scholarly journals Multiple meningiomas arising within the same hemisphere associated with Li-Fraumeni syndrome

2021 ◽  
Vol 12 ◽  
pp. 99
Author(s):  
Christopher S. Hong ◽  
E. Zeynep Erson-Omay ◽  
Jennifer Moliterno
Keyword(s):  
Right Hemisphere ◽  
Neurofibromatosis Type ◽  
World Health ◽  
Li Fraumeni Syndrome ◽  
Multiple Meningiomas ◽  
Radiation History ◽  
Li Fraumeni ◽  
Health Organization ◽  
The Right ◽  
Time Of Presentation

Background: While meningiomas are some of the most common intracranial tumors, the presence of multiple ones at the time of presentation is rare and can most commonly be observed in patients with well-described syndromes (i.e., neurofibromatosis type 2) or those with prior cranial radiation history. In others, however, the pathophysiology remains unclear. Case Description: A 49-year-old female with no significant personal or familial oncologic medical history presented with a generalized seizure and was found to have ten meningiomas arising within the right hemisphere. She underwent a two-staged resection of all tumors, with pathology revealing the World Health Organization Grade I meningioma. Whole-exome sequencing revealed somatic NF2 mutations and heterozygous deletion of chromosome 22 overlapping with NF2, and analysis of the germline uncovered mutations of TP53, rendering a diagnosis of Li-Fraumeni Syndrome. Conclusions: This case represents a novel presentation of multiple meningiomas in a patient with newly diagnosed Li-Fraumeni syndrome, suggesting meningioma may be considered as part of this tumor-predisposed patient population.

Emergence of a High-Grade Sarcoma in a Recurrent Meningioma: Malignant Progression or Collision Tumor?

2011 ◽  
Vol 135 (7) ◽  
pp. 935-940
Author(s):  
Buge Oz ◽  
Melike Pekmezci ◽  
Reza Dashti ◽  
Kutlay Karaman ◽  
Cengiz Kuday ◽  
...  
Keyword(s):  
Malignant Transformation ◽  
Neurofibromatosis Type ◽  
Collision Tumor ◽  
World Health ◽  
Malignant Neoplasms ◽  
High Grade ◽  
Multiple Meningiomas ◽  
Short Period ◽  
High Grade Sarcoma ◽  
Health Organization

Abstract Anaplastic meningiomas that resemble sarcomas often reveal clues to their meningothelial differentiation or develop in a plausible setting that confirms their meningothelial origin. Malignant mesenchymal neoplasms without obvious evidence of meningothelial differentiation or origin are more likely to be true primary or metastatic sarcomas. Because of their clinical and biological differences, it is important to distinguish anaplastic meningioma from a sarcoma. We present a 67-year-old woman with multiple meningiomas, who developed a high-grade spindle cell tumor 6 months after the resection of a World Health Organization grade I meningioma. It was not clear whether this tumor represented a malignant transformation of meningioma or a primary sarcoma. Malignant transformation of a meningioma is exceptional within this short period and a coexisting sarcoma and meningioma are equally uncommon. Even though these malignant neoplasms are rare in general, they appear to be more prevalent in patients with multiple meningiomas including those with neurofibromatosis type 2.

Implementing the Sphere Project’s standards for patient’s healthcare rights in the disaster zone: The experience of the Israeli field hospital in post-quake Nepal

2021 ◽  
Vol 16 (1) ◽  
pp. 59-66
Author(s):  
Deganit Kobliner-Friedman, RN, MPH ◽  
Ofer Merin, MD ◽  
Eran Mashiach, MD ◽  
Reuven Kedar, MD ◽  
Shai Schul, MHA ◽  
...  
Keyword(s):  
Disaster Response ◽  
Sexual Exploitation ◽  
World Health ◽  
Field Hospital ◽  
Medical Teams ◽  
Health Organization ◽  
The Individual ◽  
The Right ◽  
Israel Defense Forces ◽  
Privacy And Confidentiality

Emergency medical teams (EMTs) encounter chaos upon arriving at the scene of a disaster. Rescue efforts are utilitarian and focus on providing the technical aspects of medical care in order to save the most lives at the expense of the individual. This often neglects the basic healthcare rights of the patient. The Sphere Project was initiated to develop universal humanitarian standards for disaster response.The increase in the number of EMTs led the World Health Organization (WHO) to organize standards for disaster response. In 2016, the WHO certified the Israel Defense Forces Field Hospital (IDF-FH) as the first to be awarded the highest level of accreditation (EMT-3). This paper presents the IDF-FH’s efforts to protect the patient’s healthcare rights in a disaster zone based on the Sphere Principles.These core Sphere Principles include the right to professional medical treatment; the right to dignity, privacy, and confidentiality; the right for information in an understandable language; the right to informed consent; the obligation to maintain private medical records; the obligation to adhere to universal ethical standards, to respect culture and custom and to care for vulnerable populations; the right to protection from sexual exploitation and violence; and the right to continued treatment.

scholarly journals Lebanon Cities’ Public Spaces

2018 ◽  
Vol 3 (1) ◽  
pp. 119-152 ◽  
Author(s):  
Maryam Nazzal ◽  
Samer Chinder
Keyword(s):  
Green Space ◽  
Public Spaces ◽  
World Health ◽  
Right To The City ◽  
Social Connections ◽  
Land Prices ◽  
The Social ◽  
Health Organization ◽  
The Right ◽  
The City

In Lebanon, the social connections are undeniable and crucial. However, meeting places remain private such as houses, restaurants, malls, and beach resorts. This is mainly due to the shortage of public spaces in Lebanon resulting from lack of planning, regulations and awareness around the right to the city and the importance of public spaces. In main cities where land prices are so expensive, common practice has prioritized the use of land in real estate development, thus trumping other uses such as public and communal spaces.In the late 1990s, Lebanon saw the emergence of malls, which have arguably acted as alternatives to public spaces. Malls, with their wealth of food courts, restaurants, cinemas, and play areas, have become the new downtown for a portion of the Lebanese population. They are also considered safe, which is another important factor.In 2015, the percentage of green spaces in Lebanon has decreased to less than 13%. While the World Health Organization (WHO) recommends a minimum of 9m2 of green space per capita (UN-HABITAT, 2016), Beirut has only 0.8m2.

scholarly journals THE HUMAN RIGHT TO HEALTH: A RETROSPECTIVE ANALYSIS AFTER 70 YEARS OF INTERNATIONAL RECOGNITION

2018 ◽  
pp. 24-42
Author(s):  
MARÍA DALLI
Keyword(s):  
Human Rights ◽  
Right To Health ◽  
World Health ◽  
Health Coverage ◽  
Human Right ◽  
The Sustainable Development ◽  
Cultural Rights ◽  
Health Organization ◽  
The Individual ◽  
The Right

In 1948, the General Assembly of the United Nations adopted the first international text recognising universal human rights for all; the Universal Declaration of Human Rights. Article 25 recognises the right to an adequate standard of living, which includes the right to health and medical care. On the occasion of the 70th anniversary of the Declaration, this article presents an overview of the main developments that have been made towards understanding the content and implications of the right to health, as well as an analysis of some specific advancements that aim to facilitate the enforcement thereof. These include: a) the implication of private entities as responsible for right to health obligations; b) the Universal Health Coverage goal, proposed by the World Health Organization and included as one of the Sustainable Development Goals; and c) the individual complaints mechanism introduced by the Optional Protocol to the International Covenant on Economic, Social and Cultural Rights (adopted on the 10th December 2008, 60 years after the UDHR).

scholarly journals Osteosarcoma of the Mandible in a Patient with Florid Cemento-Osseous Dysplasia and Li–Fraumeni Syndrome: A Rare Coincidence

2020 ◽  
Author(s):  
Simon Haefliger ◽  
Dorothee Harder ◽  
Michal Kovac ◽  
Karin Linkeschova ◽  
Harald Eufinger ◽  
...  
Keyword(s):  
Root Resorption ◽  
Bilateral Breast Cancer ◽  
Li Fraumeni Syndrome ◽  
Osseous Dysplasia ◽  
Left Posterior ◽  
Bone Biopsies ◽  
Li Fraumeni ◽  
Causative Relation ◽  
High Grade Osteosarcoma ◽  
The Right

Abstract Cemento-osseous dysplasia (COD) is the most common benign fibro-osseous lesion of the jaws and generally considered non-neoplastic and self-limited. Here, we present a 30-year old female who noticed a bilateral swelling of her posterior mandible with irregular periapical mineralization and incomplete root resorption on panoramic radiographs. A biopsy revealed florid COD and no further treatment was initiated. 9 years later, she presented with a progressive expansion of her left posterior mandible after being treated for bilateral breast cancer 4 and 8 years before. CT scans showed expansile and densely mineralized lesions in all four quadrants with the left posterior mandible showing a focal penetration of the buccal cortical bone. Biopsies revealed an osteoblastic high-grade osteosarcoma in the left and a COD in the right mandible, notably with cellular atypia in the spindle cell component. The patient underwent segmental resection of the left mandible with clear margins and adjuvant chemotherapy. Subsequent genetic testing identified a heterozygous germline TP53 mutation (p.V173G) which confirmed the clinically suspected Li–Fraumeni syndrome (LFS). 3 years after the resection, the patient is free of disease and the other foci of COD remained stable in size on follow-up imaging analyses. Our case illustrates LFS-related osteosarcoma developing within florid COD. Given the rarity of this coincidence, a causative relation between the two lesions seems unlikely but in patients with tumor predisposition syndromes it might be advisable to closely monitor even benign lesions like COD.

Right diagnosis, right treatment: SAFER PRACTICES

2019 ◽  
Vol 1 (7) ◽  
pp. 356-360 ◽  
Author(s):  
Paul Silverston
Keyword(s):  
Clinical Practice ◽  
Health Professionals ◽  
Diagnostic Errors ◽  
Integrated Approach ◽  
Diagnostic Error ◽  
World Health ◽  
Formal Training ◽  
The World ◽  
Health Organization ◽  
The Right

Choosing the right treatment for the patient requires that the right diagnosis is made first. In primary and ambulatory care, however, diagnostic errors are both common and commonly preventable. The World Health Organization has recommended that all health professionals should receive formal training in the principles of diagnostic reasoning and the causes of diagnostic error, and that strategies and interventions to reduce the risk of diagnostic error should be used in clinical practice. This article describes a mnemonic checklist, SAFER PRACTICES, which can be used in an integrated approach to the prevention and detection of diagnostic errors that starts in the classroom and continues through to the consulting room.

scholarly journals Everything counts in sending the right message: science-based messaging implications from the 2020 WHO guidelines on physical activity and sedentary behaviour

2020 ◽  
Vol 17 (1) ◽  
Author(s):  
Michelle L. Segar ◽  
Marta M. Marques ◽  
Antonio L. Palmeira ◽  
Anthony D. Okely
Keyword(s):  
Physical Activity ◽  
Sedentary Behaviour ◽  
Psychological Needs ◽  
World Health ◽  
Behavioural Science ◽  
Aerobic Activity ◽  
New Guidelines ◽  
Health Organization ◽  
The Right ◽  
Country Specific

Abstract The World Health Organization (WHO) released the 2020 global guidelines on physical activity and sedentary behaviour. The new guidelines contain a significant change from the 2010 guidelines on physical activity for adults and older adults that has important implications for next-generation physical activity messaging: The removal of the need for aerobic activity to occur in bouts of at least 10 min duration. This change in the guidelines provides an opportunity to communicate in new ways that align with behavioural science, permitting physical activity communicators and promoters to better support people’s psychological needs, motivation, and ability to fit healthy levels of physical activity into their lives. The frames and messages we use to communicate about the guidelines matter because they influence whether activity is perceived as relevant, meaningful, and feasible – or not. When developing new physical activity communications there are some overarching principles, based on behavioural science, to keep in mind. Using established theory, this commentary aims to support the creation of more strategic frames and messages for increasing the value and integration of physical activity into daily living. Country-specific physical activity campaigns using these ideas will be discussed.

scholarly journals Rosette-forming Glioneuronal Tumors in the Posterior Third Ventricle

2013 ◽  
Vol 40 (6) ◽  
pp. 885-888 ◽  
Author(s):  
Ibrahim Alnaami ◽  
Keith Aronyk ◽  
Jian-Qiang Lu ◽  
Edward S. Johnson ◽  
Cian O'Kelly
Keyword(s):  
Third Ventricle ◽  
Case Series ◽  
Neurofibromatosis Type ◽  
Pineal Region ◽  
World Health ◽  
Glioneuronal Tumor ◽  
First Case ◽  
The Central Nervous System ◽  
Health Organization ◽  
Glioneuronal Tumors

Rosette-forming glioneuronal tumor (RGNT) is a rare brain tumor found almost exclusively within the fourth ventricle. These grade I tumors were first included in the World Health Organization (WHO) Classification for the central nervous system in 20071. Since then, approximately 49 cases have been published.There are no reported cases of RGNT within the third ventricle, although this tumor has also been described in the pineal region, the tectum and within the aqueduct. Supratentorial localization is quite rare, with only three published: one was found in the septum pellucidum, another within the optic nerve in neurofibromatosis type 1 (NF1) patient and one in a patient with multiple RGNT in the lateral ventricle. The authors report the first case series in the literature of RGNT occurring within the posterior third ventricle.

scholarly journals PEComa in a Young Patient with Known Li-Fraumeni Syndrome

2015 ◽  
Vol 2015 ◽  
pp. 1-4 ◽  
Author(s):  
Kyriakos Neofytou ◽  
Simone Famularo ◽  
Aamir Z. Khan
Keyword(s):  
Risk Stratification ◽  
Tumour Suppressor Gene ◽  
Malignant Potential ◽  
Renal Lesion ◽  
Epithelioid Angiomyolipoma ◽  
Li Fraumeni Syndrome ◽  
Cancer Predisposition Syndrome ◽  
Rare Tumours ◽  
Li Fraumeni ◽  
The Right

Perivascular epithelioid cells neoplasms (PEComas) constitute a family of rare tumours which have been reported virtually in all anatomic sites. The histological clarification of the malignant potential of these tumours is still problematic despite the proposed risk stratification systems. Li-Fraumeni syndrome (LFS) is caused by a germline mutation in the TP53 tumour suppressor gene. It is a rare but well-characterized cancer predisposition syndrome leading to the development of a variety of different tumour types. To the best of our knowledge, an association between this syndrome and PEComas has not been previously documented. A 24-year-old lady with known LFS presented with two uncertain-in-nature lesions, one within the right part of the liver and one within the upper pole of the right kidney. The patient underwent an uncomplicated open simultaneous right partial nephrectomy and resection of segment 7 of the liver. The morphological and immunohistochemical features of both lesions were of epithelioid angiomyolipoma (PEComa). Although the obvious scenario was that the liver lesion was a metastasis from the renal lesion, the assessment of their malignant potential according to the existing risk stratification systems was rather in favour of two synchronous primary PEComas, pointing out that the histological assessment of malignant potential of PEComas is still problematic.

scholarly journals Lipomatous tumours in adrenal gland: WHO updates and clinical implications

2017 ◽  
Vol 24 (3) ◽  
pp. R65-R79 ◽  
Author(s):  
Alfred King-yin Lam
Keyword(s):  
Adrenal Gland ◽  
Age Distribution ◽  
World Health ◽  
Adrenal Myelolipoma ◽  
Adrenal Tumours ◽  
Endocrine Organs ◽  
Lipomatous Tumour ◽  
Health Organization ◽  
The Right

Adrenal lipomatous tumour is a group of adrenal tumours with a significant component of adipose tissue. According to the current World Health Organization (WHO) classification of tumours of endocrine organs, adrenal myelolipoma is the only entity amongst the group of tumours being described. In the literature, other more recently documented adrenal lipomatous tumours included 24 lipomas, 32 teratomas and 16 angiomyolipomas. Rare fatty tumours of the adrenal gland comprised liposarcoma, hibernoma, adrenocortical tumours with fat component and rare adrenal tumours with fat component. Myelolipoma comprises approximately 3% of primary adrenal tumour. It is noted more commonly in females and in the right adrenal gland. Approximately 40 bilateral myelolipomas were reported. The tumour is most frequently recorded in patients between fifth and seventh decades of life. Adrenal lipomas are often seen in males and in the right adrenal gland. They were commonly noted in patients in the sixth decade of life. The diagnosis could only be possible on examination of the surgically removed specimen. Adrenal teratomas were more common in females and with a bimodal age distribution. Slightly over 60% of the patients with adrenal teratoma are symptomatic. Adrenal angiomyolipomas were often symptomatic, more common in females and in the fifth decades of life. To conclude, adrenal lipomatous tumour is uncommon. They are often benign and non-functional. It is important to recognize the features of this group of lipomatous tumours in the adrenal gland as they are being detected on increasing incidence as a result of the wide-spread use of modern imaging modalities.

Sign in / Sign up

Export Citation Format

Share Document