Necrobiotic Xanthogranuloma (NXG) Associated with Monoclonal Gammopathies (MG): Clinical Features and Treatment Outcomes

Blood ◽  
2015 ◽  
Vol 126 (23) ◽  
pp. 1830-1830 ◽  
Author(s):  
Larissa Higgins ◽  
Ronald S. Go ◽  
Shaji Kumar ◽  
S. Vincent Rajkumar ◽  
Francis Buadi ◽  
...  
Keyword(s):  
Clinical Response ◽  
Treatment Outcomes ◽  
Clinical Features ◽  
Skin Disease ◽  
Systemic Treatment ◽  
Case Series ◽  
High Dose ◽  
Study Cohort ◽  
Monoclonal Gammopathies

Abstract BACKGROUND: Necrobiotic Xanthogranuloma (NXG) is a rare chronic granulomatous disorder of the skin associated with monoclonal gammopathies (MG). Due to its rarity, data on response to various therapies are largely limited to single case reports or small case series. Hence, we described the clinical features in patients with NXG and MG as well as their subsequent disease course and response to treatment. METHODS: This was a retrospective study evaluating 35 patients with NXG and coexisting MG from 1994-2015 who were evaluated in the Hematology Outpatient Clinic at the Mayo Clinic, Rochester. All patients had a biopsy proven diagnosis of NXG with the concurrent presence of a serum monoclonal gammopathy. RESULTS: The median age at diagnosis was 56 years (range: 26-88). Most patients had a plasma cell dyscrasia (28 MGUS; 5 Smoldering myeloma) while 2 had chronic lymphocytic leukemia. Themost common monoclonal (M) protein was IgG kappa (60%). The median M-spike was 1.1 g/dL (range: 0-3). The most common site of NXG was periocular (66%). All patients received treatment, with over half requiring >3 different lines of treatment. The treatments were heterogeneous and included excision, intra-lesional injection, radiotherapy as well as systemic treatment. The types of systemic treatment used and clinical response are reported in Table 1. The median follow up was 46 months (range: 4-234) and the median survival was not reached but at the time of analysis 80% were still alive. At last follow-up, 80% of patients had signs of either clinical improvement or stable skin disease. Eight patients (23%) had disease progression to multiple myeloma at a median of 67 months (Range: 21 - 107). CONCLUSIONS: We report the clinical findings and treatment outcomes of one of the largest series of patients with NXG associated with MG. The clinical course is generally indolent but malignant transformation is not uncommon. Cutaneous objective responses can be achieved with various systemic agents used in the treatment of lymphoproliferative malignancies. Table 1. Treatment Types and Clinical Response Rates Treatment No of patientsA Clinical Benefit Rate B Response rateC Chemotherapy - Chlorambucil +/- Steroids - Melphalan/Steroids - Cytoxan/Steroids - Cladribine - Vincristine/doxorubicin/dexamethasone - High dose therapy with stem cell rescue - Fludarabine/cyclophosphamide/rituximab 5 3 4 3 2 3 1 2/5 (40%) 0/3 (0%) 1/4 (25%) 1/3 (33%) 1/2 (50%) 2/3 (67%) 1/1 (100%) 1/5 (20%) 0/3 (0%) 0/4 (0%) 1/3 (33%) 0/2 (0%) 2/3 (67%) 1/1 (100%) Novel agents - Thalidomide +/- Steroids - Lenalidomide +/- Steroids - Bortezomib +/- Steroids - Bortezomib/Lenalidomide/Dexamethasone 11 14 4 1 5/11 (45%) 10/14 (71%) 2/4 (50%) 1/1 (100%) 4/11 (36%) 7/14 (50%) 1/4 (25%) 1/1 (100%) Intravenous immunoglobulin 4 3/4 (75%) 2/4 (50%) Systemic steroids alone 11 4/11 (36%) 4/11 (36%) Rituximab 6 2/6 (33%) 1/6 (17%) AntibioticsD 3 0/3 (0%) 0/3 (0%) ImmunosuppressantsE 6 1/6 (17%) 0/6 (0%) ANo of patients who underwent a particular therapy amongst the 35 patients in the study cohort. BClinical Benefit: Improvement or stable skin disease CResponse: Improvement in skin disease DAntibiotics: Fluoroquinolones, Tetracycline EImmunosuppressants: Cyclosporine, Methotrexate, Plaquenil Disclosures Kumar: Celgene, Millennium, Onyx, Novartis, Janssen, Sanofi: Research Funding; Celgene, Millennium, Onyx, Janssen, Noxxon, Sanofi, BMS, Skyline: Consultancy; Skyline, Noxxon: Honoraria.

Treatment Outcomes in Multiple Sclerosis (MS) Patients after Early, Conventional and Salvage High Dose Chemotherapy (HDCT)+Autologous Stem Cell Transplantation (ASCT).

Blood ◽  
2005 ◽  
Vol 106 (11) ◽  
pp. 5467-5467
Author(s):  
Yury L. Shevchenko ◽  
Andrei A. Novik ◽  
Boris V. Afanasiev ◽  
Igor A. Lisukov ◽  
Tatiana I. Ionova ◽  
...  
Keyword(s):  
Clinical Response ◽  
Treatment Outcomes ◽  
Patient Reported Outcomes ◽  
High Dose ◽  
Base Line ◽  
Primary Progressive ◽  
Patient Reported ◽  
Relapsing Remitting ◽  
Clinical Stabilization

Abstract HDCT+ASCT is a new and promising therapy for MS patients. Among a number of unclear questions is the terms of conducting HDCT+ASCT. According to our concept there are 3 strategies of HDCT+ASCT depending on the terms of disease process: early, conventional and salvage. Another important consideration is that the major treatment outcomes for MS patients are disease-progressive free period and improvement of patient’s quality of life (QoL). With this in mind, evaluation of both clinical and patient-reported outcomes in MS patients after HDCT+ASCT is worthwhile. We aimed to study the clinical and QoL response in MS patients after early, conventional and salvage HDCT +ASCT. 17 patients with MS (secondary progressive − 8 patients, primary progressive − 6, progressive relapsing − 2, relapsing remitting − 1) were included in the study (mean age − 32.3, SD − 6.6; male/female − 4/13). Fifteen patients underwent conventional HDCT +ASCT; one patient (relapsing-remitting MS) - early HDCT+ASCT and one patient (primary progressive MS) - salvage HDCT+ASCT. Median EDSS at base-line was 6.0 (range 2.0 – 7.5). The median follow-up duration was 12 months (range 6 – 72 months). All of the patients had previously undergone conventional treatment. Neurological and QoL evaluation was provided at baseline, at discharge, 3, 6, 9, 12 months, and then every 6 months. MRI was conducted at baseline, at 6, 12 months, and at the end of follow-up. FACT-BMT and FAMS were used for QoL evaluation. QoL response was evaluated using Integral QoL index, which was calculated by the method of integral profiles. 14 (82.4%) out of 17 patients including 12 patients with conventional HDCT+ ASCT, one patient with early HDCT+ ASCT and one patient with salvage HDCT+ ASCT experienced a clinical stabilization or improvement. Three patients showed significant improvement in EDSS (by more than 1.0 point), 2 patients improved by 1.0, and 3 patients - by 0.5. Six cases remained stable. All of the patients with clinical stabilization and improvement exhibited negative MRI scans. One patient continuously worsened and died 3 years after the transplantation. Two other patients worsened by 0.5 points in their EDSS. All patients with clinical response exhibited improved QoL at 6 months post-transplant and they preserved improved QoL at the end of follow-up. At one year after HDCT+ASCT the patients exhibited a good or excellent QoL response. These levels of QoL response were preserved throughout the follow-up period. In conclusion, clinical response was observed in 82.4% MS patients after HDCT+ASCT. Notably, patients undergoing early, conventional and salvage HDCT+ASCT exhibited clinical response. All patients with clinical response had good or excellent QoL response. Thus, HDCT+ASCT appears to be an effective treatment for MS both in terms of clinical and patient-reported outcomes. The data obtained point to feasibility of early, conventional and salvage HDCT+ASCT in MS patients. Further studies should be done to investigate clinical and QoL response in MS patients receiving early, conventional and salvage HDCT+ASCT to better define treatment success.

Multicenter Experience of High Dose Chemotherapy (HDCT)+Autologous Stem Cell Transplantation (ASCT) in Multiple Sclerosis (MS) Patients.

Blood ◽  
2006 ◽  
Vol 108 (11) ◽  
pp. 3072-3072
Author(s):  
Yury L. Shevchenko ◽  
Andrei A. Novik ◽  
Alexey N. Kuznetcov ◽  
Boris V. Afanasiev ◽  
Igor A. Lisukov ◽  
...  
Keyword(s):  
Clinical Response ◽  
Treatment Outcomes ◽  
Therapeutic Option ◽  
Treatment Success ◽  
High Dose ◽  
Base Line ◽  
Post Transplantation ◽  
Patient Reported ◽  
Clinical Stabilization

Abstract During the last decade HDCT+ASCT is more often used as a therapeutic option for MS patients. The major treatment outcomes for MS patients are disease-progressive free period and improvement of patient’s quality of life (QoL). We aimed to study treatment outcomes in MS patients after early, conventional and salvage HDCT+ASCT. Thirty two patients with MS (secondary progressive - 16 patients, primary progressive - 8, progressive-relapsing - 1, relapsing-remitting - 7) from 6 medical centers were included in this study (mean age - 32.0, range: 19–49; male/female - 12/20). Two patients underwent early HDCT +ASCT, 27 patients - conventional HDCT+ASCT and 3 patients - salvage HDCT+ASCT. Median EDSS at base-line was 6.0 (range 2.0 – 8.0). The median follow-up duration was 18 months (range 6 – 78 months). All of the patients had previously undergone conventional treatment. Neurological and QoL evaluation was provided at baseline, at discharge, 3, 6, 9, 12 months, and then every 6 months after HDCT+ASCT. MRI was conducted at baseline, at 6, 12 months, and at the end of follow-up. FACT-BMT and FAMS were used for QoL evaluation. QoL response was evaluated using Integral QoL index. All 27 patients with the follow-up longer than 1 year, included in the analysis, experienced a clinical stabilization or improvement. More than half of them improved: 6 patients showed significant improvement in EDSS (by more than 1.0 point), 4 patients improved by 1.0 point, and 5 patients - by 0.5 points on EDSS. Twelve patients achieved stabilization. Two patients deteriorated to a worse score after 18 months of stabilization; 2 other patients progressed after 12 and 30 months of improvement, respectively. All the patients with clinical stabilization and improvement exhibited negative MRI scans. Out of 21 patients included in the analysis of QoL response 19 exhibited improved QoL 6 months post-transplantation. At one year after HDCT+ASCT 1 patient exhibited excellent QoL response; 3 patients - good QoL response; 7 patients - moderate QoL response and 8 patients - minimal QoL response. At 2.5 years post-transplantation 3 more patients had excellent QoL response. Further QoL improvement was observed at longer follow-up. One of the patients who experienced progression after stabilization had no QoL response; another patient who progressed after stabilization had stable QoL during 6 months post-transplant and significantly worsened at 9 months. In conclusion, clinical response was observed in 100% of MS patients after early, conventional and salvages HDCT+ASCT. The majority of patients with clinical response had a good or moderate QoL response. Further studies should be done to investigate the clinical and patient-reported outcomes of HDCT+ASCT in MS patients to better define treatment success.

scholarly journals Treatment Outcomes of Lesser Tuberosity Osteotomy Nonunion After Total Shoulder Arthroplasty

2018 ◽  
Vol 2 ◽  
pp. 247154921880777
Author(s):  
Zachary J Bloom ◽  
Cesar D Lopez, BA ◽  
Stephen P Maier ◽  
Brian B Shiu ◽  
Djuro Petkovic ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Shoulder Arthroplasty ◽  
Surgical Repair ◽  
Total Shoulder Arthroplasty ◽  
Case Series ◽  
Radiographic Evaluation ◽  
Lesser Tuberosity ◽  
Conservative Care ◽  
Conservative Group

Introduction Lesser tuberosity osteotomy (LTO) during anatomic total shoulder arthroplasty has a 13% nonunion rate. Treatment for LTO nonunion is controversial and poorly described in the literature. The purpose of this study was to compare the surgical and nonsurgical treatment outcomes of LTO nonunion. Methods A retrospective case series of 9 consecutive patients with LTO nonunion after primary anatomic shoulder arthroplasty at 1 institution from 2010 to 2016 were studied. Outcomes measured were radiographic evaluation of LTO on axillary X-ray, clinical range of motion (ROM), subscapularis strength, and pain at the time of LTO nonunion diagnosis and after either conservative care or surgical repair of the LTO nonunion. Results LTO nonunion was treated surgically in 4 and conservatively in 5 patients with average follow-up of 30 and 22 months, respectively. There were no significant differences in age, sex, or smoking status between groups. Treatment decision was a shared model of surgeon and patient. Displaced LTO nonunion was treated surgically in 2 and conservatively in 3 patients. There were no differences in LTO union rate of 50% in the surgical versus 60% in the conservative group. Abdominal compression test was abnormal in 50% of surgical versus 40% of conservative groups. At follow-up, ROM was lower in the surgical group with 128° forward elevation (FE) and 33° external rotation (ER) compared to 148° FE and 62° ER. Only 1 patient with LTO nonunion required conversion to reverse replacement. Conclusion LTO nonunion after shoulder arthroplasty is rare. Surgical repair of LTO nonunion does not significantly improve clinical or radiographic outcomes compared to conservative care.

scholarly journals P1693LONG-TERM OUTCOME OF KIDNEY TRANSPLANTATION IN PLASMA CELLS DYSCRASIAS

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Fulvia Zappulo ◽  
Gabriele Donati ◽  
Giorgia Comai ◽  
Claudia Bini ◽  
Andrea Angeletti ◽  
...  
Keyword(s):  
Kidney Transplantation ◽  
Light Chain ◽  
Graft Failure ◽  
Case Series ◽  
University Hospital ◽  
High Dose ◽  
Light Chain Deposition Disease ◽  
Light Chain Amyloidosis ◽  
First Case

Abstract Background and Aims Survival of patients with Multiple Myeloma (MM), Light Chain Amyloidosis (LCA) and Monoclonal Gammopathies of renal significance (MGRS) on chronic renal replacement therapy (RRT) is poor. The gold standand treatment of plasma cell dyscrasias (PCD) is high-dose chemotherapy followed by Autologous Stem Cell Transplantation (ASCT) which can induce complete remission and longer survival than chemotherapy alone. Kidney transplantation (KT) after ASCT could represent an option for patients with PCD and End Stage Renal Disease (ESRD). There is no evidence about the time of follow up required from MM remission and KT. Method We present a case series of 5 patients who underwent KT after ASCT and remission of MM among 2,500 transplant recipients followed at the Nephrology Dialysis and Renal Transplantation Unit of S.Orsola University Hospital from 1967 untill now. As in case of recovery from solid cancers, the feasibility of KT after MM was considered when no signs of relapse were assessed. In our cohort 3 patients were affected by Light Chain Deposition Disease (LCDD), 1 patient presented Myeloma Cast Nephropathy (MCN) and one patient Light Chain Amyloidosis (LCA). They all required RRT and underwent KT after ASCT. Results Time between ASCT and KT ranged from 3 and 11 years and clinical outcome was very different. The mean follow up period ranged from 2 to 4 years. In the first case (LCDD) KT was performed 11 years after ASCT, the graft failure occurred 6 years later because of chronic allograft nephropathy requiring RRT. In the second case (LCDD) patient received KT 3 years after ASCT. He developed Bence-Jones proteinuria requiring specific therapy with Dexametasone and Bortezomib determining progressive graft failure. In the third case (LCDD) KT was performed 4 years after ASCT and the 4 year follow up is negative for relapse of MM or ESRD. The fourth patient presented MCN and received KT 8 years after ASCT. MCN relapsed 6 years later; it caused ESRD requiring RRT. In the last patient (LCA) KT was performed 4 years after ASCT. No recurrence occurred in a 2-year follow up. Conclusion MM is the most frequent malignancy in dialytic population; the need for KT in MM remains high. ASCT improves the quality of life and offers higher survival in patients with myeloma/MGRS/amyloidosis-related ESRD. Therefore the combination of chemotherapy/ASCT and KT is pivotal to pursue renal restoring. Since high risk of recurrence larger study are required to clarify the better follow up period after MM remission and KT.

scholarly journals Extracorporeal irradiation of tumorous calvaria: a case series

2015 ◽  
Vol 122 (5) ◽  
pp. 1127-1130
Author(s):  
Daryoush Tavanaiepour ◽  
William C. Broaddus ◽  
Theodore D. Chung ◽  
Kathryn L. Holloway ◽  
Michelle A. Proper ◽  
...  
Keyword(s):  
Tumor Recurrence ◽  
Case Series ◽  
B Cell Lymphoma ◽  
Bone Flap ◽  
High Dose ◽  
Allogeneic Bone ◽  
Who Grade ◽  
Extracorporeal Irradiation

OBJECT When intracranial tumors invade the overlying skull, gross resection typically includes removal of the involved bone. Methods used to repair the resulting structural defect in the cranium include artificial prostheses, allogeneic bone grafts, and autoclaving the autologous graft. The authors have previously reported a case involving high-dose extracorporeal ionizing radiation to treat the tumorous calvaria intraoperatively, followed by reimplantation of the treated bone flap. In this paper the authors report the long-term follow-up of that case, as well as results of using extracorporeal irradiation of tumorous calvaria (EITC) for an additional 20 patients treated similarly. METHODS The decision to undergo EITC was typically anticipated preoperatively, but determined intraoperatively, if upon inspection the bone flap was invaded by tumor. The bone flap was then delivered to the radiation oncology department, where a total dose of 120 Gy was delivered, using a clinical linear accelerator, over a period of approximately 15 minutes. After the intracranial tumor resection was completed, the irradiated craniotomy bone flap was reimplanted and the wound was closed in a standard fashion. A retrospective review of patients who had undergone EITC was performed for evidence of calvarial tumor recurrence or other complications. RESULTS Since the originally reported case, 20 additional patients have received EITC during craniotomy for invasive tumors. Eighteen (86%) of 21 patients were diagnosed with meningioma: 12 (67%) with WHO Grade I, 5 (28%) with WHO Grade II, and 1 with WHO Grade III (6%). The remaining 3 patients presented with dural-based B-cell lymphoma with extensive adjacent bone invasion (n = 2) and metastatic adenocarcinoma of the lung (n = 1). Follow-up of the 21 patients ranged from 1 to 132 months, with a mean of 41 months and a median of 23 months. No patients have experienced tumor recurrence, infection associated with the treated calvaria, or evidence of bone flap resorption. CONCLUSIONS Calvaria reconstructions represent an important component in structural and cosmetic outcome following craniectomy for tumorous bone. The authors' long-term experience with EITC has been excellent with no local tumor recurrence or complications. Therefore, EITC represents an excellent and efficient option for cranial reconstruction in such patients.

scholarly journals Intracranial hemorrhage from dural arteriovenous fistulas: clinical features and outcome

2013 ◽  
Vol 34 (5) ◽  
pp. E15 ◽  
Author(s):  
David J. Daniels ◽  
Ananth K. Vellimana ◽  
Gregory J. Zipfel ◽  
Giuseppe Lanzino
Keyword(s):  
Clinical Features ◽  
Intracranial Hemorrhage ◽  
Case Series ◽  
Venous Drainage ◽  
Sudden Onset ◽  
Residual Symptoms ◽  
Arteriovenous Fistulas ◽  
Common Location ◽  
Dural Arteriovenous Fistulas

Object In this paper the authors' goal was to review the clinical features and outcome of patients with intracranial dural arteriovenous fistulas (DAVFs) who presented with hemorrhage. Methods A retrospective study of 28 patients with DAVFs who presented with intracranial hemorrhage to 2 separate institutions was performed. The information reviewed included clinical presentation, location and size of hemorrhage, angiographic features, treatment, and clinical and radiologically documented outcomes. Clinical and radiological follow-up were available in 27 of 28 patients (mean follow-up 17 months). Results The vast majority of patients were male (86%), and the most common presenting symptom was sudden-onset headache. All DAVFs had cortical venous drainage, and about one-third were associated with a venous varix. The most common location was tentorial (75%). Treatment ranged from endovascular (71%), surgical (43%), Gamma Knife surgery (4%), or a combination of modalities. The majority of fistulas (75%) were completely obliterated, and most patients experienced excellent clinical outcome (71%, modified Rankin Scale score of 0 or 1). There were no complications in this series. Conclusions Case series, including the current one, suggest that the vast majority of patients who present with intracranial hemorrhage from a DAVF are male. The most common location for DAVFs presenting with hemorrhage is tentorial. Excellent outcomes are achieved with individualized treatment, which includes various therapeutic strategies alone or in combination. Despite the hemorrhagic presentation, almost two-thirds of patients experience a full recovery with no or minimal residual symptoms.

Multiple Myeloma Profile In Latin America: Clinical and Epidemiological Observational Study

Blood ◽  
2013 ◽  
Vol 122 (21) ◽  
pp. 5327-5327
Author(s):  
Vania T M Hungria ◽  
Angelo Maiolino ◽  
Gracia Aparecida Martinez ◽  
Carmino A De Souza ◽  
Rosane Bittencourt ◽  
...  
Keyword(s):  
Multiple Myeloma ◽  
Overall Survival ◽  
Latin America ◽  
Clinical Features ◽  
Median Overall Survival ◽  
High Dose Chemotherapy ◽  
Patterns Of Care ◽  
Disease Stage ◽  
High Dose

Abstract Introduction Little is known about the incidence and clinical features of Multiple Myeloma (MM) in Latin America. A clinical registry of Latin American (LA) patients with MM represents an opportunity to gain insight into the prevalence of the disease in this region, the patterns of care and the current treatment status in different LA countries. Objective To characterize the demographic and clinical features of patients with multiple myeloma from five LA countries (Brazil, Argentina, Chile, Mexico and Peru) and to create a LA database on MM; in addition to investigating the patterns of care for MM patients in Latin America. Patients and Methods This is an observational, non-intervention study, with a prospective evaluation of data. Eligible patients were diagnosed with multiple myeloma, between January 1, 2005, and December 31, 2007, at any one of the participating centers, regardless of disease stage or treatment modality. The follow-up period extended to at least 5 years for each patient (December 31, 2012). Results Eight hundred and seventy six patients were included. The median age was 60 years old (25-97), 53.4% male and 46.6% female. The median follow-up was 31.4 months, and the median overall survival was 57 months. The median overall survival to patients who received high-dose chemotherapy was 77 months and for patients who received conventional chemotherapy was 48 months (p<0.001). The multivariate prognostic model included patient baseline variables that were associated with mortality in the Kaplan-Meier univariate analyses. Only hypercalcemia, DSS II and III, ISS stage III andnon- high-dose chemotherapy were independent predictors of mortality. Conclusion This current study, which is the largest case series of MM patients in Latin America, recognizes the feasibility of large, collaborative, observational studies among various tertiary-care hematology centers in Latin America. Note We will present more details related to the demographic and most frequently used treatments in Latin America for newly diagnosed and relapsed patients in these LA countries. Disclosures: No relevant conflicts of interest to declare.

scholarly journals Review of Clinical Features, Microbiological Spectrum, and Treatment Outcomes of Endogenous Endophthalmitis over an 8-Year Period

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Zenith H. Y. Wu ◽  
Rose P. S. Chan ◽  
Fiona O. J. Luk ◽  
David T. L. Liu ◽  
Carmen K. M. Chan ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Clinical Features ◽  
Outcome Measure ◽  
Visual Outcome ◽  
Endogenous Endophthalmitis ◽  
Light Perception ◽  
Gram Negative ◽  
Finger Counting ◽  
The Mean

Purpose. To evaluate the clinical features, microbiological spectrum, and treatment outcomes of endogenous endophthalmitis.Methods. Retrospective review of consecutive cases with infective endogenous endophthalmitis presenting from 2000 to 2007. The main outcome measure was the visual outcome at the latest follow-up visit. Other outcome measures included microbiological investigations, anatomical and clinical outcomes.Results. 22 eyes of 21 patients were included, and the mean follow-up duration was 2.7 years. Eyes with fungal endogenous endophthalmitis were more likely to have visual acuity of finger counting or better at presentation compared with those with bacterial endogenous endophthalmitis (odds , ). Gram-negative microorganisms accounted for 50% of infections, while fungal and gram-positive organisms accounted for 27.3% and 22.7%, respectively. Despite treatment, the visual outcome was poor in general as 10 (45.5%) eyes had no light perception at the latest follow-up visit and 6 (27.3%) eyes required enucleation or evisceration. Contrary to previous studies, fungal endogenous endophthalmitis did not appear to have better visual outcome compared with bacterial endogenous endophthalmitis.Conclusion. Gram-negative microorganisms were the main causative pathogens of endogenous endophthalmitis in Hong Kong. The visual prognosis of endogenous endophthalmitis is generally poor as almost 50% of eyes were blind despite treatment.

scholarly journals Clinical Features and Treatment Outcomes of Vulvar Sarcoma: A Single-Institution Review of 21 Cases

2020 ◽  
Author(s):  
Haifeng Gu ◽  
Guochen Liu ◽  
Lili Liu ◽  
Zhimin Liu ◽  
Junyun Li ◽  
...  
Keyword(s):  
Treatment Outcomes ◽  
Clinical Features ◽  
Epithelioid Sarcoma ◽  
Pelvic Mass ◽  
Adjuvant Chemoradiotherapy ◽  
Wide Resection ◽  
Cancer Center ◽  
Group A ◽  
Group B

Abstract BackgroundValvar sarcoma is an extremely rare disease in which the data for treatment and prognosis are scarce. The aim of this study was to analysis the clinical features and treatment outcomes of initial and recurrent valvar sarcoma.MethodThe medical records of patients with initial or recurrent vulvar sarcoma treated at Sun Yat-sen University Cancer Center between January 2005 and April 2019 were retrospectively reviewed.Results A total of 21 patients with vulvar sarcoma were identified. The median age of them was 43 years (range: 16 to 71). The histological subtypes were epithelioid sarcoma, leiomyosarcoma, dermatofibrosarcoma protuberans, rhabdomyosarcoma, and angiosarcoma in 9, 4, 4, 3 and 1 patients, respectively. None of the patients received wide resection when they were initially treated at communities. Thirteen patients were referred immediately after initial managements (Group A), whereas 8 patients were referred with recurrent disease (Group B). Wide resections were performed with pathologically negative margins in 20 patients, of whom 9 also received lymphadenectomy and 3 showed metastases. One patient in Group B only received chemoradiotherapy for unresectable pelvic mass. Adjuvant chemoradiotherapy were performed for 12 patients evaluated to have high risk of recurrence. The median follow-up time was 39 months (range: 11 to 174) for Group A and 107 (range: 2 to 139) for Group B. During the follow-up, relapse occurred in 3 patients in Group A and 5 in Group B. The 3- and 5-year survival rates were 92.3% and 76.9% for Group A, 50.0% and 37.5% for Group B, respectively.ConclusionsVS is usually resected as benign disease at initial management. Immediate extended resection with negative margins provided favorable outcomes for most of the patients. The prognosis for recurrent cases were poor, however there remained survival opportunities if aggressive treatments were carried out.

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