A Clearer Picture: Using Fetal MRI to Diagnose Neck Masses and Predict Airway Compromise. [PDF]
ABSTRACT Introduction Fetal neck masses are rare but can be life‐threatening if causing airway compromise. Early and accurate diagnosis of these masses allows life‐saving interventions to be undertaken at birth in the form of the EXIT procedure. Methods A single institution case series of all patients referred for fetal MRI to a tertiary center in the ...
Wilson L, Whitby EH, Raghavan A.
europepmc +2 more sources
Kaposiform Lymphangiomatosis as a Cause of Vaginal Bleeding & Discharge: A Case Report. [PDF]
Prepubertal vaginal bleeding is a common presentation for pediatric adolescent gynecologists with a broad differential diagnosis that historically may not have included complex lymphatic anomalies. However, given recent consensus criteria and imaging capabilities, this may be a condition that pediatric adolescent gynecologists see more frequently in ...
Das K +7 more
europepmc +3 more sources
Signaling pathways and inhibitors of cells from patients with kaposiform lymphangiomatosis. [PDF]
AbstractBackgroundKaposiform lymphangiomatosis (KLA) is a rare lymphatic anomaly with significant morbidity and mortality. KLA is characterized by diffuse multifocal lesions comprised of focal areas of “kaposiform” spindled cells accompanying malformed lymphatic channels.
Boscolo E +8 more
europepmc +4 more sources
A Rare Case of Neonatal Kaposiform Hemangioendothelioma With Kasabach-Merritt Phenomenon Presenting as an Abdominal Mass Without Cutaneous Manifestations. [PDF]
Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm that typically presents in infancy or early childhood, classified as a locally aggressive/borderline vascular tumor. It most commonly involves superficial and deep soft tissues, often demonstrating infiltrative growth into adjacent muscle and bone, whereas retroperitoneal or ...
Park SY +5 more
europepmc +2 more sources
Pregnancy Complicated by 3-Hydroxy-3-Methylglutaryl-CoA Lyase Deficiency and Kaposiform Lymphangiomatosis. [PDF]
BACKGROUND: To describe pregnancy complicated by deficiency of 3-hydroxy-3-methylglutaryl–CoA (HMG-CoA) lyase, an inborn error of metabolism, and kaposiform lymphangiomatosis (KLA), a lymphatic anomaly with poor prognosis.
Yang C, Saad A, Luo G.
europepmc +3 more sources
Background Kaposiform Hemangioendothelioma (KHE) is a rare vascular tumor of intermediate malignant potential which shows locally aggressive growth but only rarely metastasizes.
Qurratulain Chundriger +4 more
doaj +1 more source
Colonic Lymphangiomatosis [PDF]
Lymphangiomas are benign malformations of the lymphatic vessels which can be primary or secondary in etiology. Colonic involvement is rare, and the diagnosis is mostly incidental. Sometimes, the initial endoscopic appearance can be misleading. We present
David P Lebel +5 more
core +1 more source
ABSTRACT The objective was to prepare guidelines to perform the current optimum treatment by organizing effective and efficient treatments of hemangiomas and vascular malformations, confirming the safety, and systematizing treatment, employing evidence‐based medicine techniques and aimed at improvement of the outcomes.
Yoshiaki Kinoshita +116 more
wiley +2 more sources
mTOR Inhibitors in the complex treatment of high-risk vascular malformations in children
Recent experimental and clinical studies demonstrated that mTOR receptor inhibitors can stop the progression of vascular malformations and significantly improve patients’ quality of life.
I.M. Benzar, D.V. Zhumik
doaj +1 more source
Expansion of the Phenotype of Lymphatic Anomalies Caused by Somatic Activating BRAF Variant. [PDF]
ABSTRACT Background The somatic activating variant in BRAF (p.V600E) was recently described as a novel cause of macrocystic head and neck lymphatic malformations in three individuals. Other recent studies profiling the genetic causes of more complex lymphatic anomalies identified this same pathogenic BRAF variant.
Fox MD +11 more
europepmc +2 more sources

