Dabska Tumor 

Updated: May 13, 2019
Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD 

Overview

Background

The Dąbska 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 Weiss[1] 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 Dąbska[2] (1921-2014) 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.[3] Her pioneering study on keratoacanthoma was also accomplished at this institution. She had retired and resided in Warsaw, Poland, until her death in 2014.

This tumor is the only one named in honor of its female describer, a brave hero of the Warsaw Resurrection of 1944 who survived deportation to Germany to graduate from medical school in Gdańsk and pursue a career in pathology, serving as an inspiration to many.[4, 5, 6]

The Medscape 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 two similarly behaving tumors has been inferred.[7] Retiform hemangioendothelioma may be an adult form of DT; however, DT-like proliferations are also observed in ordinary angiosarcomas and glomeruloid hemangiomas. Focal dabskoid tufts may also be evident within ordinary capillary hemangiomas.[8, 9] Accordingly, the diagnosis of DT should be restricted to low-grade angiosarcomas having histologic features characteristic of DT. Dabska tumor can also be classified under the rubric of hemangioendothelioma, a term used for some vascular neoplasms displaying behavior intermediate between entirely benign hemangiomas and highly malignant angiosarcomas.[10]

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.[11]

Etiology

No causes have been linked to this type of tumor.

Epidemiology

Frequency

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

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.

Prognosis

Dabska tumors (DTs) usually have a favorable prognosis; however, they can be locally invasive and have the potential to metastasize. Enzinger and Weiss[1] 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. An aggressive angiosarcoma has been described as arising within a DT in soft tissue.[12]

One of Dabska's original 6 patients ultimately died of widespread pulmonary metastases.[2] An additional patient had tumor involvement of the frontal bone and cranial vault.

 

Presentation

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 al[13] 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.[14]

Primary angiosarcoma of the brain displaying an intravascular papillary pattern consistent with that of DT has been described in a child. It occurred in continuity with a massive, multifocal intravascular papillary endothelial hyperplasia.[15]

Physical Examination

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.[16] DTs can fluctuate in size and can be associated with degeneration of an underlying vascular tumor. A huge 30 × 22 × 4 cm3 recurrent Dabska tumor of the flank was described in a 23-year-old woman and was successfully excised.[17]

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[18]

  • Within an angiomatous vascular malformation on the buttocks of a boy who developed Kasabach-Merritt syndrome[19]

  • As part of a subcutaneous cavernous hemangioma fixed to underlying fascia on the forearm of a 6-year-old boy

  • A slow-growing molelike nodule on the cheek of a middle aged woman[20]

One or more of these hemangiomas may have been lymphangiomas. Two others had an adjacent lymphangioma, and two more had clusters of lymphatic vessels adjacent to the tumor. Dabska tumor has also been described in bone.[21]

 

DDx

Diagnostic Considerations

Also consider the following:

  • Reactive angioendotheliomatosis

  • Benign intravascular endothelial hyperplasia

  • Angiosarcoma

  • Retiform hemangioendothelioma

  • Hobnail hemangioendothelioma

  • Glomeruloid hemangioma

  • Pseudolymphomatous cutaneous angiosarcoma[22]

  • Cutaneous epithelioid hemangioendothelioma[23]

  • Anastomosing hemangioma[24]

Poorly differentiated Dabska tumor (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.[25]

DT has been described in the testis, first evident in a child as an inguinal hernia.[16]

Polymorphous hemangioendothelioma, a rare vascular tumor of borderline malignant potential, is a distinct entity in the hemangioendothelioma group.[26]

Hobnail hemangioendothelioma in the right thigh has been described as being "Dabska type."[27] It has also been described in the jejunum.[28]

Differential Diagnoses

 

Workup

Laboratory 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.

Diagnosis of DT has been rendered by characteristic histopathology using fine-needle aspiration.[29] This technique may produce a smear showing many small clusters of tumor cells with rosettelike arrangement, along with papillary fragments with a fibrovascular core and hobnail-like arrangement of the cells.

See the image below.

Retiform hemangioendothelioma (Dabska tumor). Cour Retiform hemangioendothelioma (Dabska tumor). Courtesy of L Wozniak & KW Zielinski (own work), via Wikimedia Commons.
 

Treatment

Surgical Care

Wide local excision is the treatment of choice for Dabska tumor (DT). 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. A standardized approach might be desirable.[30]