1. Ann Pathol. 2009 Dec;29(6):491-4.

[Retiform haemangioendothelioma: a case report]

[Article in French]

de Wind A, Meert V, Chahidi N, Theunis A, Somerhausen Nde S.

Laboratoire d'anatomie pathologique, institut Jules-Bordet, 1000 Bruxelles,
Belgique. roland.dewind@bordet.be

Retiform haemangioendothelioma is a locally aggressive, very rarely metastasizing
vascular lesion. Histologically, it is characterized by distinctive arborizing
blood vessels resembling "rete testis" and lined by endothelial cells with
characteristic hobnail morphology. We present an additional case, in the leg of a
64-year-old patient. We discuss the classification of hemangioendotheliomas. The 
term hemangioendothelioma should be restricted to vascular tumours of
"intermediate malignancy" but has been used to designate tumours with variable
histological features and clinical behaviour. Spindle cell hemangio(endothelio)ma
is currently regarded as a benign reactive lesion. Kaposiform
hemangioendothelioma is potentially lethal due to consumption coagulopathy but no
metastasizing case has been reported. Epithelioid hemangioendothelioma is
associated with a significant metastatic risk and has been included in the
category of malignant vascular tumors. The vascular lesions fulfilling the strict
definition of hemangioendothelioma include retiform hemangioendothelioma,
papillary intralymphatic angioendothelioma "Dabska's tumor", composite
hemangioendothelioma and perhaps the controversial polymorphic
hemangioendothelioma.

PMID: 20005438 [PubMed - indexed for MEDLINE]


2. J Cutan Pathol. 2008 Feb;35(2):225-30.

Cutaneous composite hemangioendothelioma with satellitosis and lymph node
metastases.

Requena L, Luis Díaz J, Manzarbeitia F, Carrillo R, Fernández-Herrera J, Kutzner 
H.

Department of Dermatology, Fundación Jiménez Díaz, Universidad Autónoma, Madrid, 
Spain. lrequena@fjd.es

The term hemangioendothelioma has been used in recent years to name a
heterogeneous group of vascular neoplasms, intermediate in both biological
behavior and histopathologic appearance between benign tumors (hemangiomas) and
frankly malignant tumors (angiosarcomas). Thus, within the spectrum of
hemangioendothelioma have been successively included epithelioid
hemangioendothelioma, spindle cell hemangioendothelioma, retiform
hemangioendothelioma, kaposiform hemangioendothelioma, polymorphous
hemagioendothelioma of the lymph nodes, papillary intralymphatic
angioendothelioma (PILA) and composite hemangioendothelioma. The latter is a
vascular neoplasm showing varying combinations of benign, low-grade malignant and
malignant vascular components. We herein report a case of composite
hemangioendothelioma showing a combination of retiform hemangioendothelioma,
epithelioid hemangioendothelioma, spindle cell hemangioma and PILA. The neoplasm 
showed a more aggressive behavior than other reported cases of composite
hemangioendothelioma and it developed satellitosis and metastases to the inguinal
lymph nodes. Neoplastic cells expressed immunoreactivity for Prox-1, supporting a
lymphatic line of differentiation.

PMID: 18190450 [PubMed - indexed for MEDLINE]


3. Pathologe. 2002 Mar;23(2):118-27.

[Tumors of the lymphatic vessel of the skin and soft tissue]

[Article in German]

Mentzel T, Kutzner H.

tmentzel@w-4.de

Tumours of lymphatic vessels comprise only a small group in the heterogeneous
spectrum of vascular neoplasms of skin and soft tissues. However, this
discrepancy between haemangiomas/angiosarcomas and
lymphangiomas/lymphangiosarcomas probably represents the present inability to
reliably differentiate between lymphatic and capillary vascular endothelium.
Histologically, neoplastic lymphatic vessels tend to be lined by endothelial
cells with plumper and more prominent, matchstick-like nuclei (in contrast to
vascular spaces in haemangiomas that are lined by flat or epithelioid endothelial
cells), often show variations in the thickness of the vessel walls and are not
completely surrounded by actin-positive (myo)pericytes. Endothelial cells in
lymphatic neoplasms tend to be negative for CD34 or stain only focally positive
for this marker and an expression of lymphatic markers as vascular endothelial
growth factor-C receptor (VEGRF-3), podoplanin(D2-40) and M2A oncofetal antigen has been
reported most recently. In addition to "traditional" lymphatic neoplasms
including lymphangioma circumscriptum, cavernous lymphangioma/cystic hygroma,
benign lymphangioendothelioma, lymphangiomatosis and the rare lymphangiosarcoma, 
histological and immunohistochemical features of a group of vascular neoplasms
with a hobnail cytomorphology (hobnail haemangioma, retiform
haemangioendothelioma, papillary intralymphatic angioendothelioma, benign
lymphangiomatous papule following radiotherapy) suggest that these lesions also
belong to the spectrum of tumours of lymphatic vessels.

PMID: 12001527 [PubMed - indexed for MEDLINE]


4. Am J Surg Pathol. 1999 Sep;23(9):1004-10.

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.

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.

PMID: 10478659 [PubMed - indexed for MEDLINE]