Reactive and malignant “angioendotheliomatosis”: a discriminant clinicopathological study

Abstract

In order to determine whether or not phenotypic differences existed between reactive angioendotheliomatosis (RAE) and malignant angioendotheliomatosis (MAE), we studied the histological and immunohistochemical features of 4 and 8 cases of these lesions, respectively. Antibodies to leukocyte common antigen (LCA), specialized B- and T-lymphocytic determinants, Factor VIII-related antigen (FVIIIRAG), blood group isoantigens A, B, and H (BGI), epithelial antigens, vimentin, and actin; and Ulex europaeus I lectin (UEL) were utilized. Cutaneous lesions in all cases of MAE were part of a disseminated, fatal, intravascular cellular proliferation, with highly atypical cytological features. Because one of the patients in this group had cardiac valvular vegetations at autopsy, this case had been reported previously as representative of RAE. However, the latter example, as well as all others of MAE, stained strongly for LCA, B-cell antigens, and vimentin in tumor cells. FVIIIRAG was seen focally in 6 cases, in cells entrapped in platelet-fibrin thrombi; however, UEL binding and reactivity for BGI were uniformly absent. Conversely, RAE was typified by a cytologically-bland intravascular proliferation, with actin-positive, perivascular, pericytic cuffs. All 4 patients in this group had cutaneous involvement only, and the lesions tended to be self-resolving. One had pulmonary tuberculosis, but evidence for an underlying infection was absent in the remainder of RAE cases. Immunohistologically, RAE displayed universal reactivity for FVIII-RAG, BGI, UEL, and vimentin, and negativity for LCA in intravascular cells. Neither MAE nor RAE showed the presence of epithelial determinants. These data indicate that MAE and RAE are clinicopathologically distinct entities, showing lymphoid and endothelial features, respectively. Because of the phenotypic properties of the former condition, it would appear advisable to substitute the term "intravascular lymphomatosis" for "malignant angioendotheliomatosis".

Cutaneous Intravascular Lymphoma following Extravascular Lymphoma of the Lung

“…Neoplastic cells may also be found outside the vasculature [3, 18]. Intraluminal fibrin deposition and/or thrombi may be seen which, along with neoplastic cells, lead to occlusion of blood vessels producing infarcts of affected tissues.…”

“…Histopathologically, the differential diagnosis for IL includes other entities showing intravascular proliferation, especially reactive angioendotheliomatosis (RAE) [18]. RAE is a much rarer, self-limiting condition characterized by a proliferation of endothelial cells within vascular lumina.…”

Reaktive Angioendotheliomatose nach Implantation einer Schultergelenksprothese

“…Bei der malignen Angioendotheliomatose bestehen die Proliferate aus atypischen mononukleären Zellen mit zahlreich vorkom− menden Zell− und Kernatypien, die in den meisten der bisher publizierten Fälle als B−Lymphozyten klassifiziert werden konn− ten [6,21]. Neben diesen intravasalen oder angiotropen B−Zell− Lymphomen wurden nur sehr vereinzelt T−Zell−Lymphome oder histiozytische Lymphome diagnostiziert [11,22,23].…”

“…Positive Markierun− gen der intravasalen Proliferationen mit Endothelzellmarkern, z. B. mit UEA1, sowie der Nachweis muskelaktin−besetzter peri− vasaler Perizytenmanschetten belegen hingegen das Vorliegen einer reaktiven Angioendotheliomatose [21]. Die Ätiopathoge− nese der Angioendotheliomatosen ist weitgehend ungeklärt.…”

Intravascular Malignant Lymphomatosis: A Cause of Subacute Dementia

“…The nosologic term IML replaced angiotropic lymphoma tosis, because 'angiotropic' may be confused with 'angiotrophic' [1], as well as malignant angioendotheliosis (MAE), after it was demonstrated that the malignant cells are of lymphoid rather than endothelial origin (as initially Accepted: February 15. 1995 thought), since they stain positive for lymphoid cell mark ers (leukocyte common antigen) but not for endothelial cell factor VUI-related antigen [2,3], IML bears a poor outcome and should be differentiated from reactive angiocndotheliosis (RAE), which implies milder forms (gen erally limited to skin involvement) and a better prognosis [4], The diagnosis of IML is histological; clinical criteria are not defined, and with time, new clinical manifesta tions are reported. The first communications emphasized the maculopapular eruption of skin over the limbs and trunk; after a few years, systemic and neurological fea tures were also reported.…”