Epithelioid and Spindle-Celled Leiomyosarcoma of the Heart

1999 ◽  
Vol 123 (9) ◽  
pp. 782-788 ◽  
Author(s):  
Michael R. Pins ◽  
Margaret A. Ferrell ◽  
Joren C. Madsen ◽  
Quirino Piubello ◽  
G. Richard Dickersin ◽  
...  
Keyword(s):  
Low Grade ◽  
High Grade ◽  
Short Term ◽  
Term Survival ◽  
Female Ratio ◽  
Small Series ◽  
Systemic Spread ◽  
Pathologic Features

Abstract Background.—Primary cardiac leiomyosarcomas are rare. Isolated reported cases and small series generally describe spindle-celled, high-grade tumors with poor short-term survival; however, the pathologic features of many of these tumors are incompletely documented. The authors report in detail the clinicopathologic features of 2 relatively low-grade epithelioid and spindle-celled primary cardiac leiomyosarcomas. Methods.—Cases 1 and 2 were studied using standard histochemical and immunohistochemical techniques, and case 1 was examined by electron microscopy. The literature was reviewed with regard to primary cardiac leiomyosarcomas. Results.—Both tumors showed epithelioid and spindle-celled areas. The tumor in case 1 was low grade, and the tumor in case 2 was predominately low grade with a high-grade focus. A review of 28 reported cases revealed a wide age range (mean, 43 years), equal male-to-female ratio, and a predilection for the left atrium (48%). Follow-up of reported cases with fewer than 5 mitoses per 10 high-power fields showed a mean survival of 22 months compared with a 9-month mean survival for all others. Conclusions.—Short-term follow-up of reported cases of high-grade cardiac leiomyosarcoma suggests a poor prognosis. Long-term follow-up in our case 2, along with follow-up of reported cases that were histologically similar to our cases, suggests that cardiac leiomyosarcomas with low-grade features or mixed low- and high-grade features also have a poor overall long-term survival, with a high rate of local recurrence and systemic spread.

xxx

2007 ◽  
Vol 25 (18_suppl) ◽  
pp. 8123-8123
Author(s):  
C. Tarella ◽  
M. Zanni ◽  
A. Rambaldi ◽  
F. Benedetti ◽  
R. Passera ◽  
...  
Keyword(s):  
Complete Remission ◽  
High Dose ◽  
Low Grade ◽  
High Grade ◽  
Term Outcome ◽  
Term Survival ◽  
Long Term Outcome ◽  
Long Term Survival

8123 Background: The high-dose sequential (HDS) chemotherapy approach, including early dose-intensification and autograft with peripheral blood progenitor cells (PBPC), was introduced several years ago (Gianni & Bonadonna, 1989); subsequently, it has been broadly used in the management of both non-Hodgkin s (NHL) and Hodgkin s Lymphoma (HL). The outcome of a large series of lymphoma patients treated with the HDS approach at 10 GITIL Centers is reported. Methods: Data have been collected on 1,266 patients, who received either the original or slightly modified HDS regimens. There were 213 HL and 1,053 NHL (630 intermediate/high-grade, 423 low-grade); median age was 46 yrs. Overall, 671 (53%) patients had refractory/relapsed disease, 595 (47%) were at diagnosis. Most patients were autografted with PBPC; 158 (12%) patients did not undergo autografting due to toxicity, disease progression or poor harvests. Results: Overall, 1,013 (80%) patients reached Complete Remission (CR) following HDS. As to December 2006, 93 (7%) patients died for early/late toxicities, 328 (26%) died for lymphoma, 844 are known to be alive. At a lead follow-up of 18 years, and a median follow-up of 5 yrs, the 5-yr Overall Survival (OS) projection is 64% (S.E.: 2%). The long-term survival was quite favorable in patients achieving a Complete Remission (CR), with a 5-yr OS projection of 76%. The prolonged OS in patients achieving CR was consistent in all lymphoma subtypes, i.e. both low and high-grade NHL (5-yr OS: 77% in both), and HL (5-yr OS: 72%). Patients at diagnosis had a significantly better outcome compared to patients treated for relapsed/refractory disease, again CR achievement was associated with prolonged survival in both subgroups (82% and 69%, respectively, at 5 yrs.). On multivariate Cox survival analysis, CR achievement was the most powerful predictor of long-term survival (HR 0.13, c.i.: 0.10–0.17). Lastly, achieving substantial tumor reduction before autografting had a major influence on the clinical outcome. Conclusions: 1. the HDS program is feasible in a multicenter setting; 2. the long-term outcome is well influenced by the CR status after HDS; 3. the influence of CR achievement on the long-term survival holds true in all lymphoma subtypes, including indolent lymphomas; 4. an adequate pre-autograft tumor debulking may contribute to a favorable long-term outcome. [Table: see text]

scholarly journals Urothelial Tumors of the Urinary Bladder in Young Patients: A Clinicopathologic Study of 59 Cases

2013 ◽  
Vol 137 (10) ◽  
pp. 1337-1341 ◽  
Author(s):  
Melissa L. Stanton ◽  
Li Xiao ◽  
Bogdan A. Czerniak ◽  
Charles C. Guo
Keyword(s):  
Urinary Bladder ◽  
Clinical Outcomes ◽  
Young Patients ◽  
Muscularis Propria ◽  
Low Grade ◽  
High Grade ◽  
Urothelial Carcinomas ◽  
Pathologic Features ◽  
Urothelial Tumors

Context.—Urothelial tumors are rare in young patients. Because of their rarity, the natural history of the disease in young patients remains poorly understood. Objective.—To understand the pathologic and clinical features of urothelial tumors of the urinary bladder in young patients. Design.—We identified 59 young patients with urothelial tumors of the urinary bladder treated at our institution and analyzed the tumors' pathologic features and the patients' clinical outcomes. Results.—All patients were 30 years or younger, with a mean age of 23.5 years (range, 4–30). Thirty-eight patients (64%) were male, and 21 (36%) were female. Most tumors were noninvasive, papillary urothelial tumors (49 of 59; 83%), including papillary urothelial neoplasms of low malignant potential (7 of 49; 14%), low-grade papillary urothelial carcinomas (38 of 49; 78%), and high-grade papillary urothelial carcinomas (4 of 49; 8%). Only a few (n = 10) of the urothelial tumors were invasive, invading the lamina propria (n = 5; 50%), muscularis propria (n = 4; 40%), or perivesical soft tissue (n = 1; 10%). Clinical follow-up information was available for 41 patients (69%), with a mean follow-up time of 77 months. Of 31 patients with noninvasive papillary urothelial tumors, only 1 patient (3%) later developed an invasive urothelial carcinoma and died of the disease, and 30 of these patients (97%) were alive at the end of follow-up, although 10 (32%) had local tumor recurrences. In the 10 patients with invasive urothelial carcinomas, 3 patients (30%) died of the disease and 5 others (50%) were alive with metastases (the other 2 [20%] were alive with no recurrence). Conclusion.—Urothelial tumors in young patients are mostly noninvasive, papillary carcinomas and have an excellent prognosis; however, a small subset of patients may present with high-grade invasive urothelial carcinomas that result in poor clinical outcomes.

Secondary brain tumors in children treated for acute lymphoblastic leukemia at St Jude Children's Research Hospital.

1998 ◽  
Vol 16 (12) ◽  
pp. 3761-3767 ◽  
Author(s):  
A W Walter ◽  
M L Hancock ◽  
C H Pui ◽  
M M Hudson ◽  
J S Ochs ◽  
...  
Keyword(s):  
Risk Factors ◽  
Acute Lymphoblastic Leukemia ◽  
Brain Tumors ◽  
Lymphoblastic Leukemia ◽  
Low Grade ◽  
High Grade ◽  
Research Hospital ◽  
Dose Dependent

PURPOSE To evaluate the incidence of and potential risk factors for second malignant neoplasms of the brain following treatment for childhood acute lymphoblastic leukemia (ALL). PATIENTS AND METHODS The study population consisted of 1,612 consecutively enrolled protocol patients treated on sequential institutional protocols for newly diagnosed ALL at St Jude Children's Research Hospital (SJCRH) between 1967 and 1988. The median follow-up duration is 15.9 years (range, 5.5 to 29.9 y). RESULTS The cumulative incidence of brain tumors at 20 years is 1.39% (95% confidence interval [CI], 0.63% to 2.15%). Twenty-two brain tumors (10 high-grade gliomas, one low-grade glioma, and 11 meningiomas) were diagnosed among 21 patients after a median latency of 12.6 years (high-grade gliomas, 9.1 years; meningiomas, 19 years). Tumor type was linked to outcome, with patients who developed high-grade tumors doing poorly and those who developed low-grade tumors doing well. Risk factors for developing any secondary brain tumor included the presence of CNS leukemia at diagnosis, treatment on Total X therapy, and the use of cranial irradiation, which was dose-dependent. Age less than 6 years was associated with an increased risk of developing a high-grade glioma. CONCLUSION This single-institution study, with a high rate of long-term data capture, demonstrated that brain tumors are a rare, late complication of therapy for ALL. We report many more low-grade tumors than others probably because of exhaustive long-term follow-up evaluation. The importance of limiting cranial radiation is underscored by the dose-dependent tumorigenic effect of radiation therapy seen in this study.

Long-term outcome of hypothalamic/chiasmatic astrocytomas in children treated with conservative surgery

1995 ◽  
Vol 83 (4) ◽  
pp. 583-589 ◽  
Author(s):  
Leslie N. Sutton ◽  
Patricia T. Molloy ◽  
Heidi Sernyak ◽  
Joel Goldwein ◽  
Peter L. Phillips ◽  
...  
Keyword(s):  
Radical Surgery ◽  
Conservative Surgery ◽  
Low Grade ◽  
Term Outcome ◽  
Term Survival ◽  
Long Term Outcome ◽  
Content Type ◽  
Fine Print

✓ The feasibility of radical surgery for astrocytomas of the optic chiasm/hypothalamus has been reported by several groups. Such surgery carries significant risks, however, including permanent damage to the pituitary gland, optic apparatus, hypothalamic structures, and carotid arteries. The benefits of radical surgery, both in terms of efficacy and toxicity, should, therefore, be evaluated against standard therapy, as is usually done for new chemotherapeutic protocols. To this end, a retrospective review was performed of 33 patients treated at Children's Hospital of Philadelphia between 1976 and 1991 who met criteria that would have made them eligible for radical surgery in many centers today, but were treated with either no surgery or conservative surgery (< 50% resection) or biopsy followed by adjuvant therapy with local radiation therapy (29 patients) and/or chemotherapy with actinomycin-D and vincristine (18 patients). The review encompassed all children with a globular enhancing mass of at least 2 cm in the hypothalamic/chiasmatic region, no evidence of optic nerve involvement or involvement of the optic radiations by computerized tomography or magnetic resonance imaging, and follow up of at least 3 years. All but one patient had tissue confirmation of a low-grade or pilocytic astrocytoma. Thirteen of the patients were 2 years of age or younger at diagnosis. Five individuals died: three of tumor progression, one of acute shunt malfunction, and one of intercurrent infection. The remaining 28 were alive at last follow up, a mean of 10.9 years from diagnosis. Twenty-three surviving patients have functional vision in at least one eye, 12 require no endocrine replacement, and 16 are in or have completed schooling with regular academic requirements. If radical surgery is to become standard care for children with low-grade astrocytomas of the hypothalamic/chiasmatic region, long-term survival and functional outcome will have to equal or surpass those of historical controls who were treated conservatively.

Long-term outcomes for low-grade intracranial ganglioglioma: 30-year experience from the Mayo Clinic

2012 ◽  
Vol 117 (5) ◽  
pp. 825-830 ◽  
Author(s):  
Julia J. Compton ◽  
Nadia N. Issa Laack ◽  
Laurence J. Eckel ◽  
David A. Schomas ◽  
Caterina Giannini ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
Mayo Clinic ◽  
Progression Free Survival ◽  
Low Grade ◽  
Term Survival ◽  
Free Survival

Object Gangliogliomas comprise less than 1% of all brain tumors and occur most often in children. Therefore, there are a limited number of patients and data involving the use or role of adjuvant therapy after subtotal resections (STRs) of gangliogliomas. The objective of this study was to examine and review the Mayo Clinic experience of 88 patients with gangliogliomas, their follow-up, risk of recurrence, and the role of radiation therapy after STR or only biopsy. Methods Eighty-eight patients with gangliogliomas diagnosed between 1970 and 2007 were reviewed. Data on clinical outcomes and therapy received were analyzed. The Kaplan-Meier method was used to estimate progression-free survival (PFS) and overall survival. Results The median age at diagnosis was 19 years. The median potential follow-up as of June 2008 was 142 months (range 9–416 months). Fifteen-year overall survival was 94%, median PFS was 5.6 years, with a 10-year PFS rate of 37%. Progression-free survival was dramatically affected by extent of initial resection (p < 0.0001). Conclusions This single-institution retrospective series of patients with gangliogliomas is unique given its large cohort size with a long follow-up duration, and confirms the excellent long-term survival rate in this group. The study also shows the importance of resection extent on likelihood of recurrence. Patients with gangliogliomas who undergo STR or biopsy alone have poor PFS. Radiation therapy may delay time to progression in patients with unresectable disease.

scholarly journals Alterations in ALK/ROS1/NTRK/MET drive a group of infantile hemispheric gliomas

2019 ◽  
Vol 10 (1) ◽  
Author(s):  
Ana S. Guerreiro Stucklin ◽  
Scott Ryall ◽  
Kohei Fukuoka ◽  
Michal Zapotocky ◽  
Alvaro Lassaletta ◽  
...  
Keyword(s):  
Tyrosine Kinases ◽  
Receptor Tyrosine Kinases ◽  
Therapeutic Strategies ◽  
Low Grade ◽  
High Grade ◽  
Term Survival ◽  
Group 3 ◽  
Group 2 ◽  
Group 1

Abstract Infant gliomas have paradoxical clinical behavior compared to those in children and adults: low-grade tumors have a higher mortality rate, while high-grade tumors have a better outcome. However, we have little understanding of their biology and therefore cannot explain this behavior nor what constitutes optimal clinical management. Here we report a comprehensive genetic analysis of an international cohort of clinically annotated infant gliomas, revealing 3 clinical subgroups. Group 1 tumors arise in the cerebral hemispheres and harbor alterations in the receptor tyrosine kinases ALK, ROS1, NTRK and MET. These are typically single-events and confer an intermediate outcome. Groups 2 and 3 gliomas harbor RAS/MAPK pathway mutations and arise in the hemispheres and midline, respectively. Group 2 tumors have excellent long-term survival, while group 3 tumors progress rapidly and do not respond well to chemoradiation. We conclude that infant gliomas comprise 3 subgroups, justifying the need for specialized therapeutic strategies.

Does combined dose intensification radiotherapy improve disease control in resectable retroperitoneal sarcoma? Long-term results of a phase I/II trial.

2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 10050-10050
Author(s):  
Myles JF Smith ◽  
Paul F Ridgway ◽  
Charles N Catton ◽  
Amanda Cannell ◽  
Brian O'Sullivan ◽  
...  
Keyword(s):  
Phase I ◽  
Disease Control ◽  
Retroperitoneal Sarcoma ◽  
Long Term Results ◽  
Low Grade ◽  
Term Survival ◽  
Primary Disease ◽  
Upper Abdomen

10050 Background: Late failure is a challenging problem in retroperitoneal sarcoma (RPS) and reported 10 yr overall survival (OS) rates range from 20-30%. Use of preoperative external beam radiotherapy (XRT) in the management of RPS remains controversial. No RCT and very few prospective trials of any type have been completed. We investigated the effects of preop XRT plus dose escalation with early postop brachytherapy (BT) on long term survival and recurrence in RPS. Methods: From 06/96 to 10/00, 40 patients (25 female) with resectable RPS were entered onto a phase I/II trial of preop XRT (50 Gy) plus postop BT (20-25 Gy). As previously reported, BT to the upper abdomen was associated with significant grade III-V postop toxicity, and from 12/98 on, BT was applied only to cases where the “field at risk” excluded the upper abdomen. Kaplan Meier survival curves were constructed; OS and recurrence free survival (RFS) were compared by log rank (SPSS 19.0). Results: Median age at study entry was 58 (38-70) yrs. Twenty nine patients presented to our center with primary disease (73%), and 22 (55%) had high grade tumors. All patients had preop XRT and total gross resection, while half (n=19) received BT. As of 12/2011, median follow-up time is 108 mos. For the entire study cohort, OS at 5 and 10 yrs were 70% and 65%, respectively; RFS at 5 and 10 yrs were 65% and 58%, respectively. RFS at 5 yrs was reduced in high vs. low grade RPS (50% vs. 83%, p=0.028), but by 10 yrs. was similar in high and low grade tumors (50% vs. 67%, p=ns). RFS was reduced in patients who presented with recurrent vs. primary disease (27% vs. 69% at 10 yrs., p=0.018), as was OS (36% vs. 76% at 10 yrs., p=0.034). Neither OS nor RFS was improved in the cohort of patients who received BT compared to the cohort who did not: at 10 yrs. RFS was 53% +BT and 62% -BT, while OS was 53% and 76%, respectively, p=ns. Conclusions: In this prospective study with mature follow-up, long term OS and RFS in patients who underwent combined preop XRT plus resection of RPS compare favorably with those reported in retrospective institutional and population-based series. Postoperative BT did not contribute to disease control.

scholarly journals The Danish comorbidity in liver transplant recipients study (DACOLT): a non-interventional prospective observational cohort study

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Magda Teresa Thomsen ◽  
Julie Høgh ◽  
Andreas Dehlbæk Knudsen ◽  
Anne Marie Reimer Jensen ◽  
Marco Gelpi ◽  
...  
Keyword(s):  
Risk Factors ◽  
Liver Transplant ◽  
Transplant Recipients ◽  
Short Term ◽  
Term Survival ◽  
Blood Samples ◽  
Liver Transplant Recipients ◽  
Short Term Survival

Abstract Background Liver transplantation is the only curative treatment for patients with end-stage liver disease. Short-term survival has improved due to improved surgical techniques and greater efficacy of immunosuppressive drugs. However, long-term survival has not improved to the same extent as the short-term survival, and the 10-year survival after liver transplantation is 60%. In addition to liver- and transplant-related causes, comorbidities such as cardiovascular, pulmonary, renal, and metabolic diseases have emerged as leading causes of morbidity and mortality in liver transplant recipients. The objective of this study is to assess the burden of comorbidities and identify both liver- and transplant-related risk factors as well as traditional risk factors that contribute to the pathogenesis of comorbidity in liver transplant recipients. Methods/design The Danish Comorbidity in Liver Transplant Recipients (DACOLT) study is an observational, longitudinal study. We aim to include all adult liver transplant recipients in Denmark (n = approx. 600). Participants will be matched by sex and age to controls from the Copenhagen General Population Study (CGPS) and the Copenhagen City Heart Study (CCHS). Physical and biological measures including blood pressure, ankle–brachial index, spirometry, exhaled nitric oxide, electrocardiogram, transthoracic echocardiography, computed tomography (CT) angiography of the heart, unenhanced CT of chest and abdomen and blood samples will be collected using uniform protocols in participants in DACOLT, CGPS, and CCHS. Blood samples will be collected and stored in a research biobank. Follow-up examinations at regular intervals up to 10 years of follow-up are planned. Discussion There is no international consensus standard for optimal clinical care or monitoring of liver transplant recipients. This study will determine prevalence, incidence and risk factors for comorbidity in liver transplant recipients and may be used to provide evidence for guidelines on management, treatment and screening and thereby contribute to improvement of the long-term survival. Trial registration ClinicalTrials.gov: NCT04777032; date of registration: March 02, 2021.

scholarly journals Very long-term of survival, 5 years and more in diffuse intrinsic pontine brainstem gliomas in children and adolescents treated with Radiotherapy and Nimotuzumab

2021 ◽  
Vol 8 (2) ◽  
pp. 86-90
Author(s):  
Alert J ◽  
Chon I ◽  
Valdes J ◽  
Ropero R ◽  
Perez M ◽  
...  
Keyword(s):  
Monoclonal Antibody ◽  
Children And Adolescents ◽  
Median Dose ◽  
Short Term ◽  
Term Survival ◽  
Diffuse Intrinsic Pontine Gliomas ◽  
Improved Outcome ◽  
Short Term Survival

Diffuse intrinsic brainstem gliomas have a bad prognosis, and short-term survival time. Radiotherapy has been the principal treatment, and chemotherapy has not improved outcome. The anti –EGFR monoclonal antibody Nimotuzumab combined with Radiotherapy was tested in a series of 41 children and adolescents with diffuse intrinsic pontine gliomas (DIPG) included between January 2008 and December 2015 and a follow-up till January 2021.They were irradiated in the Instituto Nacional de Oncologia y Radiobiologia, Havana, Cuba with a median dose of 54 Gy. Nimotuzumab was applied at a dose of 150 mg/m2, weekly during the period of irradiation, then every 2 weeks by 8 doses, and them monthly for 1,2 or more years. A response was observed in 87.8% of patients. Prolonged use of Nimotuzumab was feasible and well tolerated. Median age at diagnosis was 7 years old, median survival was 18.8 months. There were minor toxicities, only Grade I or II. Survival rate at 5 years was 34.1%, stablished till years or more. Two relapsing patients were re-irradiated. The combination of irradiation and Nimotuzumab is an option to increase survival in DIPG.

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