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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.
The prognosis for lymphangioma circumscriptum and cavernous lymphangioma is generally excellent. This condition is associated with minor bleeding, recurrent cellulitis , and lymph fluid leakage. Two cases of lymphangiosarcoma arising from lymphangioma circumscriptum have been reported; however, in both of the patients, the preexisting lesion ...
It progresses rapidly through involvement of multiple body systems and mortality occurs in less than a year from the initial diagnosis. The average age of diagnosis being 55 years. The causative mechanism is unknown. In a few cases treatment with palliative chemotherapy has been effective. [1]: 598
In addition to liver and lungs, bones and skin have been the most frequent organs. Before the initial description of Weiss, the tumor had been reported under a variety of other names, including histiocytoid hemangioendothelioma , intravascular bronchoalveolar tumor (in the lung), and sclerosing cholangiocarcinoma .
Approximately 5% of adults have liver hemangiomas in the United States, but most are asymptomatic. [30] Liver hemangiomas usually occur between the ages of 30 and 50 and more commonly in women. [4] Cases of infantile liver cavernomas are extremely rare. Cavernous hemangioma of the eye is more prevalent in women than men and between the ages of ...
[7] [11] [12] Some reports indicate a good prognosis [13] but metastasis is occasionally seen. Infantile hemangioendothelioma is a rare benign vascular tumour arising from mesenchymal tissue and is usually located in the liver. It often presents in infancy with cardiac failure because of extensive arteriovenous shunting within the lesion.
Blue rubber bleb nevus syndrome is difficult to diagnose because of how rare the disease is. Diagnosis is usually based on the presence of cutaneous lesions with or without gastrointestinal bleeding and/or involvement of other organs. [6] Cutaneous angiomas are found on the surface of the skin and from the scalp to the soles of feet. [6]