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Retiform hemangioendothelioma. A new case in a child with diffuse
endovascular papillary endothelial proliferation.
Sanz-Trelles A, Rodrigo-Fernandez I, Ayala-Carbonero A, Contreras-Rubio
F.
Department of Pathology, Regional Hospital Carlos Haya, Malaga,
Spain.
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J Cutan Pathol 1997 Aug;24(7):440-4 Abstract quote
We present a new case of retiform hemangioendothelioma (RH), an entity
first described by Calonje et al. in 1994.
The tumor was intradermal and located on the toe of an 11-year-old
boy. Histologically, in addition to the distinctive retiform pattern
of proliferating vessels in RH there are intraluminal papillae with
hyaline cores similar to those seen in malignant endovascular papillary
angioendothelioma (Dabska's tumor), but usually they are infrequent,
focal and poorly developed. In our case, these papillary structures
were well formed and distributed in a diffuse way. They were most conspicuous
in superficial areas where the blood vessels were dilated. In deep areas,
where the pattern of neoplastic vessels was retiform, the papillae filled
their lumina totally, resembling solid cords.
Our case shares the clinical and morphologic features of both retiform
hemangioendothelioma and Dabska's tumor, supporting a relationship between
these two kinds of neoplasms. The benign behavior of this case, with
no recurrence or metastases over a 4-year follow-up, corresponds to
the low malignancy of this kind of vascular neoplasm.
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Multiple lesions of intravascular papillary endothelial hyperplasia
(Masson's lesions).
Stewart M, Smoller BR.
Department of Pathology, Stanford University Medical Center, CA
94305.
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Arch Pathol Lab Med 1994 Mar;118(3):315-6 Abstract quote
We recently encountered an elderly female patient with multiple, rapidly
growing vascular lesions on her forearm.
Histologic examination and immunoperoxidase studies were performed
to identify cells present within the lesions. The growths proved to
be intravascular papillary endothelial hyperplasia. Immunohistochemical
studies showed these lesions to be proliferations of factor VIII+, factor
XIIIa+, and CD34+ cells. Intravascular papillary endothelial hyperplasia
lesions are growths that histologically can mimic angiosarcoma, especially
when they are multiple. They appear to be proliferations of endothelial
cells and also contain dermal dendrocytes. This finding raises the question
as to the exact origin of the cells that give rise to these tumors.
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Reactive angioendotheliomatosis. Case report and review of the literature.
Lazova R, Slater C, Scott G.
Department of Pathology, University of Rochester School of Medicine
and Dentistry, NY 14642, USA.
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Am J Dermatopathol 1996 Feb;18(1):63-9 Abstract quote
Angioendotheliomatosis is an uncommon disease characterized histologically
by proliferation of cells within vascular lumina with secondary intravascular
thrombi resulting in obliteration of the involved vessels.
While angioendotheliomatosis was initially thought to be a single disease
entity, recent studies show that the disease may be divided into benign
(reactive) and malignant variants, with the malignant variant representing
intravascular malignant lymphoma. The reactive variant is rare, with
only 16 cases reported in the literature, and is characterized histologically
by proliferating endothelial cells within vessel lumina.
In this report we present a case of reactive angioendotheliomatosis
and review the literature on this rare entity.
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Papillary intralymphatic angioendothelioma (PILA): a report of twelve
cases of a distinctive vascular tumor with phenotypic features of lymphatic
vessels.
Fanburg-Smith JC, Michal M, Partanen TA, Alitalo K, Miettinen M.
Department of Soft Tissue Pathology, Armed Forces Institute of
Pathology, Washington, DC 20306-6000, USA.
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Am J Surg Pathol 1999 Sep;23(9):1004-10 Abstract quote
Six childhood vascular tumors were designated as "malignant endovascular
papillary angioendothelioma" by Dabska in 1969. Since then, a few reports
of similar cases were published, often called "Dabska tumors."
Twelve similar cases were identified in review of vascular tumors from
the authors' institutions.
There were five men and seven women, including seven adults. Patient
ages ranged from 8 to 59 years (mean, 30 years). The tumors occurred
in the dermis or subcutis of the buttocks or thigh (n = 6), thumb or
hand (n = 3), abdomen (n = 2), and heel (n = 1). The tumor sizes ranged
from 1 to more than 40 cm (mean, 7.0 cm).
The unifying feature of all cases was distinctive intravascular growth
of well-differentiated endothelial cells presenting as a matchstick
columnar configuration, sometimes with a large production of matrix
that was positive for collagen type IV. In half the cases, these intravascular
proliferations had an associated actin-positive pericytic proliferation.
There was minimal cytologic atypia and rare to absent mitotic activity.
Two cases had an adjacent lymphangioma, and two additional cases had
clusters of lymphatic vessels adjacent to the tumor. All but two of
the cases showed varying degrees of stromal or intraluminal lymphocytes.
Occasional epithelioid endothelial cells were seen, but no cases had
features typical of epithelioid, spindle cell, or retiform hemangioendothelioma.
Tumor cells were positive for vimentin, von Willebrand factor, CD31,
and focally for CD34 and were negative for keratins, epithelial membrane
antigen, S-100 protein, and desmin. Vascular endothelial cell growth
factor receptor type 3, a recently introduced marker for lymphatic endothelia,
was positive in all eight cases that were studied, supporting a lymphatic
phenotype. Follow-up in 8 of the 12 cases showed no evidence of recurrences,
metastases, or residual disease during follow-ups ranging from 1 to
17 years (mean, 9 years).
Based on the proliferative borderline features and the lymphatic phenotype,
we propose to designate these tumors as papillary intralymphatic angioendothelioma.
Additional cases with extensive follow-up should be studied to rule
out variants with malignant potential.
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