scholarly journals Biological Drivers of Wilms Tumor Prognosis and Treatment

Children ◽  
2018 ◽  
Vol 5 (11) ◽  
pp. 145 ◽  
Author(s):  
Hannah Phelps ◽  
Saara Kaviany ◽  
Scott Borinstein ◽  
Harold Lovvorn

Prior to the 1950s, survival from Wilms tumor (WT) was less than 10%. Today, a child diagnosed with WT has a greater than 90% chance of survival. These gains in survival rates from WT are attributed largely to improvements in multimodal therapy: Enhanced surgical techniques leading to decreased operative mortality, optimization of more effective chemotherapy regimens (specifically, dactinomycin and vincristine), and inclusion of radiation therapy in treatment protocols. More recent improvements in survival, however, can be attributed to a growing understanding of the molecular landscape of Wilms tumor. Particularly, identification of biologic markers portending poor prognosis has facilitated risk stratification to tailor therapy that achieves the best possible outcome with the least possible toxicity. The aim of this review is to (1) outline the specific biologic markers that have been associated with prognosis in WT and (2) provide an overview of the current use of biologic and other factors to stratify risk and assign treatment accordingly.

Aorta ◽  
2016 ◽  
Vol 04 (02) ◽  
pp. 33-41 ◽  
Author(s):  
Tyler Gunn ◽  
Sotiris Stamou ◽  
Nicholas Kouchoukos ◽  
Kevin Lobdell ◽  
Kamal Khabbaz ◽  
...  

Background: The goal of this study was to compare the early and late outcomes of different techniques of proximal root reconstruction during the repair of acute Type A aortic dissection, including aortic valve (AV) resuspension, aortic valve replacement (AVR), and a root replacement procedure. Methods: All patients who underwent acute Type A aortic dissection repair between January 2000 and October 2010 at four academic institutions were compiled from each institution’s Society of Thoracic Surgeons Database. This included 189 patients who underwent a concomitant aortic valve (AV) procedure; 111, 21, and 57 patients underwent AV resuspension, AVR, and the Bentall procedure, respectively. The median age of patients undergoing a root replacement procedure was significantly younger than the other two groups. Early clinical outcomes and 10-year actuarial survival rates were compared. Trends in outcomes and surgical techniques throughout the duration of the study were also analyzed. Results: The operative mortality rates were 17%, 29%, and 18%, for AV resuspension, AVR, and root replacement, respectively. Operative mortality (p = 0.459) was comparable between groups. Hemorrhage related re-exploration did not differ significantly between groups (p = 0.182); however, root replacement procedures tended to have decreased rates of bleeding when compared to AVR (p = 0.067). The 10-year actuarial survival rates for the AV resuspension, Bentall, and AVR groups were 72%, 56%, and 36%, respectively (log-rank p = 0.035). Conclusions: The 10-year actuarial survival was significantly lower in those receiving AVR compared to those receiving root replacement procedures or AV resuspension. Operative mortality was comparable between the three groups.


2016 ◽  
Vol 82 (6) ◽  
pp. 487-492 ◽  
Author(s):  
Don K. Nakayama ◽  
Patrick C. Bonasso

Multimodal therapy—surgery, radiation therapy, and chemotherapy—the foundation of modern cancer treatment, has led to dramatic improvements in survival. How the three disciplines coalesced to conquer Wilms’ tumor is a compelling story that includes two of history's greatest discoveries, X-rays and antibiotics. By the mid-20th century both fields had matured to where dedicated clinicians and creative scientists could apply them to Wilms’ tumor and achieve successive improvements in survival. William Ladd was able to achieve a zero operative mortality by 1940, but was left with a 32 per cent survival with surgery alone. Robert Gross and Edwin Neuhauser combined surgery and radiotherapy and achieve 47 per cent survival rate in 1950. Sidney Farber and his colleagues added an antibiotic, dactinomycin, to the treatment regimen and reached 80 per cent survival rate in 1966. The National Wilms’ Tumor Study, organized in 1968, was a multidisciplinary effort of surgeons, radiotherapists, and pediatric oncologists across the country. By the 1990s, the National Wilms’ Tumor Study achieved survival rates above 95 per cent while minimizing long-term effects through shortening courses of chemotherapy and radiation. The story of Wilms’ tumor serves as a paragon for all types of cancer, in both children and adults.


Author(s):  
Deenadayalan Theivasikamani ◽  
Lakshminarasimhan Srinivasan ◽  
Ramkumar Bakthavachalam

Cancer Cervix is the most common women related cancer in India. It mainly affects middle aged female of 40-55 years age group, largely attributed to HPV infection. Advances in radiation therapy, surgical techniques and chemotherapy, have resulted in improved survival rates. Still 20-30% patients with cancer cervix would develop recurrent or persistent disease. Although the typical manifestations of recurrent cervical carcinoma are pelvic masses and lymphadenopathy, less typical manifestations like solid organ metastases and peritoneal carcinomatosis also can occur. The increasing prevalence of these less typical manifestations is partially related to the use of intensive pelvic radiation therapy, resulting in a shift away from pelvic recurrence toward distant metastasis, as well as related to improvements in cross-sectional imaging techniques. This article describes a clinical case of a 40-year-old patient with cancer cervix, who achieved complete remission with concurrent chemo radiotherapy and 3 years later, she relapsed with adrenal metastasis.


2011 ◽  
Vol 07 (01) ◽  
pp. 48 ◽  
Author(s):  
Stacey L Urbach ◽  
Normand Laperriere ◽  
Ute Bartels ◽  
◽  
◽  
...  

Radiation therapy is an integral part of the treatment of paediatric patients with medulloblastoma and ependymoma. With newer surgical techniques and adjuvant chemotherapy regimens, recurrence-free survival rates have improved dramatically. Unfortunately, improved survival has come at the cost of significant late effects, including endocrine dysfunction. Prior to the 1990s, radiation fields were planned using 2D X-rays, exposing large volumes of healthy tissue to harmful radiation. Over the past two decades, radiation oncologists have increasingly applied 3D computed tomography scans to radiation therapy planning. By shaping target fields more precisely, higher doses of radiation may be delivered directly to the tumour while limiting exposure to healthy tissues and reducing side effects. The objective of this article is to review recent evidence about late endocrine effects among survivors of childhood medulloblastoma and ependymoma who received radiation therapy and to assess whether the introduction of 3D radiation planning techniques has affected the prevalence of these effects. Patients treated for medulloblastoma with current doses of craniospinal radiation continue to be at high risk of growth hormone deficiency and primary hypothyroidism, even when conformal radiation is used to deliver posterior fossa radiation. Patients with ependymoma who are treated with focal radiation alone, however, demonstrate fewer late endocrine effects.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Eik Schiegnitz ◽  
Lena Katharina Müller ◽  
Keyvan Sagheb ◽  
Lisa Theis ◽  
Vahide Cagiran ◽  
...  

Abstract Background and purpose The aim of this clinical study was to investigate the clinical long-term and patient-reported outcome of dental implants in patients with oral cancer. In addition, analysis of the influence of radiation therapy, timing of implant insertion, and augmentation procedures on implant survival was performed. Material and methods This retrospective study investigated the clinical outcome of 711 dental implants in 164 oral cancer patients, inserted by experienced surgeons of the Department of Oral and Maxillofacial Surgery, University Medical Center Mainz, Germany. Oral health-related quality of life (OHRQoL) was evaluated. Results Cumulative 5-year and 10-year implant survival rates for all included implants were 87.3% and 80.0%. Implants placed straight after ablative surgery (primary implant placement) and implants placed after completing the oncologic treatment (secondary implant placement) showed a comparable implant survival (92.5% vs. 89.5%; p = 0.635). Irradiation therapy had no significant influence on implant survival of secondary placed implants (p = 0.929). However, regarding implant site (native bone vs. augmented bone) and radiation therapy (non-irradiated bone vs. irradiated bone), implants inserted in irradiated bone that received augmentation procedures showed a statistically significant lower implant survival (p < 0.001). Patients reported a distinct improvement in OHRQoL. Conclusions Promising long-term survival rates of dental implants in patients after treatment of oral cancer were seen. In addition, patients benefit in form of an improved OHRQoL. However, bone augmentation procedures in irradiated bone may result in an impaired implants’ prognosis.


2021 ◽  
Vol 82 (01) ◽  
pp. 081-090
Author(s):  
Jacquelyn Laplant ◽  
Kimberly Cockerham

Abstract Objective Primary orbital malignancy is rare. Awareness of the characteristic clinical and imaging features is imperative for timely identification and management. Surgery remains an important diagnostic and treatment modality for primary orbital malignancy, but determining the optimal surgical approach can be challenging. The purpose of this article is to explore recent advances in the diagnosis, management, and surgical approaches for primary orbital malignancies. Design In this review, the clinical presentation, imaging features, and medical and surgical management of primary orbital malignancies with representative cases will be discussed. Setting Outpatient and inpatient hospital settings. Participants Patients with diagnosed primary orbital malignancies. Main Outcome Measures Descriptive outcomes. Results Advancements in orbital imaging, microsurgical techniques, and multimodal therapy have improved the diagnosis and management of primary orbital malignancies. Special considerations for biopsy or resection are made based on the tumor's location, characteristics, nearby orbital structures, and goals of surgery. Minimally invasive techniques are supplanting traditional approaches to orbital surgery with less morbidity. Conclusions Advances in imaging technologies and surgical techniques have facilitated the diagnosis and management of primary orbital malignancies. Evolution toward less invasive orbital surgery with focus on preservation and restoration of function is underway.


2018 ◽  
Vol 5 (2) ◽  
pp. 1
Author(s):  
Sylvester Luu ◽  
Brian C. Benson ◽  
Kelly A. Haeusler ◽  
Robert O. Brady ◽  
Katherine M. Cebe ◽  
...  

A 60-year-old male with prior history of laryngeal carcinoma and active smoking presented with six months of solid food dysphagia. Endoscopy showed a large, friable gastroesophageal junction mass. Biopsies revealed a high-grade, poorlydifferentiated neuroendocrine carcinoma. He was subsequently started on platinum based chemotherapy and radiation therapy and his tumor decreased dramatically in size. This case is unique as neuroendocrine carcinomas (NECs) are rarely found in the esophagus and usually have a poor prognosis at time of diagnosis.


1999 ◽  
Vol 17 (7) ◽  
pp. 2127-2127 ◽  
Author(s):  
Roger J. Packer ◽  
Joel Goldwein ◽  
H. Stacy Nicholson ◽  
L. Gilbert Vezina ◽  
Jeffrey C. Allen ◽  
...  

PURPOSE: Medulloblastoma is the most common malignant brain tumor of childhood. After treatment with surgery and radiation therapy, approximately 60% of children with medulloblastoma are alive and free of progressive disease 5 years after diagnosis, but many have significant neurocognitive sequelae. This study was undertaken to determine the feasibility and efficacy of treating children with nondisseminated medulloblastoma with reduced-dose craniospinal radiotherapy plus adjuvant chemotherapy. PATIENTS AND METHODS: Over a 3-year period, 65 children between 3 and 10 years of age with nondisseminated medulloblastoma were treated with postoperative, reduced-dose craniospinal radiation therapy (23.4 Gy) and 55.8 Gy of local radiation therapy. Adjuvant vincristine chemotherapy was administered during radiotherapy, and lomustine, vincristine, and cisplatin chemotherapy was administered during and after radiation. RESULTS: Progression-free survival was 86% ± 4% at 3 years and 79% ± 7% at 5 years. Sites of relapse for the14 patients who developed progressive disease included the local tumor site alone in two patients, local tumor site and disseminated disease in nine, and nonprimary sites in three. Brainstem involvement did not adversely affect outcome. Therapy was relatively well tolerated; however, the dose of cisplatin had to be modified in more than 50% of patients before the completion of treatment. One child died of pneumonitis and sepsis during treatment. CONCLUSION: These overall survival rates compare favorably to those obtained in studies using full-dose radiation therapy alone or radiation therapy plus chemotherapy. The results suggest that reduced-dose craniospinal radiation therapy and adjuvant chemotherapy during and after radiation is a feasible approach for children with nondisseminated medulloblastoma.


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