We have located links that may give you full text access.
Locally Invasive Papillary Intralymphatic Angioendothelioma Arising Within a Lymphatic/Venous Malformation.
American Journal of Case Reports 2023 October 8
BACKGROUND Papillary intralymphatic angioendothelioma (PILA) is a rare vascular tumor affecting children and young adults, with less than 50 cases reported in the literature. This tumor typically presents in the extremities, exhibits borderline behavior, and has a prominent lymphatic phenotype. Originally thought to be malignant, PILA was later recognized for its borderline behavior and lymphatic features, leading to its current classification as a "rarely metastasizing lymphatic vascular neoplasm". CASE REPORT We present the case of a 10-year-old girl with a 6-year history of a right facial venous malformation, which was ultimately diagnosed as PILA in the background of lymphatic/venous malformation (LVM). After undergoing surgical excision of a right facial soft-tissue tumor, histopathological examination revealed scattered lymphatics and thin-walled vascular channels with blood in skeletal muscle and fibroadipose tissue. Intraluminal papillary proliferation of vascular spaces lined by cytologically bland spindle cells was observed, along with Kaposiform morphology and small-vessel proliferation. Immunohistochemical staining confirmed endothelial cell markers (D2-40, ERG, CD34, and CD31) and numerous CD3(+) lymphocytes in the lumen, surrounded by CD3(+) T lymphocytes and CD20(+) B lymphocytes in the surrounding stroma. The tumor lacked pleomorphism, significant mitotic activity, and necrosis. CONCLUSIONS PILA presents a diagnostic challenge and should be considered in the differential diagnosis of cutaneous vascular neoplasms. Long-term follow-up is crucial due to its borderline behavior and potential for local invasiveness and metastasis. Accurate diagnosis, aided by characteristic histological and immunohistochemical features, is essential for appropriate management of this rare vascular tumor.
Full text links
Related Resources
Trending Papers
Mineralocorticoid receptor antagonists and reno-protection: What's the evidence & where do they fit? A guide for non-specialists.Diabetes, Obesity & Metabolism 2024 May 8
Angiotensin Receptor Blocker-Neprilysin Inhibitor for Heart Failure with Reduced Ejection Fraction.Pharmacological Research : the Official Journal of the Italian Pharmacological Society 2024 May 12
The Therapy and Management of Heart Failure with Preserved Ejection Fraction: New Insights on Treatment.Cardiac Failure Review 2024
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app
All material on this website is protected by copyright, Copyright © 1994-2024 by WebMD LLC.
This website also contains material copyrighted by 3rd parties.
By using this service, you agree to our terms of use and privacy policy.
Your Privacy Choices
You can now claim free CME credits for this literature searchClaim now
Get seemless 1-tap access through your institution/university
For the best experience, use the Read mobile app