Proliferating Systematized Angioendotheliomatosis

Systemic endotheliomatosis: a case report.

“…The cutaneous manifestations in our patient parallel those reported by Braverman andLerner (1961), Haber, Harris-Jones, andWells (1964), Fievez, Fievez, and Hustin (1971), and Pfleger and Tappeiner (1959). These patients all had 'Slides reviewed by R. Rachman, MD, Einstein Medical Center, Philadelphia, Pa, and 1.…”

“…The patient was subsequently lost to follow-up. Both Pfleger (1963) andFievez et al (1971) have implicated the young age of the original patient (first involvement at age 24) as being the beneficial factor in the comparatively benign clinical course. This concept of age as a prognostic factor must await additional support.…”

Systemic angioendotheliomatosis with metastases.

“…(M), skull bones (M), brain (V), spleen (M) Shtern and Likhachev (1963) Brain Haber et al (1964) Skin,? abdomen (clinical signs only) Midana and Ormea (1965) Skin Strouth et al (1965) Brain (V), adrenal (V) Abulafia et al (1969) Skin (V arteries and veins) Fievez et al (1971) Skin; capillaries in most of body Okagaki and Richart (1971) Genital tract (V) Madara et al (1975) Skin; capillaries of adbominal organs Scott et al (1975) Skin puzzling feature of the present case is the failure to find the tumour cells in the peripheral blood. A series of buffy coat films collected between the time of amputation and death were examined without success.…”

Neoplastic angioendotheliosis: a case of subacute dementia with unusual cerebral CT appearances and a review of the literature.

“…12 Complete remission has been described in some cases with predominantly or solely dermatological features, but the treatment has usually included chemotherapy with agents such as vincristine, methotrexate and cyclophosphamide.4 14 Given that there is probably a lymphomatous basis to at least some cases and that the disease process is basically an intravascular one, chemotherapy should be considered in future cases with neurological features. The lymphomatous basis of this case was not apparent before detailed histopathological study was undertaken and lymphoma might prove to be a commoner cause of neoplastic angioendotheliosis than suspected at present.…”

“…"2 Pasyk and Depowski reported a case in a 5 month old infant and suggested that the condition might have two forms: a relatively benign inflammatory or vasculitic form and a more serious truly malignant variety. 13 From a patho-aetiological point of view, Many reports have been concerned with dermatological manifestations4 10 12 14 15 but neurological manifestations are common, particularly a subacute progressive dementia. 8 16 The purpose of this communication is to report a further case, and to describe cerebral CT appearances not previously reported in this condition.…”