This rare tumor of blood vessels is best classified as a sarcoma of low grade malignancy. It occurs in many soft tissue locations but predominately involves the skin.
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EPIDEMIOLOGY CHARACTERIZATION SYNONYMS Malignant endovascular papillary angioendothelioma
Papillary intralymphatic angioendothelioma
The Dabska tumor: a thirty-year retrospect.
Schwartz RA, Dabski C, Dabska M.
Dermatology and Pathology, New Jersey Medical School, Newark, N.J., and Pathology, State University of New York at Buffalo School of Medicine, Buffalo, N.Y., USA
Dermatology 2000;201(1):1-5 Abstract quote
The Dabska tumor is a rare low-grade angiosarcoma also known as malignant endovascular papillary angioendothelioma of childhood. It primarily affects the skin of children and has a distinctive histologic pattern of anastomosing vascular channels with intravascular papillary outpouchings projecting, sometimes, in a glomerulus-like pattern, into a lumen lined by atypical columnar endothelial cells. Since its original description 30 years ago by one of us, knowledge in this area has expanded.
AGE RANGE-MEDIAN Infants and children
DISEASE ASSOCIATIONS CHARACTERIZATION LYMPHANGIOMA CIRCUMSCRIPTUM
- Dabska tumor arising in lymphangioma circumscriptum.
Department of Dermatopathology, Mount Sinai Medical Center, New York, NY, USA.
- J Cutan Pathol. 2008 Jan;35(1):65-9. Abstract quote
We describe a case of Dabska tumor (DT) occurring within a large congenital lymphangioma circumscriptum on the thigh of a 14-year-old female. Diagnostic biopsy showed numerous intravascular papillary projections lined by atypical endothelial cells within the anastomosing vascular channels of a lymphangioma circumscriptum. DT is regarded as a vascular tumor of intermediate malignant potential, most probably of lymphatic origin.
Although it has been described in pre-existing lymphangiomas, to the best of our knowledge this is the first case to be described in a pre-existing lymphangioma circumscriptum.
During a follow-up of 9 years, the lymphangioma circumscriptum has recurred, but there has been no evidence of DT recurrence or metastasis.
CLINICAL APPEARANCE/GROSS DISEASE VARIANTS CHARACTERIZATION
Endovascular papillary angioendothelioma (Dabska tumor) of bone.
McCarthy EF, Lietman S, Argani P, Frassica FJ.
The Johns Hopkins Hospital, Department of Pathology, Baltimore, MD 21287, USA.
Skeletal Radiol 1999 Feb;28(2):100-3 Abstract quote
Endovascular papillary angioendothelioma, also known as Dabska tumor, is a rare vascular neoplasm that usually involves the skin or subcutaneous tissue of children. There have been no reported cases of this lesion occurring in bone. We report a Dabska tumor in the distal femur of a 45-year-old woman who, clinically and radiologically, was felt to have an osteoid osteoma. Histologic study of the lesion showed a hemangioma with budding fronds of endothelial cells, a feature characteristic of the Dabska tumor.
We feel that the lesion arose in a pre-existing hemangioma, a hypothesis suggested in a few other case reports. Endovascular papillary angioendothelioma is a low-grade malignant neoplasm, although only one patient has died due to this lesion. Our patient is asymptomatic without evidence of recurrence 1 year post curettage.
Endovascular papillary angioendothelioma (Dabska tumor) in an elderly woman.
Yamada A, Uematsu K, Yasoshima H, Sakurai K, Hori K, Ohya M, Ohkubo E, Ogasawara H.
Department of Pathology (Hospital), Hyogo College of Medicine, Nishinomiya, Japan.
Pathol Int 1998 Feb;48(2):164-7 Abstract quote
A patient with endovascular papillary angioendothelioma with a low grade of malignancy showing papillary proliferation of endothelioid cells is presented.
The patient, an 83-year-old woman, underwent resection of a tumor of the neck. At operation a 9 x 7 cm cystic tumor containing yellow transparent liquid with clots was found in the subcutaneous tissue. Histological studies showed endothelioid cells with spindle-shaped nuclei proliferated in layers around the fibrovascular cores, which showed the characteristic appearance of papillary proliferation. These cells were immunohistochemically positive for CD31, CD34 and factor VIII-related antigen. Based on these observations, the tumor was considered to be an endovascular papillary angioendothelioma (Dabska tumor). Dabska tumor is a vascular tumor with a low grade of malignancy and usually occurs in infants and young children. About 13 cases of Dabska tumor have been reported.
The occurrence of a Dabska tumor in an aged patient is considered to be rare.
HISTOLOGICAL TYPES CHARACTERIZATION GENERAL Dilated thin walled intradermal vessels containing cellular papillary structures of endothelial cells and lymphocytes
Endothelial cells are small with small portions of eosinophilic cytoplasm and hobnail nuclei with cytologic atypia
Papillary tufts may have hyaline core with lymphocytes clustering around
Malignant endovascular papillary angioendothelioma. Cutaneous borderline tumor.
Patterson K, Chandra RS.
Arch Pathol Lab Med 1985 Jul;109(7):671-3 Abstract quote
Malignant endovascular papillary angioendothelioma, a rare but distinctive vascular tumor of childhood, was first described in 1969. It was termed malignant because of its mitotic activity, areas of necrosis, and demonstrated ability to metastasize to regional lymph nodes. Despite these features it had a uniformly good prognosis.
We report an example of this lesion in a 6-year-old boy that does not show malignant histologic features. Based on its good long-term prognosis and variable histologic features, this lesion is better classified as a borderline vascular tumor.
Endovascular papillary angioendothelioma (Dabska tumor)
Pathol Int 1998 Oct;48(10):840-1 Abstract quote
The histological diagnosis of endovascular papillary angioendothelioma (EPA) (Dabska tumor) is controversial although the tumor is included in the World Health Organization classification. Papillary endothelial proliferation with a central hyaline core is one of the most characteristic features of EPA; however, this type of proliferation has been observed in other vascular tumors, such as angiosarcoma, retiform hemangioendothelioma and glomeruloid hemangioma. Several vascular tumors have EPA-like foci and EPA is not well defined generally. Endovascular papillary angioendothelioma may not be a distinct entity and may well include a heterogeneous group of lesions.
Malignant endovascular papillary angioendothelioma (Dabska tumor) arising within a deep intramuscular hemangioma.
Argani P, Athanasian E.
Department of Pathology, Memorial Sloan-Kettering Cancer Center, New York, NY 10021, USA.
Arch Pathol Lab Med 1997 Sep;121(9):992-5 Abstract quote
We report a rare malignant endovascular papillary angioendothelioma (Dabska tumor) arising within the deep periosteal soft tissue of the ulna of an 8-year-old girl. To our knowledge, this is the first report of a Dabska tumor of deep soft tissue that did not extend down from the dermis. This Dabska tumor appears to be a focal change within a larger, well-defined, cavernous hemangioma of deep muscle.
We suggest that this represents secondary neoplastic progression of an existing benign lesion, a common phenomenon among vascular tumors. A review of the family of "hobnailed" endothelial cell lesions to which Dabska tumor belongs is presented.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES
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.
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.
Multiple lesions of intravascular papillary endothelial hyperplasia (Masson's lesions).
Stewart M, Smoller BR.
Department of Pathology, Stanford University Medical Center, CA 94305.
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.
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.
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.
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.
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.
PROGNOSIS AND TREATMENT CHARACTERIZATION Metastasis Rare case of metastasis Treatment Wide excision
Macpherson and Pincus. Clinical Diagnosis and Management by Laboratory Methods. Twentyfirst Edition. WB Saunders. 2006.
Rosai J. Ackerman's Surgical Pathology. Ninth Edition. Mosby 2004.
Sternberg S. Diagnostic Surgical Pathology. Fourth Edition. Lipincott Williams and Wilkins 2004.
Robbins Pathologic Basis of Disease. Seventh Edition. WB Saunders 2005.
DeMay RM. The Art and Science of Cytopathology. Volume 1 and 2. ASCP Press. 1996.
Weedon D. Weedon's Skin Pathology Second Edition. Churchill Livingstone. 2002
Fitzpatrick's Dermatology in General Medicine. 6th Edition. McGraw-Hill. 2003.
Weiss SW and Goldblum JR. Enzinger and Weiss's Soft Tissue Tumors. Fifth Edition. Mosby Elesevier 2008
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