Papillary intralymphatic angioendothelioma

Papillary intralymphatic angioendothelioma (PILA), also known as Dabska tumor or Dabska-type hemangioendothelioma, is a rare form of vascular tumor that typically occurs in the skin or subcutaneous tissue. It was first described by Maria Dabska in 1969.

Clinical Presentation

PILA usually presents as a slow-growing, painless nodule or mass. It is most commonly found in the skin or subcutaneous tissue, but can also occur in other locations such as the lymph nodes, spleen, bone, and testis. The tumor can occur at any age, but is most common in children and young adults.

Pathology

Histologically, PILA is characterized by the presence of papillary structures within lymphatic vessels. These papillary structures are lined by endothelial cells that show mild to moderate atypia. The endothelial cells often contain vacuoles, which may be filled with lymph.

Diagnosis

The diagnosis of PILA is based on the histological features, as well as the clinical presentation. Immunohistochemistry can be helpful in confirming the diagnosis. The tumor cells are typically positive for vascular endothelial growth factor receptor 3 (VEGFR-3), D2-40, and CD31, which are markers of lymphatic endothelial cells.

Treatment and Prognosis

The treatment of choice for PILA is surgical excision. In some cases, radiotherapy or chemotherapy may be used. The prognosis of PILA is generally good, but the tumor can recur and metastasize, particularly if it is not completely excised.

See Also

• Hemangioendothelioma
• Vascular tumor
• Lymphatic system

References

Papillary intralymphatic angioendothelioma Resources
Latest articles Most recent articles Ongoing trials	Wikipedia