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Multifocal lymphangioendotheliomatosis, also known as congenital cutaneovisceral angiomatosis with thrombocytopenia and multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT), [1] is a skin condition that presents at birth with hundreds of red-brown plaques as large as several centimeters.
Lymphangiomatosis is a condition where a lymphangioma is not present in a single localised mass, but in a widespread or multifocal manner. It is a rare type of tumor which results from an abnormal development of the lymphatic system.
Multifocal lymphangioendotheliomatosis (congenital cutaneovisceral angiomatosis with thrombocytopenia, multifocal lymphangioendotheliomatosis with thrombocytopenia) Multinucleate cell angiohistocytoma; Multiple cutaneous and uterine leiomyomatosis syndrome (leiomyomatosis cutis et uteri, multiple leiomyomatosis, Reed's syndrome)
Multiple distinct red globules are seen under a microscope, and a finely pigmented network is seen around the periphery. [8] Histologically, microvenular hemangioma is made up of venules with thin walls that branch erratically and have barely noticeable vascular lumina.
Lymphatic malformations are benign slow-flow type of vascular malformation of the lymphatic system characterized by lymphatic vessels which do not connect to the normal lymphatic circulation.
Proliferating Angioendotheliomatosis has historically been divided into two groups, (1) a reactive, involuting type and (2) a malignant, rapidly fatal type. [1]: 598 ...
Multifocal multisystem LCH, also called Letterer-Siwe disease, is an often rapidly progressing disease in which Langerhans cells proliferate in many tissues. It is mostly seen in children under age 2, and the prognosis is poor: even with aggressive chemotherapy, the five-year survival is only 50%.
They have been described as masses that fall between a hemangioma and angiosarcoma.They are vascular tumors that commonly present with an enlarging mass and most commonly involve the lungs, liver, and musculoskeletal system, although many other body sites have been reported, including the head and neck, intestines, lymph nodes, pleura, retroperitoneum, heel, stomach.