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.

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.
References in periodicals archive ?
Sherman, "Histiocytic lymphoma and malignant angioendotheliomatosis. One disease or two?," Cancer, vol.
Parkinson, "Reassessment of malignant "angioendotheliomatosis".
Umbert, "Malignant proliferative angioendotheliomatosis or angiotropic lymphoma associated with a soft-tissue lymphoma," Journal of the American Academy of Dermatology, vol.
Romani et al., "Intravascular lymphomatosis" (angioendotheliomatosis): Evidence for a T-cell origin in two cases," Human Pathology, vol.
Initially described by Pflegrand and Tappeiner in 1959 as "angioendotheliomatosis proliferans systemisata" and in 2008 defined by the World Health Organization (WHO) as intravascular large B cell lymphomas (IVBCL), IVL is a type of extra-nodal large B cell lymphoma where growth is restricted to the lumina of the vessels, particularly the capillaries [7].
Scheithauer, "Reassessment of malignant "angioendotheliomatosis".
Immunohistochemical and cytogenetic studies indicate that malignant angioendotheliomatosis is a primary intravascular (angiotropic) lymphoma.
Believing that the intravascular growth of malignant cells represented a neoplasm of the vascular endothelium, they described the disease as "angioendotheliomatosis proliferans systemisata." In 1986 Sheibani et al (2) proved, via immunohistochemical investigation, that the cells of intravascular lymphoma were of lymphoid origin and described the disease as angiotropic (intravascular) large-cell lymphoma.
Reactive Angioendotheliomatosis and Intravascular Histiocytopathy.--In the past, the term angioendotheliomatosis was used to describe a condition that has now been renamed intravascular lymphomatosis (IVL).
Many terms have been used in the past to describe IVL, including angioendotheliomatosis proliferans systematica and angiotropic large cell lymphoma.[1,2] However, most authors prefer the more descriptive term IVL.[3,4] The majority of IVL cases are of B-cell phenotype, as demonstrated by immunohistochemical,[3,4] Southern blot, and gene rearrangement studies.[5]
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.
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.[4]