Denise Adams

26 statements · 2 topics

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The ISSVA (International Society for the Study of Vascular Anomalies) classification system, updated in 2014, divides lymphatic anomalies into lymphatic tumors and lymphatic malformations, with malformations further categorized as macrocystic, microcystic, combined, generalized lymphatic anomaly, Gorham-Stout disease, or central conducting anomalies.
Lymphatic anomalies are classified into lymphatic tumors and lymphatic malformations, with malformations further divided into macrocystic, microcystic, combined lesions, generalized lymphatic anomaly, Gorham-Stout disease, and central conducting anomalies
When bleomycin was first used for vascular anomalies, there were reported cases of pneumonitis because practitioners didn't understand there is a maximum safe dose
Sirolimus (rapamycin) is an mTOR inhibitor that blocks the PIK3CA pathway, which has been shown to be a somatic mutation in many lymphatic malformations

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Denise's statements about Lymphatic Malformation 17 statements

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Lymphatic Anomalies

Statement The ISSVA (International Society for the Study of Vascular Anomalies) classification system, updated in 2014, divides lymphatic anomalies into lymphatic tumors and lymphatic malformations, with malformations further categorized as macrocystic, microcystic, combined, generalized lymphatic anomaly, Gorham-Stout disease, or central conducting anomalies. Open
Quotation I think what Belinda said cannot be stressed enough. I think that we are very lucky here that we have an interdisciplinary team. So it's not one person making the diagnosis and making the plan. It's a group of us working together. Open
Statement Sirolimus (rapamycin) and everolimus are mTOR inhibitors that block the PIK3CA pathway, which is implicated in somatic mutations found in many lymphatic malformations. Open
Statement Preoperative mTOR inhibitor therapy can soften lymphatic tissue, decrease lesion size, and make surgical debulking less extensive. Open
Statement A 2008 FDA-funded study at Boston Children's Hospital demonstrated that sirolimus improved quality of life, reduced pain, and decreased infection frequency in patients with complicated vascular anomalies with a lymphatic component. Open
Statement Sclerotherapy agents are chosen based on lesion type: doxycycline for macrocysts, bleomycin for microcysts (with monitoring for side effects), and sodium tetradecyl sulfate (STS) for venous components. Open
Statement Bleomycin is a cancer medicine that can cause pneumonitis if maximum dose limits are not observed during sclerotherapy. Open
Quotation I think you need to have some sort of understanding of what sclerosis you use for what type of malformation, or you're not going to have that safety element. Open

Lymphatic Anomalies

Statement The ISSVA classification system was updated in 2014 and is based on John Mulligan's original classification from Boston Children's Hospital Open
Statement Lymphatic anomalies are classified into lymphatic tumors and lymphatic malformations, with malformations further divided into macrocystic, microcystic, combined lesions, generalized lymphatic anomaly, Gorham-Stout disease, and central conducting anomalies Open
Quotation I really felt that vascular anomalies is an umbrella term, and even if you take lymphatic anomalies, there are many different kinds of lymphatic anomalies that maybe need different medicines. Open
Statement Sirolimus (rapamycin) is an mTOR inhibitor that blocks the PIK3CA pathway, which has been shown to be a somatic mutation in many lymphatic malformations Open
Statement Boston Children's Hospital received FDA funding in 2008 to study sirolimus for complicated vascular anomalies based on the hypothesis that the PIK3CA pathway controls blood vessel growth Open
Statement Sirolimus improved quality of life, decreased pain, and reduced infection frequency in patients with complicated lymphatic anomalies Open
Quotation I feel very passionate that it needs to be used in a controlled manner where we actually are following outcomes. Open
Statement Preoperative sirolimus therapy can soften lymphatic tissue and decrease lesion size, making surgical procedures less extensive Open
Statement When bleomycin was first used for vascular anomalies, there were reported cases of pneumonitis because practitioners didn't understand there is a maximum safe dose Open
Denise's statements about Sarcoma (Ewing/Rhabdo) 9 statements

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Lymphatic Anomalies

Statement The ISSVA classification system was updated in 2014 and is based on John Mulligan's original classification from Boston Children's Hospital Open
Statement Lymphatic anomalies are classified into lymphatic tumors and lymphatic malformations, with malformations further divided into macrocystic, microcystic, combined lesions, generalized lymphatic anomaly, Gorham-Stout disease, and central conducting anomalies Open
Quotation I really felt that vascular anomalies is an umbrella term, and even if you take lymphatic anomalies, there are many different kinds of lymphatic anomalies that maybe need different medicines. Open
Statement Sirolimus (rapamycin) is an mTOR inhibitor that blocks the PIK3CA pathway, which has been shown to be a somatic mutation in many lymphatic malformations Open
Statement Boston Children's Hospital received FDA funding in 2008 to study sirolimus for complicated vascular anomalies based on the hypothesis that the PIK3CA pathway controls blood vessel growth Open
Statement Sirolimus improved quality of life, decreased pain, and reduced infection frequency in patients with complicated lymphatic anomalies Open
Quotation I feel very passionate that it needs to be used in a controlled manner where we actually are following outcomes. Open
Statement Preoperative sirolimus therapy can soften lymphatic tissue and decrease lesion size, making surgical procedures less extensive Open
Statement When bleomycin was first used for vascular anomalies, there were reported cases of pneumonitis because practitioners didn't understand there is a maximum safe dose Open