pelvic recurrence and lung metastasis and review the relevant literature.
CASE REPORT
A 71-year-old female who underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy for an ovarian GCT in her early 50s presented with pelvic recurrence 16 years later. Lung metastasis was accidentally discovered during a preoperative computed tomography scan of the pelvic tumor. The patient received surgical resection and adjuvant chemotherapy. She remains alive and healthy without evidence of disease after 12 months of follow up.
CONCLUSION
This case illustrates the importance of long-term follow-up for early-stage GCTs. It also highlights the requirement of extended examination for possible extra-abdominal/pelvic tumors.
Topics: Aged; Female; Granulosa Cell Tumor; Humans; Lung Neoplasms; Neoplasm Metastasis; Neoplasm Recurrence, Local; Ovarian Neoplasms; Salpingo-oophorectomy
PubMed: 35181019
DOI: 10.1016/j.tjog.2021.10.002 -
International Journal of Molecular... May 2022Uterine leiomyosarcoma (uLMS) is a rare and aggressive cancer with few effective therapeutics. The Notch signaling pathway is evolutionarily conserved with oncogenic...
Uterine leiomyosarcoma (uLMS) is a rare and aggressive cancer with few effective therapeutics. The Notch signaling pathway is evolutionarily conserved with oncogenic properties, but it has not been well studied in uLMS. The purpose of our study was to determine expression of Notch family genes and proteins and to investigate the therapeutic effect of γ-secretase inhibitors (GSIs), indirect inhibitors of Notch signaling, in uLMS. We determined expression of Notch genes and proteins in benign uterine smooth muscle tissue, fibroids, and uLMS samples by immunostaining and in two uLMS cell lines, SK-UT-1B (uterine primary) and SK-LMS-1 (vulvar metastasis) by RT-PCR, Western blot and immunostaining. We exposed our cell lines to GSIs, DAPT and MK-0752, and measured expression of , a downstream effector of Notch. Notch proteins were differentially expressed in uLMS. Expression of NOTCH3 and NOTCH4 was higher in uLMS samples than in benign uterine smooth muscle and fibroids. Expression of NOTCH4 was higher in SK-LMS-1 compared to SK-UT-1B. Exposure of SK-UT-1B and SK-LMS-1 to DAPT and MK-0752 decreased expression of and decreased uLMS cell viability in a dose- and time-dependent manner that was unique to each GSI. Our findings suggest that GSIs are potential therapeutics for uLMS, albeit with limited efficacy.
Topics: Female; Gamma Secretase Inhibitors and Modulators; Humans; Leiomyoma; Leiomyosarcoma; Pelvic Neoplasms; Platelet Aggregation Inhibitors; Receptors, Notch; Signal Transduction; Uterine Neoplasms
PubMed: 35682660
DOI: 10.3390/ijms23115980 -
Internal Medicine (Tokyo, Japan) Mar 2023
Topics: Humans; Pelvic Neoplasms; Rectal Neoplasms; Lymph Nodes; Kidney Neoplasms; Pelvis
PubMed: 35945014
DOI: 10.2169/internalmedicine.0258-22 -
Tumori Nov 2017Paraganglioma, a kind of pheochromocytoma originating from embryonic neural crest, is a rare neuroendocrine neoplasm commonly located at extra-adrenal areas such as... (Review)
Review
PURPOSE
Paraganglioma, a kind of pheochromocytoma originating from embryonic neural crest, is a rare neuroendocrine neoplasm commonly located at extra-adrenal areas such as head, neck, and abdomen. There are a few reports on renal paragangliomas; fewer than 5 reported cases are renal pelvic paragangliomas, including our case.
METHODS
Our patient, who had not experienced headache, hypertension, or palpitation, was founded to have a fixed mass in the left renal pelvis incidentally. Ultrasonography and computed tomography (CT) demonstrated a heterogeneous mass before surgery, and histopathologic test subsequently revealed that the infrequent mass was paraganglioma.
RESULTS
During 6 months follow-up, ultrasonography and CT examinations showed no signs of recurrence or metastasis.
CONCLUSIONS
The present case report confirms surgery as the standard to treat patients with paraganglioma. We present this case to serve as a reminder of suspected paraganglioma when it has occurred in renal pelvis.
Topics: Adult; Humans; Kidney Neoplasms; Kidney Pelvis; Male; Paraganglioma; Pelvic Neoplasms; Prognosis; Young Adult
PubMed: 28799639
DOI: 10.5301/tj.5000677