angioendotheliomatosis

an·gi·o·en·do·the·li·o·ma·to·sis

(an'jē-ō-en'dō-thē'lē-ō-mă-tō'sis),
Proliferation of endothelial cells within blood vessels.

angioendotheliomatosis

/an·gio·en·do·the·lio·ma·to·sis/ (-en″do-the″le-o-mah-to´sis) intravascular proliferation of tumors derived from endothelial cells.

intravascular large B-cell lymphoma

A high-grade non-Hodgkin lymphoma arising in and generally confined to vascular lumina, which usually begins in the skin and CNS and rapidly spreads elsewhere.

Clinical findings
Often begins with fever of unknown origin and nonspecific cutaneous (e.g., plaques) and neurologic complaints.
 
DiffDx
Skin lesions (erythematous/purple plaques on the trunk and lower legs) may be confused with mycosis fungoides, sarcoidosis, vascular neoplasms (e.g., Kaposi sarcoma) or involvement by lymphoma or leukaemia.

Prognosis
Extremely poor; short survival is the norm despite aggressive chemotherapy. The diagnosis is often established postmortem.

an·gi·o·en·do·the·li·o·ma·to·sis

(an'jē-ō-en-dō-thē'lē-ō-mă-tō'sis)
Proliferation of endothelial cells within blood vessels.

angioendotheliomatosis

a state marked by the development of multiple endotheliomas; the clinical syndrome which results varies with the location of the major lesions.
References in periodicals archive ?
Immunohistochemical and cytogenetic studies indicate that malignant angioendotheliomatosis is a primary intravascular (angiotropic) lymphoma.
Malignant proliferative angioendotheliomatosis or angiotropic lymphoma associated with a soft-tissue lymphoma.
Fulling K, Gersell D: Neoplastic angioendotheliomatosis.
Angioendotheliomatosis proliferans systemisata [Angioendotheliomatosis proliferans systematisata: a clinically and pathohistologically new disease picture].
Proliferating systematized angioendotheliomatosis in a 5-month-old infant.
Benign reactive proliferating angioendotheliomatosis [in Italian].
Many terms have been used in the past to describe IVL, including angioendotheliomatosis proliferans systematica and angiotropic large cell lymphoma.
We favor the designation macroglossal angiodysplasia over macroglossal lymphangioendotheliomatosis, because the latter is liable to be confused with the entity of angioendotheliomatosis, which is a malignant vasoformative neoplastic process of the central nervous system.
For instance, the studies of Ansell et al[14] and Wick et al[15] have demonstrated that malignant angioendotheliomatosis is a lymphoma and not a vascular tumor.