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, 8] 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. [9, 10] 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. [11]
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. [12]
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. [13]
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.
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Retiform hemangioendothelioma (Dabska tumor). Courtesy of L Wozniak & KW Zielinski (own work), via Wikimedia Commons.
