Endovascular papillary angioendothelioma is a low grade, rare vascular neoplasm (cancer) found in the skin and subcutaneous tissue. It is called low grade as its progress and invasive abilities are low. It affects the skin. This tumor is locally invasive and can metastasize. Metastasis is the ability of the cancerous cells to spread to the distant parts or organs of the body. This cancer mostly affects children. The Survival rate of the patients suffering from this cancer is luckily high. The first case of Endovascular papillary angioendothelioma was presented by Maria Dabska in 1979. She named it Malignant Endovascular papillary angioendothelioma of the skin in childhood. However, this cancer is also known as the Dabaska tumor after Maria Dabska’s name. Dabska tumors are fleshy masses that are firm; however, there are numerous blood vessels present in it these tumors giving it bluish or pinkish appearance. The sizes of the tumors vary depending on the site of the body and the extent of tumor growth.
Disease Epidemiology
This cancer compromises almost 2% of the skin cancer cases. Endovascular papillary angioendothelioma is mostly seen in children. Adults make only 25% of the cases. The most prevalent sites for its development are neck, head, and extremities. The lesion is rare in nature.
Disease Etiology
The cause of Endovascular papillary angioendothelioma is still unknown. Few recent research studies suggest that it has the origin of lymphatic differentiation. However, further studies are needed to confirm its exact origin and cause.
Sign and Symptoms
Fleshy masses are observed in Endovascular papillary angioendothelioma. However, if the lymph nodes are involved further pathological conditions can be assessed. Additionally, if the tumor has invaded deeper or has a large occupying area, it can disturb the functions of the adjacent organs.
Diagnosis and Treatment
Diagnosis is made through proper physical examination and laboratory tests and diagnostic procedures can be used to confirm the diagnosis:
- CBC with peripheral blood smear
- Histological Examination
- VEGF-3 (a lymphatic marker)
- CT scan to assess its invasion and progress
- Immunostaining with D2-40 ( found positive in these cancers)
- MRI
Treatment is done for a curative purpose; following surgical interventions are usually done to attain optimum curative results:
- Wide local excision of the lesion.
- Lymph node dissection if the involvement of the lymph node is found.
- Long term clinical follow-up is necessary as cancer tends to recur.
- Multidisciplinary follow-up and communication between patient and specialist to assess the metastatic spread of cancer.
What support can we give for Endovascular Papillary Angioendothelioma (Dabska Tumor)?
Endovascular Papillary Angioendothelioma (Dabska Tumor) is rare cancer, meaning it is not as well known as other forms of cancer. Without a Ribbon is an Australian organisation that provides support for individuals who suffer from rare cancers. We provide a designated platform for Warriors to obtain information specific to their Rare Cancer. We also provide annual opportunities for our Warriors to meet and learn from each other. If you suffer from rare cancer such as Endovascular Papillary Angioendothelioma (Dabska Tumor), we can help and support you through your journey thanks to the generous donations we receive. Click the link below to sign up and become a Warrior today!
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