Papillary intralymphatic angioendothelioma

Papillary intralymphatic angioendothelioma (PILA), also known as Dabska tumor or endovascular papillary angioendothelioma, is an extremely rare vascular tumor that arises from the cells lining lymphatic vessels. It is classified as a borderline (low-grade malignant) vascular tumor, meaning it has the potential to behave aggressively in some cases but generally follows a relatively indolent (slow-growing) course. The tumor most commonly appears as a painless, slow-growing mass in the skin or soft tissues, often in the arms or legs, though it can occur in other locations including the head, neck, spleen, or bone. PILA typically presents as a firm, bluish or reddish nodule under the skin that may gradually enlarge over weeks to months. In some cases, the overlying skin may appear discolored or swollen. The tumor can sometimes involve nearby lymph nodes, which is why careful evaluation is important after diagnosis. While distant spread (metastasis) is rare, local recurrence after removal has been reported in some patients. Treatment usually involves complete surgical removal of the tumor with clear margins. Because of its rarity, there is no standardized chemotherapy or radiation protocol, though these may be considered in cases where the tumor cannot be fully removed or has spread. Long-term follow-up with imaging and clinical examinations is recommended to watch for recurrence. The overall prognosis is generally favorable when the tumor is completely excised, though ongoing monitoring is important.

Bring these to your next appointment

• Q1.Was the tumor completely removed with clear margins?,What is the risk of the tumor coming back, and how will we monitor for recurrence?,How often should follow-up imaging or examinations be scheduled?,Are there any signs or symptoms I should watch for that would require immediate attention?,Should I seek a second opinion from a specialist in rare vascular tumors?,Is there any role for additional treatment such as radiation or chemotherapy in my case?,Are there any clinical trials or research studies I could participate in?

Common questions about Papillary intralymphatic angioendothelioma

Related conditions

Other rare diseases commonly researched alongside Papillary intralymphatic angioendothelioma.

• Angiosarcoma→
• Composite hemangioendothelioma→
• Kaposi sarcoma→
• Kaposiform hemangioendothelioma→
• Retiform hemangioendothelioma→