Dabska tumor, a low-grade angiosarcoma, often affects the skin and subcutaneous tissues. It is commonly found in children. It is a rare type of hemangioendothelioma, distinguished by the presence of intraluminal papillary endothelial structures. It is often congenital or may sometimes grow from a pre-existing benign vascular lesion. Most of the tumors develop superficial, but some may develop in deeper tissues also.
Dabska tumor was first originally described by Maria Dabska in the year of 1969 by presenting six patients in a 14-year duration of study. She was working at Maria Sklodowska-Curie oncology institute in Poland. There, she was a member of the pathology department. Maria Dabska named this tumor endovascular papillary angioendothelioma of the skin. Since it was first described, there have been only 30 case reports presenting Dabska tumors with varying patterns.
Enzinger and Weiss termed the Dabska tumor as a low-grade angiosarcoma occurring in the skin and subcutis of infants. Although this tumor is rare in adults, few cases have been reported by younger adults.
How Is Dabska Tumor Caused?
-
The cause of the Dabska tumor is yet to be found. There were not many cases reported till now; hence, the etiology is still unknown. There are no risk factors identified till now.
-
Few studies have been conducted recently suggesting that there is lymphatic differentiation. It is viewed as intralymphatic neoplasia. This tumor is similar to that of retiform hemangioendothelioma.
-
Due to its similarity, the histogenic relationship is assumed. Retiform hemangioendothelioma might be an adult form of the Dabska tumor. This tumor can be classified under hemangioendothelioma since it is a neoplasm of blood vessels. It displays behavior varying from benign hemangiomas to malignant angiosarcomas.
-
Tumor marker studies of Dabska tumors suggest that these tumors are lymphangiomas and not angiosarcoma due to the identification of lymphatic endothelial cell marker vascular endothelial cell growth factor 3.
-
Frequency and race are not yet established due to the lesser number of cases reported now. It is more commonly found in children than in adults.
What Does a Dabska Tumor Look Like?
-
It is a slow-growing tumor that develops into a nodule within the layers of the skin.
-
It can grow up to 40 cm in size.
-
It is asymptomatic.
-
It is localized to subcutaneous tissues of extremities and dermis.
-
Few tumors are poorly circumscribed.
-
Few tumors show surface nodularity with ulceration.
-
Rarely do Dabska tumors have satellite nodules.
-
The color of the tumor may vary. It can be pink, violet, or blue.
-
Dabska tumor has atrophic dermis.
-
It can occur anywhere in the body.
-
Commonly found on the head and extremities.
-
Other areas of the body where Dabska tumors can develop are the neck, trunk, tongue, cheeks, palms, heels, buttocks, brain, spleen, bone, and testis.
-
Few Dabska tumors are observed to be associated with the degeneration of underlying vascular tumors.
-
Rarely, Dabska tumors are painful, ulcerate, and cause bleeding.
-
Symptomatic tumors are often associated with regional nodal involvement and pulmonary metastasis.
What Is the Histological Appearance of Dabska Tumor?
Diagnosis of a Dabska tumor is often a challenging one due to its rarity, multifocality, and morphological differentiation. Confirmation of diagnosis of Dabska tumor is possible only by biopsy followed by laboratory investigation. The specimen that is collected is sent to a laboratory for immunohistochemistry studies and histopathological examination.
-
Dabska tumors are present in the dermis and/or subcutaneous tissues. They are characterized by numerous interconnecting vascular channels with papillary tufts. These vascular channels are lined by endothelial cells forming a hobnail or matchstick appearance, assuming a focal pattern of rosettes.
-
The nucleus of the cells is apically placed, giving rise to a surface bulge.
-
The tumor shows moderate mitotic activity. It shows a high nuclear-to-cytoplasmic ratio.
-
It also shows intravascular and perivascular infiltrates.
-
The finding that helps in distinguishing Dabska tumors is papillary projections. The tumor shows intraluminal lymphocytes attached to endothelial cells.
-
Immunohistochemistry shows that tumor cells react positively to vascular and lymphatic markers.
-
It is positive for the lymphatic endothelial marker, D2-40, and Factor VIII-related antigen.
-
Dabska tumor shows positive reactivity for CD31, CD34, and CD68.
-
It displays a positive reaction for alpha-smooth muscle action.
-
Under an electron microscope, small granules consisting of Von Willebrand factor and P-selectin are called Weibel-Palade bodies.
-
Electron microscopy also reveals the presence of pinocytotic vesicles and cells with irregular nuclei rich in perinuclear cytoplasmic filaments.
-
Hyaline globules with electron-dense basement membranes are also found.
What Is the Treatment for Dabska Tumor?
Wide local excision is the treatment of choice for these vascular tumors. Recurrence after excision is unusual. Though it has a good prognosis, it can be locally invasive and undergo metastatic transformation. If the nodal spread is suspected, lymph node dissection should be conducted.
Conclusion:
Dabska tumor is a rare, low-grade vascular neoplasm found in skin and subcutaneous tissue. It has slow progress with low invasive ability. The outcome of this tumor is good, and the survival rate of patients is luckily high. Once surgical excision is performed, periodic, regular check-ups are suggested to ensure that there is no recurrence or nodal involvement. Long-term clinical follow-up is necessary along with the multi-disciplinary approach to assess the metastatic spread of cancer, if any.
