-
The Journal of Obstetrics and... Nov 2020This review aims to analyze the pathological aspects, diagnosis and treatment of rare mesenchymal uterine tumors. (Review)
Review
OBJECTIVE
This review aims to analyze the pathological aspects, diagnosis and treatment of rare mesenchymal uterine tumors.
METHODS
On August 2019, a systematic review of the literature was done on Pubmed, MEDLINE, Scopus, and Google Scholar search engines. The systematic review was carried out in agreement with the Preferred Reporting Items for Systematic Reviews and Meta-Analyzes statement (PRISMA). The following words and key phrases have been searched: "endometrial stromal sarcoma", "low-grade endometrial stromal sarcoma", "high-grade endometrial stromal sarcoma", "uterine sarcoma", "mesenchymal uterine tumors" and "uterine stromal sarcoma". Across these platforms and research studies, five main aspects were analyzed: the biological characteristics of the neoplasms, the number of cases, the different therapeutic approaches used, the follow-up and the oncological outcomes.
RESULTS
Of the 94 studies initially identified, 55 were chosen selecting articles focusing on endometrial stromal sarcoma. Of these fifty-five studies, 46 were retrospective in design, 7 were reviews and 2 randomized phases III trials.
CONCLUSION
Endometrial stromal sarcomas are rare mesenchymal uterine neoplasms and surgery represents the standard treatment. For uterus-limited disease, the remove en bloc with an intact resection of the tumor (without the use of morcellation) is strongly recommended. For advanced-stage disease, the standard surgical treatment is adequate cytoreduction with metastatectomy. Pelvic and para-aortic lymphadenectomy is not recommended in patients with Low-grade Endometrial Stromal Sarcoma (ESS), while is not clear whether cytoreduction of advanced tumors improves patient survival in High-grade ESS. Administration of adjuvant radiotherapy or chemotherapy is not routinely used and its role is still debated.
Topics: Endometrial Neoplasms; Female; Humans; Retrospective Studies; Sarcoma, Endometrial Stromal; Uterine Neoplasms
PubMed: 32830415
DOI: 10.1111/jog.14436 -
Gynecologic Oncology Apr 2014The aim of this systematic review is to determine the incidence of lymph-node metastasis in clinical stage I and II sex cord stromal tumours and germ cell tumours of the... (Review)
Review
OBJECTIVES
The aim of this systematic review is to determine the incidence of lymph-node metastasis in clinical stage I and II sex cord stromal tumours and germ cell tumours of the ovary.
METHODS
Relevant articles were identified from MEDLINE and EMBASE and supplemented with citations from the reference lists of the primary studies. Eligibility was determined by two authors. Included studies were prospective or retrospective cohort and cross-sectional studies analysing at least ten patients with clinical early-stage non-epithelial ovarian cancer who underwent lymphadenectomy or lymph-node sampling as part of a staging laparotomy.
RESULTS
For sex cord stromal tumours, five articles including 578 patients were analysed and lymph-node metastasis was not detected in the 86 patients who underwent lymph-node removal. The median number of removed lymph nodes was 13 (range 9-29). For malignant germ cell tumours, three articles were eligible including 2436 patients of whom 946 patients underwent lymph-node resection. The mean number of removed nodes was 10 (range 2-14) with a mean incidence of lymph-node metastasis of 10.9% (range 10.5-11.8%).
CONCLUSIONS
The incidence of lymph-node metastasis in patients with clinical stage I and II sex cord stromal tumours is low, whereas the incidence in patients with clinical stage I-II germ cell tumours is considerable, although limited data are available.
Topics: Female; Humans; Lymph Nodes; Lymphatic Metastasis; Neoplasm Staging; Neoplasms, Germ Cell and Embryonal; Ovarian Neoplasms; Sex Cord-Gonadal Stromal Tumors
PubMed: 24440833
DOI: 10.1016/j.ygyno.2014.01.011 -
Urology Sep 2022To perform a systematic review of mixed epithelial stromal tumor of the seminal vesicle (SV) to characterize the diagnosis and treatment of this rare condition. (Review)
Review
OBJECTIVE
To perform a systematic review of mixed epithelial stromal tumor of the seminal vesicle (SV) to characterize the diagnosis and treatment of this rare condition.
METHODS
"Seminal vesicle mixed epithelial stromal tumor" OR "seminal vesicle cystadenoma" were searched on PubMed/MEDLINE for relevant articles through 6 September 2021. Articles were eligible if they were in English, accessible via our university library services, and if the abstract was concordant with the content of the publication. Reference lists of included articles were reviewed to identify additional relevant articles.
RESULTS
In total, 66 articles were identified, of which 34 (N = 36 patients) were included. The most common presenting symptoms were lower urinary tract symptoms (33%, 12/36), dysuria (22%, 8/36), lower abdominal pain (17%, 6/36), and hematuria (17%, 6/36). However, there were eight cases (23%, 8/36) of asymptomatic incidental SV tumors. A biopsy was performed in 47% of cases (17/36), of which 53% (9/17) showed benign findings, 29% (5/17) were inconclusive, and 18% (3/17) SV cystadenoma. Surgical resection was performed using open (57%, 20/35), laparoscopic (26%, 9/35), or robotic (17%, 6/35) techniques. The majority (94%, 34/36) of the SV tumors were low-grade. Long-term follow-up was reported for 15 patients in which two patients (13%, 2/15) had tumor recurrence.
CONCLUSION
High rate of inconclusive biopsy of SV tumors suggests that routine biopsy is of questionable utility. Surgical excision frequently relieves symptoms and confirms accurate pathologic diagnosis. After tumor removal, patients should be surveilled with cross-sectional imaging of the pelvis given the possibility of tumor recurrence.
Topics: Cystadenoma; Genital Neoplasms, Male; Humans; Male; Neoplasm Recurrence, Local; Pelvis; Seminal Vesicles
PubMed: 35231450
DOI: 10.1016/j.urology.2022.02.012 -
Annals of Surgical Oncology Oct 2013This study is a systematic review and meta-analysis that compares the short- and long-term outcomes of laparoscopic gastric resection (LR) versus open gastric resection... (Review)
Review
BACKGROUND
This study is a systematic review and meta-analysis that compares the short- and long-term outcomes of laparoscopic gastric resection (LR) versus open gastric resection (OR) for gastric gastrointestinal stromal tumors (GISTs).
METHODS
Comparative studies reporting the outcomes of LR and OR for GIST were reviewed.
RESULTS
A total of 11 nonrandomized studies reviewed 765 patients: 381 LR and 384 OR. A higher proportion of high-risk tumors and gastrectomies were in the OR compared with LR (odds ratio, 3.348; 95 % CI, 1.248-8.983; p = .016) and (odds ratio, .169; 95 % CI, .090-.315; p < .001), respectively. Intraoperative blood loss was significantly lower in the LR group [weighted mean difference (WMD), -86.508 ml; 95 % CI, -141.184 to -31.831 ml; p < .002]. The LR group was associated with a significantly lower risk of minor complications (odds ratio, .517; 95 % CI, .277-.965; p = .038), a decreased postoperative hospital stay (WMD, -3.421 days; 95 % CI, -4.737 to -2.104 days; p < .001), a shorter time to first flatus (WMD, -1.395 days; 95 % CI, -1.655 to -1.135 days; p < .001), and shorter time for resumption of oral intake (WMD, -1.887 days; 95 % CI, -2.785 to -.989 days; p < .001). There was no statistically significant difference between the two groups with regard to operation time (WMD, 5.731 min; 95 % CI, -15.354-26.815 min; p = .594), rate of major complications (odds ratio, .631; 95 % CI, .202-1.969; p = .428), margin positivity (odds ratio, .501; 95 % CI, .157-1.603; p = .244), local recurrence rate (odds ratio, .629; 95 % CI, .208-1.903; p = .412), recurrence-free survival (RFS) (odds ratio, 1.28; 95 % CI, .705-2.325; p = .417), and overall survival (OS) (odds ratio, 1.879; 95 % CI, .591-5.979; p = .285).
CONCLUSIONS
LR results in superior short-term postoperative outcomes without compromising oncological safety and long-term oncological outcomes compared with OR.
Topics: Gastrectomy; Gastrointestinal Stromal Tumors; Humans; Laparoscopy; Meta-Analysis as Topic; Postoperative Complications; Prognosis; Stomach Neoplasms
PubMed: 23793362
DOI: 10.1245/s10434-013-3051-1 -
Tumori 2010In patients with localized gastrointestinal stromal tumors, surgery remains the elective treatment. Nowadays, imatinib therapy has been standardized in advanced... (Meta-Analysis)
Meta-Analysis Review
AIMS AND BACKGROUND
In patients with localized gastrointestinal stromal tumors, surgery remains the elective treatment. Nowadays, imatinib therapy has been standardized in advanced gastrointestinal stromal tumors, showing continuous improvements in progression-free and overall survival. A combination of imatinib therapy and surgery may also be effective in a subset of patients with metastatic or unresectable gastrointestinal stromal tumors. In this review, the authors analyzed the role of imatinib mesylate associated to surgery in unresectable and/or metastatic gastrointestinal stromal tumors.
METHODS AND STUDY DESIGN
We searched for all published and unpublished randomized controlled clinical trials and controlled clinical trials. We conducted the review according to the recommendations of The Cochrane Collaboration. We used Review Manager 5 software for the statistical analysis.
RESULTS
There are currently no randomized controlled clinical trials or controlled clinical trials on this issue. We performed a subgroup analysis in the patients preoperatively treated with imatinib mesylate. This subgroup revealed a minor incidence of recurrent or metastatic gastrointestinal stromal tumors and a greater incidence of locally unresectable gastrointestinal stromal tumors in the responsive disease group (P = 0.001). In this patient group, more complete resections were observed (P = 0.00001). Furthermore, in the same patient group we observed a more significant 12 and 24-month disease-free survival after imatinib treatment and complete resection (respectively P= 0.06 and P= 0.003) and also a better 24-month overall survival (P = 0.004).
CONCLUSIONS
There is actually only one ongoing European randomized study evaluating surgery of residual disease in patients with metastatic gastrointestinal stromal tumors responding to imatinib mesylate. Imatinib mesylate represents the standard treatment as preoperative supplement for locally unresectable and/or metastatic gastrointestinal stromal tumors, and a trial to compare the approach versus surgery alone is not necessary. For patients responding to imatinib or patients with prolonged stable disease, resection of residual disease should be considered. A phase III randomized study evaluating surgery of residual disease in patients with metastatic gastrointestinal stromal tumor responding to imatinib mesylate, EORTC 62063, has been opened. Moreover, surgery should be considered for patients at higher risk of complications during pharmacological debulking. In advanced gastrointestinal stromal tumors, the advantages of the integrated treatment are significant in the complete or partial response disease group in terms of more complete resections and better disease-free and overall survival.
Topics: Antineoplastic Agents; Benzamides; Chemotherapy, Adjuvant; Controlled Clinical Trials as Topic; Gastrointestinal Stromal Tumors; Humans; Imatinib Mesylate; Piperazines; Protein Kinase Inhibitors; Pyrimidines; Randomized Controlled Trials as Topic; Treatment Outcome
PubMed: 20845798
DOI: 10.1177/030089161009600303 -
Health Technology Assessment... Jun 2011Imatinib dose escalation is advocated for gastrointestinal stromal tumour (GIST) treatment, but its effectiveness compared with sunitinib and best supportive care (BSC)... (Review)
Review
Clinical effectiveness and cost-effectiveness of imatinib dose escalation for the treatment of unresectable and/or metastatic gastrointestinal stromal tumours that have progressed on treatment at a dose of 400 mg/day: a systematic review and economic evaluation.
BACKGROUND
Imatinib dose escalation is advocated for gastrointestinal stromal tumour (GIST) treatment, but its effectiveness compared with sunitinib and best supportive care (BSC) after failure at the 400 mg/day dose is unknown.
OBJECTIVES
To assess the effectiveness and cost-effectiveness of imatinib at escalated doses of 600 or 800 mg/day for patients with unresectable and/or metastatic GISTs whose disease had progressed on 400 mg/day.
DATA SOURCES
Electronic databases, including MEDLlNE, MEDLINE In-Process, EMBASE, BIOSIS, Science Citation Index, Health Management Information Consortium and the Cochrane Controlled Trials Register, were searched until September 2009.
REVIEW METHODS
A systematic review of the literature was carried out according to standard methods. An economic model was constructed to assess the cost-effectiveness of seven alternative pathways for treating patients with unresectable and/or metastatic GISTs.
RESULTS
Five primary studies involving 669 people were included for clinical effectiveness; four reported imatinib and one reported sunitinib. The data were essentially observational as none of the studies was designed to specifically assess treatment of patients whose disease had progressed on 400 mg/day imatinib. For 600 mg/day imatinib, between 26% and 42% of patients showed either a partial response (PR) or stable disease (SD). Median time to progression was 1.7 months (range 0.7-24.9 months). For 800 mg/day imatinib, between 29% and 33% of patients showed either a PR or SD. Median overall survival (OS) was 19 months [95% confidence interval (CI) 13 to 23 months]. Progression-free survival ranged from 81 days to 5 months (95% CI 2 to 10 months). Median duration of response was 153 days (range 37-574 days). Treatment progression led to 88% discontinuations but between 16% and 31% of patients required a dose reduction, and 23% required a dose delay. There was a statistically significant increase in the severity of fatigue (p < 0.001) and anaemia (p = 0.015) following dose escalation. For sunitinib, median OS was 90 weeks (95% CI 73 to 106 weeks). For the cost-effectiveness review, only one full-text study and one abstract were identified, comparing imatinib at an escalated dose, sunitinib and BSC, although neither was based on a UK context. The definition of BSC was not consistent across the studies, and the pattern of resources (including drugs for treatment) and measures of effectiveness also varied. Within the model, BSC (assumed to include continuing medication to prevent tumour flare) was the least costly and least effective. It would be the care pathway most likely to be cost-effective when the cost per quality-adjusted life-year threshold was < £25,000. Imatinib at 600 mg/day was most likely to be cost-effective at a threshold between £25,000 and £45,000. Imatinib at 600 mg/day followed by further escalation followed by sunitinib was most likely to be cost-effective at a threshold > £45,000.
LIMITATIONS
The evidence base was sparse, data were non-randomised and potentially biased. The economic model results are surrounded by a considerable degree of uncertainty and open to biases of unknown magnitude and direction.
CONCLUSIONS
Around one-third of patients with unresectable and/or metastatic GIST, who fail on 400 mg/day of imatinib, may show response or SD with escalated doses. Between a threshold of £25,000 and £45,000, provision of an escalated dose of imatinib would be most likely to be cost-effective. However, these results should be interpreted with caution owing to the limited evidence available on outcomes following imatinib dose escalation or sunitinib for this group of patients.
FUNDING
The National Institute for Health Research Health Technology Assessment programme.
Topics: Antineoplastic Agents; Benzamides; Confidence Intervals; Cost-Benefit Analysis; Disease Progression; Gastrointestinal Stromal Tumors; Humans; Imatinib Mesylate; Incidence; Models, Economic; Piperazines; Pyrimidines; Quality-Adjusted Life Years; Survival Analysis; Time Factors; Treatment Outcome; Uncertainty; United States
PubMed: 21689502
DOI: 10.3310/hta15250 -
Journal of Pediatric Surgery Sep 2018Gastrointestinal stromal tumors (GIST) are extremely rare in children. Imaging plays a key role in staging and monitoring therapy (surgical and with tyrosine kinase... (Review)
Review
BACKGROUND
Gastrointestinal stromal tumors (GIST) are extremely rare in children. Imaging plays a key role in staging and monitoring therapy (surgical and with tyrosine kinase inhibitors). The vast majority of articles addressing imaging of GIST base on adults and are based on CT. The subtype "pediatric GIST" - if at all - is only mentioned in a dependent clause. Although the imaging features in children and adults are similar, histology, clinical course and thus imaging approach are different.
METHODS
A PubMed search using the search terms "Gastrointestinal stromal tumor, GIST, WT GIST, children, pediatric, carney's triad, imaging, staging, follow-up, MRI, CEUS, ultrasonography, Positron emission tomography" was conducted. Studies that reported on laparoscopy, endoscopy and surgical techniques only were excluded.
RESULTS
Based on our selective literature review, we present alternative radiological imaging strategies using MRI, contrast enhanced ultrasound (CEUS) and PET-CT to stage and follow-up pediatric GIST patients. As pediatric GIST often is a chronic disease, minimizing exposure to ionizing radiation is mandatory.
CONCLUSION
MRI, contrast enhanced ultrasound and PET-CT instead of CT are the imaging modalities to evaluate pediatric GIST.
TYPE OF STUDY
Systematic review LEVEL OF EVIDENCE: III.
Topics: Child; Chondroma; Contrast Media; Female; Gastrointestinal Stromal Tumors; Humans; Leiomyosarcoma; Lung Neoplasms; Magnetic Resonance Imaging; Male; Multimodal Imaging; Paraganglioma, Extra-Adrenal; Positron Emission Tomography Computed Tomography; Stomach Neoplasms; Tomography, X-Ray Computed
PubMed: 29685489
DOI: 10.1016/j.jpedsurg.2018.03.022 -
Scientific Reports Sep 2015Many types of KIT mutations have been observed in gastrointestinal stromal tumors (GISTs), but their prognostic and predictive significance are still unclear. A... (Meta-Analysis)
Meta-Analysis Review
Many types of KIT mutations have been observed in gastrointestinal stromal tumors (GISTs), but their prognostic and predictive significance are still unclear. A meta-analysis and literature review were conducted to estimate the contribution of KIT mutations in prognostic parameters and clinic-pathological significance of GISTs. A total of 18 relevant articles from PubMed, EMBASE and Web of Science databases were included in this study. The frequency of KIT mutation was significantly increased in the GIST patients with higher mitosis (≥5/50 high-power fields (HPFs) and larger size (≥5 cm) of tumors than in those with lower MI (≤5/50HPFs) and smaller size (≤5 cm) of GISTs respectively. The rate of KIT mutation was not significantly changed between GISTs in stomachs and in small intestines. KIT mutational status has prognostic significance for patients' outcome. GIST patients with KIT exon 9 mutations have higher risk of progression than those with exon 11 mutations. 5 year relapse-free survival (RFS) rate was significantly higher in patients with KIT exon 11 deletion than in those with other type of KIT exon 11 mutations. The deletion involving KIT exon 11, particularly codons 557-558, is a valuable predictor of prognosis for patients with GISTs.
Topics: Exons; Gastrointestinal Stromal Tumors; Genetic Association Studies; Genetic Predisposition to Disease; Humans; Mutation; Odds Ratio; Prognosis; Proto-Oncogene Proteins c-kit; Receptor, Platelet-Derived Growth Factor alpha; Risk
PubMed: 26349547
DOI: 10.1038/srep13718 -
BMC Cancer Nov 2019Intracranial metastasis of Gastrointestinal Stromal Tumors (GISTs) is rare but presents unique treatment challenges. We present a case of intracranial metastasis of GIST...
BACKGROUND
Intracranial metastasis of Gastrointestinal Stromal Tumors (GISTs) is rare but presents unique treatment challenges. We present a case of intracranial metastasis of GIST with a systematic review of the literature. A literature search using key terms "'gastrointestinal stromal tumor' AND brain AND metastasis"" was conducted through May 2019 via Embase and Pubmed according to PRISMA guidelines. Only cases describing intradural metastases rather than calvarial or intraorbital metastases were included.
CASE PRESENTATION
A 57-year-old woman with history of GIST metastatic to the liver presented with a six-week history of left facial weakness, left hearing loss, and left facial numbness, and a one-week history of headaches, gait disturbance, and dizziness. MRI revealed a contrast-enhancing dural-based left middle cranial fossa mass measuring 2.9 cm × 3.1 cm × 3.4 cm with extension into the internal auditory canal and cerebral edema. A left temporal craniotomy was performed to excise the lesion, and the patient was discharged to a rehabilitation facility at her preoperative baseline. Intraoperative pathology revealed a spindle cell neoplasm, postoperative MRI demonstrated gross total resection of the lesion, and microscopic analysis demonstrated sheets of spindled tumor cells with short ovoid, irregular, hyperchromatic nuclei and scattered large atypical nuclei without extensive necrosis. Immunohistochemical staining was positive for KIT proto-oncogene (CD117, c-KIT), and the patient was put on imatinib (400 mg/day).
CONCLUSIONS
Of the 18 cases analyzed and our present case, metastasis typically involved the cerebrum with only one in infratentorial elements. The tumors in seven of the cases involved the dura, and one case metastasized to the pituitary. Eight patients died following treatment. Surgery remains the mainstay of intracranial metastatic GIST, however there are many reports of good responses to radiation or chemotherapy alone. More investigation is required to determine the best treatment course for these patients.
Topics: Brain Neoplasms; Female; Gastrointestinal Neoplasms; Gastrointestinal Stromal Tumors; Humans; Middle Aged; Prognosis; Proto-Oncogene Mas
PubMed: 31730471
DOI: 10.1186/s12885-019-6316-7 -
Scientific Reports Feb 2016The impact and management of microscopically positive margins in gastrointestinal stromal tumors (GISTs) remain unclear. The aim of this study is to estimate the... (Meta-Analysis)
Meta-Analysis Review
The impact and management of microscopically positive margins in gastrointestinal stromal tumors (GISTs) remain unclear. The aim of this study is to estimate the prognostic value of surgical margins for disease-free survival (DFS) and overall survival (OS) in patients with primary GISTs. Twelve studies with 1985 GIST patients were included. The overall recurrence rate in R1 resection and R0 resection group was 0.364 (95% CI 0.299-0.429) and 0.296 (95% CI 0.161-0.430), respectively. Meta-analysis confirmed that a microscopically positive margin could significantly impact the disease-free survival (HR 1.596, 95% CI 1.128-2.258; I(2) = 37.5%, P value = 0.091), but had no influence on overall survival (HR 1.430, 95% CI 0.608-3.363; I(2) = 60.8%, P value = 0.013). Importantly, subgroup analysis revealed that adjuvant imatinib treatment could attenuate the risk of recurrence for primary GIST patients who received R1 resection. (HR 1.308, 95% CI 0.583-2.935; I(2) = 53.2%, P value = 0.074). The level of evidence achieved in this study was "moderate" for DFS and "low" for OS. In conclusion, this study revealed that a microscopically positive margin is an unfavorable prognostic factor for GIST patients with R1 resection, and adjuvant imatinib treatment is proved to be effective.
Topics: Disease-Free Survival; Gastrointestinal Stromal Tumors; Humans; Margins of Excision; Neoplasm Recurrence, Local; Odds Ratio; Prognosis; Proportional Hazards Models; Publication Bias
PubMed: 26891953
DOI: 10.1038/srep21541