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.
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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.
Angiotropic large cell lymphoma (ALCL) of the superior vena cava (SVC)
Reactive Angioendotheliomatosis and Intravascular Histiocytopathy.
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].
Selected pseudoneoplastic lesions of the skin
Many terms have been used in the past to describe IVL, including angioendotheliomatosis proliferans systematica and angiotropic large cell lymphoma.
Renal Cell Carcinoma With Intravascular Lymphomatosis
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.
Molecular Characterization of Colorectal Neoplasia in Translational Research
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.
Congenital Macroglossal Angiodysplasia ("Lymphangioendotheliomatosis")

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