Malignant Melanoma of the Genitourinary System

2021 ◽  
Vol 156 (Supplement_1) ◽  
pp. S73-S73
Author(s):  
A Khayyat ◽  
A Amin

Abstract Introduction/Objective Malignant melanoma (MM) of mucosal membranes (excluding anus and head and neck) is a rare but aggressive disease with poor outcome. There is little information available on the development mechanism, risk factors, and management of this tumor, mainly due to the low number of cases. Methods/Case Report We performed a comprehensive literature review on mucosal MM (between 1970-2020). We identified 52 papers reporting 242 patients with primary genitourinary melanomas (limited to urothelium, vagina and cervix). A comprehensive review of the demographics, stage, treatment and survival was performed. Results (if a Case Study enter NA) The demographics are provided in Table 1. In 204 cases, lesions were located in the urothelium (urethra: 202, bladder: 2) followed by 34 in the vagina and 4 in uterine cervix. None of the cases had any other known primary source of MM. Molecular studies in a subset of cases revealed alterations in c-KIT, NRAS, BRAF (non-V600E and V600E), TP53 and NF1 (Table 2). Tumor stage was provided in a few (n=10) cases (stage 1=1, stage 2=1, stage 3=3 & stage 4=5). 3 patients had distant metastasis (brain, retroperitoneal and inguinal lymph nodes). Treatment data was available in 178 cases. 177 patients were managed by surgery. In addition to surgery, 10 received chemotherapy, 7 received immunotherapy and chemotherapy, 2 received chemoradiation, 1 received immunotherapy and 1 received radiotherapy. In 43 patients, the treatment regimen was not provided. The outcome was available in 178 cases (range of follow up between 3-97 months), showing 15 deaths of disease. Median survival based on available information in 28 patients was 82% (5-year survival). Table 1 legend: Demographics of genitourinary malignant melanoma cases. Table 2 legend: Frequency of molecular alterations in genitourinary malignant melanoma. Conclusion Similarities in genetic signatures of mucosal and skin MM suggest similar mechanism of development; however, unlike skin melanoma, there is less BRAF mutation and more of PI3K/AKT/mTOR pathway alterations in mucosal MM. Prolonged administration of chemotherapy (i.e., methotrexate) and immune-modulating agents (i.e., natalizumab) may be a risk factor. In light of absence of a definite adjuvant therapy, tumor stage at the time of diagnosis and proper surgical removal have central role in the prognosis and survival of patients.

2021 ◽  
Vol 7 (2) ◽  
pp. 366-371
Author(s):  
Sunil Ganekal ◽  
Varun Ganekal

To compare the visual acuity outcomes after surgical treatment of epiretinal membranes (ERM) in cases with and without ectopic inner foveal layers (EIFL). : A total of 100 eyes of 98 patients with idiopathic ERM were retrospectively staged according to the newer OCT classification and divided into two groups on the basis of presence or absence of EIFL. The two groups were evaluated for various parameters including visual acuity, thickness of the EIFL, re-appearance of the foveal contour, outer retinal changes and complications before and after ERM peeling. Correlation analysis was done using chi square test and p-values were obtained for corresponding variables.: Of 100 eyes with ERM, 20% had stage 1, 29% had stage 2, 42% had stage3 & 9% had stage 4 disease. VA was worse in eyes with EIFL as compared those without it. Correlation of VA with EIFL thickness however was not significant. Surgical removal of ERM didn’t alter EIFL frequency though the thickness decreased. New EIFL appeared in 4% eyes and 35% demonstrated thickened fovea after surgery. Anatomical reappearance of foveal contour was poorer in eyes with EIFL. Outer retinal changes were more common in non EIFL eyes. Lamellar holes and nontractional cysts were more common in EIFL eyes.: EIFL is a significant prognostic tool to predict the visual outcome of surgery in cases of Epiretinal membrane. EIFL eyes did not regain foveal contour, had less outer retinal damage but were associated with poorer visual outcomes.


Author(s):  
Max P. Brinkmann ◽  
Stephan Michels ◽  
Carolin Brinkmann ◽  
Felix Rommel ◽  
Mahdy Ranjbar ◽  
...  

Abstract Background Clinical studies have shown that epiretinal membranes (ERM) as well as abnormalities of the central foveal bouquet (CB) can be classified in different stages according to their morphological appearance. Furthermore, visual acuity correlates with the different stages of these features. The present study evaluated how these findings change after the surgical removal of the ERM and their impact on functional outcomes. Methods In this retrospective study eyes with ERM were evaluated by SD-OCT scans before and after pars plana vitrectomy (PPV) with macular ERM and internal limiting membrane (ILM) peeling. CB abnormalities were classified according to their morphological appearance from stage 0 (no abnormalities) to stage 3 (acquired vitelliform lesion). ERMs were classified ranging from stage 0 (absence of ERM) to stage 4 (ERM with significant anatomic disruption of macula). Changes in morphology were correlated with visual acuity before and after surgery. Results 151 eyes were included into the study. Before surgery 27.2% (n = 41) of eyes showed CB abnormalities with stage 1 being the most common (11.9%, n = 18). Before surgery ERM was seen in all patients. The most common form was stage 1 (28.5%, n = 43), followed by stage 3 (27.8%, n = 42) and 2 (25.2%, n = 38). Only 18.5% (n = 28) presented with stage 4 ERM. The mean BCVA was 0.42 (logMAR) before and increased to 0.19 (logMAR) 8 weeks after vitrectomy (95% CI 0.20–0.28; p < 0.001). Patients who suffered from CB abnormalities had less increase in BCVA than patients who had no evidence of CB (0.28 vs. 0.14 logMAR; p < 0.001). Of all the patients with CB abnormalities at baseline, 68% had lower CB grading after the surgery (n = 28; 95% CI; p < 0.001). All patients showed an improvement of their ERM grading, with 98.7% reaching stage 0 (n = 151 vs. n = 149; 95% CI; p < 0.001). Conclusions The study indicates that the presence of CB abnormalities correlates with worse visual function. They are furthermore associated with worse visual outcomes after PPV with ERM and ILM peeling. These findings are valuable for deciding on PPV in patients with ERM.


2021 ◽  
Vol 9 (7_suppl3) ◽  
pp. 2325967121S0014
Author(s):  
Marshall Spiegel ◽  
Jon Minor ◽  
Nancy Phu ◽  
Jared Kliner ◽  
Prem Kumar Thirunagari ◽  
...  

Background: There are multiple subtypes identified in patients with Persistent Post Concussion symptoms (PPCS) in young athletes including mood subtype that is traditionally believed to be in higher incidence with PPCS. Although there may be an association of poor mood symptoms with worse PPCS outcomes, empirical analysis of patients’ mood symptoms and the duration of PPCS is scant. By better understanding the incidence of mood symptoms in pediatric PPCS, we may uncover other primary causes of ongoing brain dysfunction. Purpose: To explore the relationship between mood-symptom severity and the duration of PPCS. Methods: A retrospective cohort study of pediatric PPCS patients (age < 18 years) treated at private concussion clinic in Tucson, AZ between 7/2018 to 7/2019. Based on written questionnaires completed by patients at office visits, patients were assigned a mood and anxiety score between 0 and 3, with 0 being the least severe and 1 being the most severe. Patients were also classified into stages 1 through 4 based on PPCS duration (see table). [Table: see text] Results: Data from 59 pediatric patients (ages ranged from 7 to 17 with a mean age of 13.9, 33:26 female to male) were included. ANOVA yielded p-value of 0.94. Average (mean) mood and anxiety scores for each PPCS substage were as follows: 0.9 for stage 1 (n=23); 0.8 for stage 2 (n=18); 0.8 for stage 3 (n=13); 0.6 for stage 4 (n=5). Conclusion: Our review data showed no significance between PPCS stages in regard to average self-reported mood symptoms. Contrary to traditional thinking this preliminary data in pediatric athletes illustrates that mood severity does not increase with increasing PPCS stage. This may suggest that other domains such as migraine or vestibulocular dysfunction may be the primary source of ongoing symptoms.


Author(s):  
L. Vacca-Galloway ◽  
Y.Q. Zhang ◽  
P. Bose ◽  
S.H. Zhang

The Wobbler mouse (wr) has been studied as a model for inherited human motoneuron diseases (MNDs). Using behavioral tests for forelimb power, walking, climbing, and the “clasp-like reflex” response, the progress of the MND can be categorized into early (Stage 1, age 21 days) and late (Stage 4, age 3 months) stages. Age-and sex-matched normal phenotype littermates (NFR/wr) were used as controls (Stage 0), as well as mice from two related wild-type mouse strains: NFR/N and a C57BI/6N. Using behavioral tests, we also detected pre-symptomatic Wobblers at postnatal ages 7 and 14 days. The mice were anesthetized and perfusion-fixed for immunocytochemical (ICC) of CGRP and ChAT in the spinal cord (C3 to C5).Using computerized morphomety (Vidas, Zeiss), the numbers of IR-CGRP labelled motoneurons were significantly lower in 14 day old Wobbler specimens compared with the controls (Fig. 1). The same trend was observed at 21 days (Stage 1) and 3 months (Stage 4). The IR-CGRP-containing motoneurons in the Wobbler specimens declined progressively with age.


2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
A.L Van Wijngaarden ◽  
Y.L Hiemstra ◽  
P Van Der Bijl ◽  
V Delgado ◽  
N Ajmone Marsan ◽  
...  

Abstract Background The indication for surgery in patients with severe primary mitral regurgitation (MR) is currently based on the presence of symptoms, left ventricular (LV) dilatation and dysfunction, atrial fibrillation and pulmonary hypertension. The aim of this study was to evaluate the prognostic impact of a new staging classification based on cardiac damage including the known risk factors but also including global longitudinal strain (GLS), severe left atrial (LA) dilatation and right ventricular (RV) dysfunction. Methods In total 614 patients who underwent surgery for severe primary MR with available baseline transthoracic echocardiograms were included. Patients were classified according to the extent of cardiac damage (Figure): Stage 0-no cardiac damage, Stage 1-LV damage, Stage 2-LA damage, Stage 3-pulmonary vasculature or tricuspid valve damage and Stage 4-RV damage. Patients were followed for all-cause mortality. Results Based on the proposed classification, 172 (28%) patients were classified as Stage 0, 102 (17%) as Stage 1, 134 (21%) as Stage 2, 135 (22%) as Stage 3 and 71 (11%) as Stage 4. The more advanced the stage, the older the patients were with worse kidney function, more symptoms and higher EuroScore. Kaplan-Meier curve analysis revealed that patients with more advanced stages of cardiac damage had a significantly worse survival (log-rank chi-square 35.2; p&lt;0.001) (Figure). On multivariable analysis, age, male, chronic obstructive pulmonary disease, kidney function, and stage of cardiac damage were independently associated with all-cause mortality. For each stage increase, a 22% higher risk for all-cause mortality was observed (95% CI: 1.064–1.395; p=0.004). Conclusion In patients with severe primary MR, a novel staging classification based on the extent of cardiac damage, may help refining risk stratification, particularly including also GLS, LA dilatation and RV dysfunction in the assessment. Funding Acknowledgement Type of funding source: None


Author(s):  
Ryan Austin Fisher ◽  
Nancy L. Summitt ◽  
Ellen B. Koziel

The purpose of this study was to describe the voice change and voice part assignment of male middle school choir members. Volunteers ( N = 92) were recruited from three public middle school choral programs (Grades 6-8). Participants were audio-recorded performing simple vocal tasks in order to assess vocal range and asked to share the music they were currently singing in class. Results revealed 23.91% of participants’ voices could be categorized as unchanged, 14.13% as Stage 1, 3.26% as Stage 2, 10.87% as Stage 3, 26.09% as Stage 4, and 21.74% as Stage 5. The majority of sixth-grade participants were classified as unchanged or in Stage 1 of the voice change and the majority of eighth-grade participants were classified in Stages 4 to 5 of the voice change. Of the participants labeled “tenors” in their choir, over 60% were classified as either unchanged voices or in Stage 1 of the voice change.


2021 ◽  
Vol 29 ◽  
pp. 297-309
Author(s):  
Xiaohui Chen ◽  
Wenbo Sun ◽  
Dan Xu ◽  
Jiaojiao Ma ◽  
Feng Xiao ◽  
...  

BACKGROUND: Computed tomography (CT) imaging combined with artificial intelligence is important in the diagnosis and prognosis of lung diseases. OBJECTIVE: This study aimed to investigate temporal changes of quantitative CT findings in patients with COVID-19 in three clinic types, including moderate, severe, and non-survivors, and to predict severe cases in the early stage from the results. METHODS: One hundred and two patients with confirmed COVID-19 were included in this study. Based on the time interval between onset of symptoms and the CT scan, four stages were defined in this study: Stage-1 (0 ∼7 days); Stage-2 (8 ∼ 14 days); Stage-3 (15 ∼ 21days); Stage-4 (> 21 days). Eight parameters, the infection volume and percentage of the whole lung in four different Hounsfield (HU) ranges, ((-, -750), [-750, -300), [-300, 50) and [50, +)), were calculated and compared between different groups. RESULTS: The infection volume and percentage of four HU ranges peaked in Stage-2. The highest proportion of HU [-750, 50) was found in the infected regions in non-survivors among three groups. CONCLUSIONS: The findings indicate rapid deterioration in the first week since the onset of symptoms in non-survivors. Higher proportion of HU [-750, 50) in the lesion area might be a potential bio-marker for poor prognosis in patients with COVID-19.


2021 ◽  
pp. 481-484
Author(s):  
Masahisa Watanabe ◽  
Harumasa Yokota ◽  
Hiroshi Aso ◽  
Hirotsugu Hanazaki ◽  
Junya Hanaguri ◽  
...  

Herein, we report the longitudinal observation of a case with reopening of the macular hole associated with a lamellar macular hole-associated epiretinal proliferation (LHEP) followed by spontaneous closure in patients with stage 2 idiopathic macular hole. A 64-year-old woman was referred for the decreased visual acuity (VA) and acute anorthopia in the right eye. Funduscopy and optical coherence tomography (OCT) showed stage 2 full-thickness macular hole without posterior vitreous detachment (PVD) and operculum formation. Her best-corrected visual acuity (BCVA) was 20/32. One month later, the diameter of the macular hole was getting small and VA improved. Six months later, the macular hole was treated spontaneously with the attached hyaloid membrane to the macula by OCT and the BCVA improved to 20/20. Fourteen months after the first visit, the BCVA decreased to 20/50 and the patient was diagnosed with stage 4 macular hole with complete PVD. OCT showed full-thickness macular hole with a LHEP in the right eye. After 25G-gauge vitrectomy with the peeling of internal limiting membrane (ILM) and LHEP, the macular hole was closed and BCVA finally improved to 20/25. Spontaneous macular hole closure without PVD may rarely occur in patients with LHEP. The surgical removal of ILM and LHEP may contribute to the successful macular hole closure after vitrectomy.


2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Alessandro Roggeri ◽  
Daniela Paola Roggeri ◽  
Carlotta Rossi ◽  
Marco Gambera ◽  
Rossana Piccinelli ◽  
...  

Abstract Background and Aims Chronic kidney disease (CKD) is a chronic illness with important implications for the health of the population and for the commitment of resources by public health services. CKD staging makes it possible to assess the severity of the disease and its distribution in the population. The distribution of the stages of CKD diagnosed through hospitalization were analyzed using administrative database of the Local Health Authority of a province with a population of about 1 million inhabitants in northern Italy. Method Patients with hospital discharge with a diagnosis of CKD (ICD9CM 5851, 5852, 5853, 5854) in 2011- 2012 years, without dialysis treatment, neither transplantation procedure nor acute renal failure were selected. Demographic characteristics, comorbidities, dialysis treatment, drugs prescription and nephrological follow-up were investigated. This cohort of patients was examined over a 7-year period (2011-2017). Stage five was not considered to avoid possible misunderstanding with five D stage. Results 1808 patients diagnosed with CKD were extracted from the 2011-2017 administrative database; of these, 1267 had a diagnosis with the CKD stage specification. The distribution of 1267 patients in the CKD stages at the first hospital discharge was as follows: 7.4% stage 1, 30.9% stage 2, 42.3% stage 3, 19.3% stage 4. The 832 patients described in the study were still alive as of Jan. 1, 2013 while 435 (34.3%) died by Dec. 31, 2012. Until Dec. 31, 2017, 503 of the 832 patients died representing the 52.8% of stage 1 patients, 62% of stage 2 patients, 58.2% of stage 3 patients, 66.4% of stage 4 patients. Males were the most prevalent gender (58.5%), without any significant difference into CKD stages. Our patients have a fairly high age as can be seen from the table 1. The presence of co-morbidities was assessed either directly for the main risk factors or by the modified Charlson index (MCI) for CKD patients. The average value of the MCI is 3.8 ± 3.1 for all patients and 3.4 ±3.0 for stage 1, 4.1 ± 3.3 for stage 2, 3.7 ± 3.1 for stage 3, 3.7 ± 2.9 for stage 4, with maximum values of 12.0, 17.0, 16.0 and 14.0 respectively. About 40% of patients had diabetes mellitus, with the highest prevalence in stage 4 (49.3%) and the lowest in stage 1 (25%). Cardiovascular disease was distributed almost equally among all patients with a value between 82% in stage 1 and 86.3% in stage 4. Cancer were present in 26.3% of patients with similar values in all stages. Just about 9% of patients underwent dialysis treatment for achieving ESRD, with a percentage of 5.6% among patients in stage 1 and 17.1% among those in stage 4. Hemodialysis represented first choice treatment (86%) compared with peritoneal one (14%). Time from the diagnosis of CKD to the first dialysis was variable with an average of 3.4 ±1.7 years; the longest interval for patients in stage 1 (5.1±1.8) and the shortest (3.0 ±1.6) for patients in stage 4. The number of nephrological visits at renal units was analyzed for an assessment of the extent of follow-up and prevention upon reaching the ESRD (table2). More than 90% of patients had prescribed drugs antagonists of the renin angiotensin system, in all stages of CKD; other antihypertensive drugs (Ca channel blockers and peripheral vasodilators) had a similar prescription level. Anemia control drugs (ESA and iron) had an incremental prescription with stages of the disease from 51.4% in stage 1 to 74% in stage 4, similarly to Ca-P metabolism control drugs ranging from 44.4% in stage 1 to 67.8% in stage 4. Conclusion Correct staging of CKD is very important to assess the prognosis of patients, but the major determinants of outcome are comorbidities and age of the patients. The cohort examined has a high mortality rate, far higher than reported in the literature for CKD. It should be noted that the sample was identified by hospitalization for cardiovascular diseases more than 50% complicated by diabetes and hypertension, so death represents the main outcome and not ESRD.


2017 ◽  
Vol 28 (3) ◽  
pp. 272-277 ◽  
Author(s):  
Cemil Yildiz ◽  
Yusuf Erdem ◽  
Kenan Koca

Introduction: The aim of this study was to report the clinical and radiological outcomes for 21 patients (28 hips) treated for osteonecrosis of the femoral head using the lightbulb technique, a nonvascularised bone grafting technique. Methods: The study group included 14 men and 7 women, with a mean age of 33.2 (range 22-50) years, presenting with avascular necrosis of the femoral head of stage 4a or earlier, according to the Steinberg classification. Patients were treated using the nonvascularised lightbulb bone grafting technique. The primary clinical outcome was the Harris Hip Scores (HHS), while primary outcomes of treatment effectiveness and disease progression were based on radiographic evidence of disease progression and the need for total hip replacement. The rate of treatment success and failure was evaluated using the Kaplan-Meier survival analysis. Results: The mean HHS increased from 52.66 to 74.33 after treatment, with excellent-to-good outcomes obtained in 21 (75%) of the cases. Fair-to-poor results were obtained in 7 (25%) of the cases, with total hip arthroplasty subsequently required in 5 of these cases. The radiological failure rate was 50% among cases treated in Steinberg stage 1 (1/2), 42% in stage 3 (5/12), and 100% in stage 4 (2/2). Conclusions: The lightbulb technique can provide a clinically acceptable rate of successful treatment of osteonecrosis of the femoral head when used in the early stages of the disease, prior to collapse of the femoral head.


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