The Liddy Shriver Sarcoma Initiative

What We've Learned as Leaders of the Liddy Shriver Sarcoma Initiative

Wed, 5 Nov 2014 06:24:18 -0500

What We've Learned as Leaders of the Liddy Shriver Sarcoma Initiative
by Bruce Shriver, PhD and Beverly Shriver, RN

We formed the Liddy Shriver Sarcoma Initiative in November 2003, two months before our daughter Liddy passed away. In the eleven years since then, we have traveled with many hundreds of sarcoma families down the uncertain path leading from diagnosis through treatments and beyond. We have shared the joy of those who now experience no evidence of disease and the grief and sorrow of those who have lost a loved one to sarcoma.

We have learned much in our personal journey with sarcoma, in the sharing of the journeys of others, through the publishing of ESUN, and in the funding of sarcoma research. As this is the last issue of ESUN which we will publish (see "Preparing for a Transition"), we want to share with you some of what we've learned as well as some of our conjectures and suggestions.

Read more at http://sarcomahelp.org/articles/learned.htm

A Glimpse Inside the Liddy Shriver Sarcoma Initiative

Wed, 5 Nov 2014 06:24:18 -0500

A Glimpse into the Liddy Shriver Sarcoma Initiative
by Mary Sorens

As the person responsible for the Initiative's website, I have had a unique window into the world of sarcoma advocacy and research. In the midst of working with Bruce and Bev to gradually end the Initiative, I'd like to share some of my incredible experiences with our donors and supporters.

There are a lot of people out there who have no faith in the "medical machine," in pharmaceutical companies, toxic therapies, and even in physicians. While I deal with my own doubts about the system...

Read more at http://sarcomahelp.org/articles/glimpse.html

Palliative Surgery for Sarcoma

Wed, 5 Nov 2014 06:24:18 -0500

Palliative Surgery for Sarcoma
by J. Harrison Howard, MD and Raphael Pollock, MD, PhD

As surgeons we pride ourselves on being able to offer sarcoma patients the opportunity for cure. Radiation and chemotherapy may make our scalpels more effective, but surgery remains the cornerstone of sarcoma therapy. Experience with the disease quickly humbles our pride as we have patients that recur despite multidisciplinary care and our personal best efforts. After recurrence, perhaps we have a second opportunity to operate for cure. We might even have a third or fourth chance, but for many sarcoma patients there will come a time when the scalpel can no longer offer the opportunity for cure; however, can it still help?

Read more at http://sarcomahelp.org/articles/palliative-surgery.html

Are We Winning the War on GIST?

Wed, 5 Nov 2014 06:24:18 -0500

Are We Winning the War on GIST?
by Ping Chi, MD, PhD

The "War on Cancer" was established in 1971 at a time when combinations of newly discovered chemotherapies were, for the first time, curing rare types of cancers, such as childhood leukemia and testicular cancer. Despite the fact that most chemotherapeutic agents act nonspecifically through killing rapidly dividing cells, the prevailing optimism reasoned that with time, we can define combinations to cure all types of cancers. Yet, there were tumour types that do not respond to any conventional systemic chemotherapeutic agent, as exemplified by gastrointestinal stromal tumor (GIST), one of the most common subtypes of soft tissue sarcomas. Patients suffering from GISTs used to be subjected to toxic and ineffective systemic chemotherapy. It became clear that the "war" cannot be won without detailed understanding of the biology of each tumour type through collaborative research between basic scientists and clinicians. In this context, GIST became a poster child.

Read more at http://sarcomahelp.org/GIST.html

A Model Research Grants Program for Rare Cancers

Wed, 5 Nov 2014 06:24:18 -0500

A Model Research Grants Program for Rare Cancers
by Bruce Shriver, PhD

The Research Grants Program of the Liddy Shriver Sarcoma Initiative is widely respected in the sarcoma community for its scope, international focus, quality peer-review process, accountability and openness. Since the publication of Preparing for a Transition in the July 2014 issue of ESUN, we have received numerous requests to explain this program in more detail so that other groups can consider adopting the principles and mechanisms we employ in funding rare cancer research.

Read more at http://sarcomahelp.org/sarcoma-research.html

$50,000 Grant Funds Osteosarcoma Research

Wed, 5 Nov 2014 06:24:18 -0500

$50,000 Grant Funds Research on Osteosarcoma

The Liddy Shriver Sarcoma Initiative has awarded a $50,000 grant for promising osteosarcoma research at Massachusetts General Hospital. In this study, Amity Lynn Manning, PhD aims to reveal vulnerabilities within osteosarcoma cells that can be exploited to render the disease less aggressive and more sensitive to traditional therapies.

Osteosarcoma is a rare cancer that usually affects children, adolescents and young adults. The disease is treated with a combination of aggressive therapies, but a significant number of patients deal with disease relapse and progression.

Osteosarcoma cells are known to display a high degree of chromosomal instability (CIN). The link between CIN and poor prognosis has been observed for some time, but the mechanisms that contribute to CIN are not well understood. Thus, the effect of suppressing CIN in osteosarcoma has yet to be tested.

Read more at http://sarcomahelp.org/research/osteosarcoma-CIN.html

Modeling Treatment Response of NF1-Deleted Sarcoma

Wed, 5 Nov 2014 06:24:18 -0500

Modeling Treatment Response of NF1-Deleted Sarcoma

Neurofibromin 1 (NF1) is a tumor suppressor that functions as a negative regulator of the Ras pathway. NF1 mutations are present in many types of sarcoma, yet its role in the disease and tumor response to treatment is an underexplored area of sarcoma research. The loss of NF1 is well-characterized in malignant peripheral nerve sheath tumors (MPNSTs), and NF1 mutations have been recently identified in a wide variety of pediatric and adult soft-tissue sarcomas including myxoidfibrosarcoma, liposarcoma, and rhabdomyosarcoma. In addition, patients with Neurofibromatosis Type 1 (NF1), caused by genetic inactivation of the NF1 gene, are at increased risk for developing soft-tissue sarcomas, including MPNST and myogenic sarcomas such as rhabdomyosarcoma. The identification of novel mutations in sarcomas with complex karyotypes provides new opportunities to model human sarcomas and to investigate new therapies for this difficult to treat disease.

We designed this project to bridge boundaries in sarcoma research by using a novel mouse model of NF1-deleted sarcoma to identify new therapies for sarcoma patients.

Read more at http://sarcomahelp.org/research/nf1.html

胞巣状軟部肉腫

Wed, 5 Nov 2014 06:24:18 -0500

胞巣状軟部肉腫

胞巣状軟部肉腫(ASPS)は、稀で、予後が不良な腫瘍です。特異的な組織学的・分子生物学的特徴を持ち、独特の臨床像を示しますが、発生起源については不明です。一般的に若年者に発生します。他の軟部肉腫と違い、脳に転移することもあります。遠隔転移を生じても手術を行うことで治療成績の改善が見込めます。その一方で、従来の化学療法や放射線治療によっては有意な生命予後の改善は期待できません。ここでは胞巣状軟部肉腫の臨床所見、診断、画像の特徴、治療の概要について述べます。

Read more at http://sarcomahelp.org/translate/ja-asps.html

骨肉腫

Wed, 5 Nov 2014 06:24:18 -0500

骨肉腫

骨肉腫は、類骨と呼ばれる未熟な骨の形成を特徴とする多様な悪性紡錘形細胞腫瘍の一群です。悪性の度合い、すなわちどれだけ遠隔転移をする（体のあちこちに広がる）可能性が高いかは、病理組織学的悪性度（顕微鏡でどのくらい悪くみえるか）によって決まります。言い換えれば、骨肉腫と呼ばれる腫瘍の中には、手術のみで治癒可能なものから、強力な治療にもかかわらず予後不良なものまで、様々なものが含まれます。転移のない（限局性の）病変であれば、さまざまな治療法を組み合わせることにより、治癒率は65-70%に上りますが、長く辛い治療はしばしば1年以上に及びます。生存率の改善に伴い、骨肉腫患者の写真の長期的なケアに関する新しい試みがさまざまな領域で始まっています。骨肉腫は治療設備や専門的なスタッフが整ったがん専門病院で治療されることが望まれます。以下、骨肉腫の様々な亜型にも適宜触れながら、骨肉腫の大多数を占める通常型の高悪性度骨肉腫に焦点をあてて記述します。以下の記載は骨肉腫に関するすべてを網羅しているものではありませんので、むしろ患者・医師間のコミュニケーションの一助になるような、現在の知見のまとめであるとご理解ください。

Read more at http://sarcomahelp.org/translate/ja-osteosarcoma.html