scholarly journals The Diagnosis and Treatment of Craniopharyngioma

2019 ◽  
Vol 110 (9-10) ◽  
pp. 753-766
Author(s):  
Hermann L. Müller
Keyword(s):  
Histological Grade ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
High Standard ◽  
Proton Beam Therapy ◽  
Braf V600e ◽  
Treatment Quality ◽  
Multidisciplinary Teams ◽  
Centers Of Excellence ◽  
Neuropsychological Sequelae

Craniopharyngioma (CP) is a rare embryonic malformation of the sellar/parasellar region with a low histological grade. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. Recent insight into the molecular pathogenesis of CP opens new perspectives on targeted therapy in papillary CP harboring BRAF-V600E mutations. Further research to elucidate pathogenic mechanisms and hopefully prevent hypothalamic involvement of CP is warranted. If the tumor is favorably localized, the therapy of choice is complete resection, with care taken to preserve the optical and hypothalamic functions. In patients with unfavorable tumor localization (i.e., hypothalamic involvement), the recommended therapy is a limited hypothalamus-sparing surgical strategy followed by local irradiation. Surgical treatment strategies should be based on a multidisciplinary approach involving experienced teams. Centralizing the treatment of CP in experienced “centers of excellence” and multicenter-based networks for reference assessments should be considered to assure a high standard of treatment quality. CP recurrence and progression are frequent. Irradiation has proven effective in reducing recurrences and progression. Proton beam therapy, available in a wider range in the near future, will help to avoid radio-oncological side effects. Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious sequelae that compromise quality of life (QoL), such as hypothalamic obesity and psychopathological symptoms. Novel insights into neuropsychological sequelae after CP occurrence should be the basis for the development of therapeutic neuropsychological interventions. CP should be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients’ clinical and QoL consequences by experienced multidisciplinary teams.

scholarly journals MANAGEMENT OF ENDOCRINE DISEASE: Childhood-onset craniopharyngioma: state of the art of care in 2018

2019 ◽  
Vol 180 (4) ◽  
pp. R159-R174 ◽  
Author(s):  
Hermann L Müller
Keyword(s):  
Multidisciplinary Treatment ◽  
Treatment Strategies ◽  
Residual Tumor ◽  
Proton Beam Therapy ◽  
Treatment Quality ◽  
Special Focus ◽  
Childhood Onset ◽  
Centers Of Excellence ◽  
Neuropsychological Sequelae ◽  
High Standards

This review presents an update on current concepts of pathogenesis, diagnostics, multidisciplinary treatment and follow-up care, with special focus on neuropsychological sequelae of childhood-onset craniopharyngioma (CP) based on most recent publications on these topics. Recent insight in molecular pathogenesis of CP opens new perspectives on targeted therapy. Further research to elucidate pathogenic mechanisms and to prevent hypothalamic involvement of CP is warranted. Surgical treatment strategies should be based on a multidisciplinary approach involving experienced teams aiming at posterior hypothalamus-sparing treatment for prevention of quality of life impairments. Centralization of CP treatment in experienced ‘centers of excellence’ is recommended. However, such centralization includes high thresholds concerning infrastructure not achievable in all health systems. Alternatives such as multicenter-based networks used for reference assessments should be considered to assure high standards of treatment quality. Irradiation is efficient in preventing further growth or recurrence in CP patients with residual tumor. Proton beam therapy – available on a wider range in the near future – will help to avoid radiooncological side effects. Novel insights into neuropsychological sequelae after CP should be the basis for the development of future therapeutic neuropsychological interventions. Due to the rareness of the disease, common international efforts in research and treatment are recommended and should lead to an international registry for childhood-onset CP, as a first step toward efficient coordination of scientific and clinical initiatives.

scholarly journals Craniopharyngioma

2014 ◽  
Vol 35 (3) ◽  
pp. 513-543 ◽  
Author(s):  
Hermann L. Müller
Keyword(s):  
Quality Of Life ◽  
Histological Grade ◽  
Survival Rates ◽  
Age Distribution ◽  
Clinical Manifestations ◽  
Incidence Rates ◽  
Multidisciplinary Teams ◽  
Childhood Onset ◽  
Cognitive Problems

Abstract This report is a review of findings on the diagnosis, treatment, clinical course, and prognosis of craniopharyngioma patients. Craniopharyngiomas are rare, partly cystic and calcified embryonic malformations of the sellar/parasellar region with low histological grade (WHO I°). A bimodal age distribution has been shown, with peak incidence rates in childhood-onset at 5–14 years and adult-onset craniopharyngioma at 50–74 years. Clinical manifestations are related to hypothalamic/pituitary deficiencies, visual impairment, and increased intracranial pressure. If the tumor is favorably localized, the therapy of choice is complete resection, with care taken to preserve optical and hypothalamic functions. In patients with unfavorable tumor localization (ie, hypothalamic involvement), recommended therapy is a limited hypothalamus-sparing surgical strategy followed by local irradiation. Although overall survival rates are high (92%), recurrences and progressions are frequent. Irradiation has proven effective in reducing recurrences and progression, and timing of postsurgical irradiation in childhood-onset cases is currently under investigation in a randomized multinational trial (KRANIOPHARYNGEOM 2007). Anatomical involvement and/or surgical lesions of posterior hypothalamic areas can result in serious quality of life-compromising sequelae such as hypothalamic obesity, psychopathological symptoms, and/or cognitive problems. It is crucial that craniopharyngioma be managed as a frequently chronic disease, providing ongoing care of pediatric and adult patients' clinical and quality of life consequences by experienced multidisciplinary teams.

Using Naïve Bayes Algorithm to Estimate the Response to Drug in Lung Cancer Patients

2019 ◽  
Vol 21 (10) ◽  
pp. 734-748 ◽  
Author(s):  
Baoling Guo ◽  
Qiuxiang Zheng
Keyword(s):  
Lung Cancer ◽  
Side Effects ◽  
Cancer Patients ◽  
Drug Response ◽  
Treatment Options ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Cancer Resistance ◽  
Lung Cancer Patients ◽  
Bayes Algorithm

Aim and Objective: Lung cancer is a highly heterogeneous cancer, due to the significant differences in molecular levels, resulting in different clinical manifestations of lung cancer patients there is a big difference. Including disease characterization, drug response, the risk of recurrence, survival, etc. Method: Clinical patients with lung cancer do not have yet particularly effective treatment options, while patients with lung cancer resistance not only delayed the treatment cycle but also caused strong side effects. Therefore, if we can sum up the abnormalities of functional level from the molecular level, we can scientifically and effectively evaluate the patients' sensitivity to treatment and make the personalized treatment strategies to avoid the side effects caused by over-treatment and improve the prognosis. Result & Conclusion: According to the different sensitivities of lung cancer patients to drug response, this study screened out genes that were significantly associated with drug resistance. The bayes model was used to assess patient resistance.

scholarly journals Primary immune deficiencies with defects in neutrophil function

Hematology ◽  
2016 ◽  
Vol 2016 (1) ◽  
pp. 43-50 ◽  
Author(s):  
Mary C. Dinauer
Keyword(s):  
Fungal Infections ◽  
Leukocyte Adhesion ◽  
Genetic Disorders ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Adaptor Protein ◽  
Invasive Fungal Disease ◽  
Neutrophil Function ◽  
Microbial Pathogens ◽  
Immune Deficiencies

Abstract Immune deficiencies resulting from inherited defects in neutrophil function have revealed important features of the innate immune response. Although sharing an increased susceptibility to bacterial and fungal infections, these disorders each have distinctive features in their clinical manifestations and characteristic microbial pathogens. This review provides an update on several genetic disorders with impaired neutrophil function, their pathogenesis, and treatment strategies. These include chronic granulomatous disease, which results from inactivating mutations in the superoxide-generating nicotinamide dinucleotide phosphate oxidase. Superoxide-derived oxidants play an important role in the control of certain bacterial and fungal species, and also contribute to the regulation of inflammation. Also briefly summarized are updates on leukocyte adhesion deficiency, including the severe periodontal disease characteristic of this disorder, and a new immune deficiency associated with defects in caspase recruitment domain–containing protein 9, an adaptor protein that regulates signaling in neutrophils and other myeloid cells, leading to invasive fungal disease.

scholarly journals Dry Eye Syndrome in Patients with Diabetes Mellitus: Prevalence, Etiology, and Clinical Characteristics

2016 ◽  
Vol 2016 ◽  
pp. 1-7 ◽  
Author(s):  
Xinyuan Zhang ◽  
Lin Zhao ◽  
Shijing Deng ◽  
Xuguang Sun ◽  
Ningli Wang
Keyword(s):  
Diabetes Mellitus ◽  
Dry Eye ◽  
Keratoconjunctivitis Sicca ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Dry Eye Syndrome ◽  
Surface System ◽  
Patients With Diabetes ◽  
Function Unit ◽  
Substantial Progress

There has been substantial progress in our understanding of the ocular surface system/lacrimal function unit in the past 15 years. Keratoconjunctivitis sicca, more commonly referred to as dry eye syndrome (DES), is the most frequently encountered condition and diabetes mellitus (DM) has been identified as one of the leading causes of DES. Poor glycemic control affects both the anterior and the posterior segments of the eye and increasing prevalence of diabetes-associated DES (DMDES) has been reported in recent years. The pathogenesis and specific features of DMDES remain uncertain and interventions are limited to those used in DES. This review outlines the pathogenesis, clinical manifestations, and the current preventive and treatment strategies for diabetes-related DES.

Anaplastic Thyroid Cancer

2019 ◽  
Author(s):  
Geeta Lal
Keyword(s):  
Thyroid Cancer ◽  
Kinase Inhibitors ◽  
Braf Inhibitor ◽  
Anaplastic Thyroid Cancer ◽  
Mek Inhibitor ◽  
Braf V600e ◽  
Multidisciplinary Teams ◽  
Neoadjuvant Radiotherapy ◽  
Current State ◽  
Targeted Treatments

Anaplastic thyroid cancer (ATC) is a rare thyroid malignancy with a nearly uniform poor prognosis. Most patients present with advanced disease, and optimal management requires rapid diagnosis, staging, and involvement of multidisciplinary teams. Treatment may include surgery in patients with resectable disease and adjuvant or neoadjuvant radiotherapy and chemotherapy. Improved understanding of molecular pathogenesis has allowed the assessment of tyrosine kinase inhibitors and other targeted treatments in these patients.  The FDA recently approved the combination of dabrafenib (BRAF inhibitor) and trametinib (MEK inhibitor) for the treatment of BRAF V600E mutation positive, unresectable or metastatic ATC. This review summarizes the current state-of-the-art concepts in the management of patients with ATC. This review contains 3 figures, 2 tables, and 25 references. Key words: anaplastic thyroid cancer, goals of care discussion, management, surgery, radiotherapy, chemotherapy novel therapies, NCCN and ATA guidelines

scholarly journals Staging of Locally Advanced Rectal Cancer Beyond TME

2020 ◽  
Vol 33 (05) ◽  
pp. 258-267
Author(s):  
Deborah S. Keller
Keyword(s):  
Rectal Cancer ◽  
Best Practice ◽  
Locally Advanced ◽  
Treatment Strategies ◽  
Treatment Decision ◽  
Advanced Rectal Cancer ◽  
Cancer Staging ◽  
Locally Advanced Rectal Cancer ◽  
Multidisciplinary Teams ◽  
Treatment Decision Making

AbstractThe management of rectal cancer is complex and continually evolving. With advancements in technology and the use of multidisciplinary teams to guide the treatment decision making, staging, oncologic, and functional outcomes are improving, and the management is moving toward personalized treatment strategies to optimize each individual patient's outcomes. Key in this evolution is imaging. Magnetic resonance imaging (MRI) has emerged as the dominant method of pelvic imaging in rectal cancer, and use of MRI for staging is best practice in multiple international guidelines. MRI allows a noninvasive assessment of the tumor site, relationship to surrounding structures, and provides highly accurate rectal cancer staging, which is necessary for determining the appropriate treatment strategy. However, the applications of MRI extend far beyond pretreatment staging. MRI can be used to predict outcomes in locally advanced rectal cancer and guide the surgical or nonsurgical plan, serving as a predictive and prognostic biomarker. With continued MRI hardware improvement and new sequence development, MRI may offer new perspectives in the assessment of treatment response and new innovations that could provide better insight into the staging, restaging, and outcomes with rectal cancer.

The Role of Centers of Excellence With Multidisciplinary Teams in the Management of Abnormal Invasive Placenta

2018 ◽  
Vol 61 (4) ◽  
pp. 841-850 ◽  
Author(s):  
ALIREZA A. SHAMSHIRSAZ ◽  
KARIN A. FOX ◽  
HADI ERFANI ◽  
MICHAEL A. BELFORT
Keyword(s):  
Multidisciplinary Teams ◽  
Centers Of Excellence ◽  
Invasive Placenta

Preliminary Exploration of the Diagnosis and Treatment of Skull-Based Chondromyxoid Fibromas

2017 ◽  
Vol 15 (3) ◽  
pp. 270-277
Author(s):  
Kangmin He ◽  
Shize Jiang ◽  
Xin Zhang ◽  
Ying Mao ◽  
Wei Zhu ◽  
...  
Keyword(s):  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Diagnosis And Treatment ◽  
Benign Tumors ◽  
Symptom Duration ◽  
Study Cohort ◽  
Imaging Characteristics ◽  
Subtotal Removal ◽  
Base Of The Skull

Abstract BACKGROUND Chondromyxoid fibromas (CMFs) are benign tumors that occur rarely in the skull base. OBJECTIVE To conduct a preliminary exploration of the diagnosis and treatment of cranial CMFs. METHODS A retrospective analysis of 19 cases of CMFs in the base of the skull between 2009 and 2014 in our hospital was conducted. The clinical manifestations, imaging characteristics, pathology, treatment strategies, and outcomes were examined. RESULTS The study cohort included 7 women (36.8%) and 12 men (63.2%), and symptom duration ranged from 1 mo to 5 yr. Of the 19 intracranial CMF cases examined, 15 (78.9%) conformed with the diagnostic criteria for extracranial CMF. Resection operations yielded subtotal removal of 13 tumors (68.4%) and partial removal of 6 tumors (31.6%). Postoperative pathological analysis demonstrated that the tumors were characterized by spindle-shaped or stellate cells arranged in a myxoid matrix without mitoses or permeation. Follow-up (range 2-7.3 yr; mean, 4.4 ± 1.7 yr) revealed that symptoms improved postoperatively in 15 cases (78.9%), were maintained in 2 cases (10.5%), and worsened in 2 cases (10.5%). Imaging follow-up revealed that residual tumors were stable in 18 cases (94.7%) and enlarged in 1 case (5.3%). CONCLUSION An accurate diagnosis should involve comprehensive consideration of clinical, radiological, and pathological features. The treatment strategy for CMFs consists of maximizing tumor removal while protecting adjacent key structures. Postoperative stereotactic radiotherapy is appropriate for residual tumors.

scholarly journals Lipoma of the cerebellopontine angle: case reports and literature review

1994 ◽  
Vol 52 (1) ◽  
pp. 58-63 ◽  
Author(s):  
Marcelo P. Ferreira ◽  
Nelson P. Ferreira ◽  
Rene Lenhardt
Keyword(s):  
Surgical Treatment ◽  
Literature Review ◽  
Cerebellopontine Angle ◽  
Case Reports ◽  
Clinical Manifestations ◽  
Treatment Strategies ◽  
Diagnostic Methods ◽  
Surgical Indications ◽  
Complete Excision

Two patients with cerebellopontine angle (CPA) lipoma were studied. They were submitted to surgical treatment. Available literature was reviewed and 29 cases with same lesion were identified which had been treated by surgery. Clinical manifestations, possibility of diagnostic methods, surgical indications and treatment strategies are discussed. Attention is called to the peculiarities of CPA lipomas and the doubtful vality of attempting complete excision in all cases.

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