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AUTHOR AND EDITOR INFORMATION

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Author: Robert A Schwartz, MD, MPH, Professor and Head of Dermatology, Professor of Medicine, Professor of Pediatrics, Professor of Pathology, Professor of Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School

Robert A Schwartz is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Editors: Albert C Yan, MD, Section Chief, Associate Professor, Department of Pediatrics, Section of Dermatology, Children's Hospital of Philadelphia and University of Pennsylvania; David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Director, Division of Dermatology, Scott and White Clinic; Director Dermatology Residency Training Program, Scott and White Clinic; Lester F Libow, MD, Dermatopathologist, South Texas Dermatopathology Laboratory; Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University; Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center

Author and Editor Disclosure

Synonyms and related keywords: endovascular papillary angioendothelioma, malignant endovascular papillary angioendothelioma, papillary intralymphatic angioendothelioma, Dabska's tumor, angiosarcoma, retiform hemangioendothelioma, childhood neoplasm, childhood tumor, pediatric neoplasm, pediatric tumor

INTRODUCTION

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Background

The Dabska tumor (DT) is a rare, low-grade angiosarcoma that often affects the skin of children. It has a distinctive histologic architecture of anastomosing vascular channels with intravascular papillary outpouchings projecting, sometimes in a glomeruluslike pattern, into a lumen lined by atypical columnar endothelial cells. Only approximately 30 patients have been described. In 1995, Enzinger and Weiss1 labeled DT "a form of low-grade angiosarcoma occurring in skin or subcutis of infants or young children, although we have seen rare cases in adults." A recent series of 12 patients detailed DT in 5 children and 7 adults. The diagnosis of DT or a DT-like tumor in a few, mainly adult, patients has been appropriately challenged.

Maria Dabska2 originally described DT in 1969 and named it malignant endovascular papillary angioendothelioma of the skin in childhood. She described 6 patients during a 14-year period (1953-1967) at the Maria Sklodowska-Curie Institute of Oncology in Warsaw, Poland, where she was a member of the Pathology faculty. Her pioneering study on keratoacanthoma was also accomplished at this institution.

The eMedicine article Angiosarcoma may be of interest.

Pathophysiology

Controversy remains about the DT, particularly whether it is a unique disorder or a heterogeneous group of neoplasms. DT may be viewed as a distinct intralymphatic neoplasia and renamed papillary intralymphatic angioendothelioma. Because some tumors share features of both DT and those of retiform hemangioendothelioma, a close histiogenic relationship between these 2 similarly behaving tumors has been inferred. Retiform hemangioendothelioma may be an adult form of DT; however, DT-like proliferations are also observed in ordinary angiosarcomas and glomeruloid hemangiomas. Accordingly, the diagnosis of DT should be restricted to low-grade angiosarcomas having histologic features characteristic of DT.

DT tumor marker studies suggest that DT is more like a lymphangioma than a hemangioma. This idea is supported by the finding of lymphatic endothelial cell marker vascular endothelial cell growth factor receptor type 3 in 8 of 8 cases examined. This receptor also occurs in fetal blood vessels prior to lymphatic differentiation.

D2-40 is a novel monoclonal antibody to a sialoglycoprotein that reacts with a fixation-resistant epitope in lymphatic endothelium. Expression of D2-40 was identified in lymphatic endothelium of normal tissues and in vascular tumors, with 3 of 3 Dabska tumors being positive for D2-40.3

Frequency

International

The incidence of DT is unknown. Only approximately 30 patients have been described worldwide.

Mortality/Morbidity

This is a locally aggressive low-grade neoplasm with metastatic potential. Thus, it has the ability to invade into bone, muscle, and fascia, producing local morbidity. It also may spread to regional lymph nodes and produce disseminated lung metastases with an unfavorable outcome.

Race

No ethnic or racial predilection is evident.

Sex

No predilection is yet evident. Of 30 persons affected, 9 of 18 children and 6 of 12 adults were females.

Age

The original group of 6 children ranged in age from 4 months to 15 years. Four had congenital DTs, and 2 had DTs that were discovered at age 7 months and 14 months, both with lymph node metastases. Of 30 persons affected, 18 were children and 12 were adults. The age range was from birth to 83 years.


CLINICAL

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History

  • Patients may notice a slow-growing, painless, intradermal nodule that grows to 2-3 cm in diameter.
  • Although it usually occurs in the skin or subcutis of infants and young children, in 2003, Takaoka et al4 described a DT in the tongue of a 67-year-old man. It was as a well-defined, reddish tumor measuring 11 mm X 8 mm X 7 mm at the submucosal area of the left tongue margin.
  • A DT may occur within a large congenital lymphangioma circumscriptum, as has been described on the thigh of a teenage girl.5

Physical

  • A DT tends to be a slow-growing intradermal nodule; violaceous, pink, or bluish-black in coloration; and several centimeters in diameter.
  • Some grow to 40 cm in diameter as an ill-defined mass. Others show discrete surface nodularity or even surface ulceration, and occasionally DTs have satellite nodules.
  • DTs have no apparent predilection for any anatomic site.
    • The head and extremities are commonly affected.
    • DTs also may be seen on the palms, forearms, heels, knees, cheeks, temples, pinnae of the ears, neck, buttocks, abdominal skin, and upper back. It has been described in the testis of a child.6 DTs can fluctuate in size and can be associated with degeneration of an underlying vascular tumor.
  • DTs have been described in the following situations:
    • A focal degeneration within a deep cavernous hemangioma of leg soft tissue in an 8-year-old girl
    • Within an angiomatous vascular malformation on the buttocks of a boy who developed Kasabach-Merritt syndrome7
    • As part of a subcutaneous cavernous hemangioma fixed to underlying fascia on the forearm of a 6-year-old boy
  • One or more of these hemangiomas may have been lymphangiomas.
  • Two others had an adjacent lymphangioma, and 2 more had clusters of lymphatic vessels adjacent to the tumor.

Causes

No causes have been linked to this type of tumor.


DIFFERENTIALS

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Acquired Digital Fibrokeratoma
Acute Hemorrhagic Edema of Infancy
Angiolymphoid Hyperplasia with Eosinophilia
Infantile Hemangioma
Kaposi Sarcoma

Other Problems to be Considered

Reactive angioendotheliomatosis
Benign intravascular endothelial hyperplasia
Angiosarcoma
Retiform hemangioendothelioma
Glomeruloid hemangioma

Poorly differentiated DT may resemble other angiosarcomas. Reactive angioendotheliomatosis, benign intravascular endothelial hyperplasia, angiosarcoma, and retiform hemangioendothelioma are all neoplasms that display intravascular proliferations.

Retiform hemangioendothelioma may have occasional intraluminal papillae with hyaline cores as in DT. Although retiform hemangioendothelioma and DT have biologic behavior and some histologic features in common, retiform hemangioendothelioma tends to involve young and middle-aged adults, with a tendency for involvement of the limbs and trunk. A DT-like tumor delineated in an elderly man may represent an intermediate form. Focal morphologic patterns of DT may sometimes be noted in ordinary angiosarcomas. In one series, 14% of 80 patients with angiosarcomas of soft tissue had papillary fronds, delicate and reminiscent of DT in 4 cases. The unique feature of DT is its papillary structure lined by atypical columnar endothelial cells. A similar formation has been noted extravascularly in patch-stage Kaposi sarcoma. These neoplasms do not usually express D2-40 and vascular endothelial growth factor receptor-3.8

DT has been described in the testis, first evident in a child as an inguinal hernia.6


WORKUP

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Lab Studies

  • DT tumor cells express immunoreactivity for factor VIII–related antigen, Ulex europaeus agglutinin I, vimentin, blood group isoantigens, and C 2.1 antibody, but not for S-100 protein, cytokeratins, epithelial membrane antigen, Leu-M1, HLA-DR, alpha-1-antichymotrypsin, and leukocyte common antigen. They also may show positive reactivities for CD31, CD34, and alpha-smooth muscle actin in most of the tumor cells and CD68 in some tumor cells.
    • The factor VIII–related antigen and Ulex europaeus agglutinin I immunoreactivity and other findings, even in the absence of HLA-DR expression, imply these neoplastic cells differentiate toward high endothelial cells, ie, those with surface receptors that serve as ligands in the selective homing of lymphocytes in lymphoid organs.
    • In one patient, dilated tumor vessels appeared to be filled with lymph rather than blood.
    • Accordingly, the DT appeared to be more like a lymphangioma than a hemangioma.
    • This concept is further embellished by the finding of the lymphatic endothelial cell marker vascular endothelial cell growth factor receptor type 3 in 8 of 8 cases examined.
    • Nevertheless, note that this marker also may be evident in fetal blood vessels prior to lymphatic differentiation.

Histologic Findings

DTs usually have normal overlying epidermis. The dermis has multiple vascular channels that interconnect and are lined by atypical endothelial cells. They may be partially filled with clear fluid. At low power, DTs appear similar to cavernous lymphangiomas. A unique pattern is their papillary structure lined with atypical columnar endothelial cells. Some papillary plugs have a central hyalinized core and project into a lumen lined by atypical columnar endothelial cells. These outpouchings sometimes appear in a glomeruluslike pattern.

Individual endothelial cells range from cuboidal to tall and cylindrical with vacuolated cytoplasm and hyperchromatic eccentric nuclei on their luminal border, thus resembling epithelium. Mitotic figures are uncommon. Many intraluminal lymphocytes may be evident, often attached to the endothelial cells. Focal changes characteristic of retiform hemangioendothelioma may occasionally be observed. Intravascular proliferations may stain positively for actin.

Ultrastructural studies demonstrate tumor cells with irregular nuclei, abundant perinuclear cytoplasmic filaments, and many pinocytotic vesicles. Weibel-Palade bodies may be evident. The hyaline globules consist of electron-dense basement membrane material.


TREATMENT

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Surgical Care

Wide local excision is the treatment of choice.

  • Recurrence after excision is unusual; however, the locally aggressive nature of DTs, with either diffuse lateral expansion or deep invasion into bone, musculature, fascia, and/or tendons, indicates the importance of follow-up evaluations.
  • Regional lymph node dissection may be indicated, especially if nodal involvement is detected.


FOLLOW-UP

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Prognosis

  • DTs usually have a favorable prognosis; however, they can be locally invasive and have the potential to metastasize.
  • Enzinger and Weiss1 classified DTs as an intermediate malignancy, a borderline category between angioma and angiosarcoma; however, 3 of the original 6 cases were locally aggressive, with tumor invasion into deeper structures, including bone, musculature, fascia, and/or tendons.
  • One of Dabska's2 original 6 DT patients ultimately died of widespread pulmonary metastases.
  • An additional patient had tumor involvement of the frontal bone and cranial vault.


MISCELLANEOUS

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Medical/Legal Pitfalls

  • Failure to evaluate vascular neoplasms in childhood: Not all are benign.
  • Failure to investigate properly for DTs: DTs may develop in deep tissue; for example, a changing deep cavernous hemangioma may represent the development of a focal DT.


REFERENCES

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Dabska Tumor excerpt

Article Last Updated: May 13, 2008