Papillary intralymphatic angioendothelioma: An extremely rare tumor

Abstract

Papillary intralymphatic angioendothelioma (PILA), an intralymphatic lesion of vascular ducts, is an extremely rare tumor. It is generally encountered as an unexpected pathology following excision of a vascular skin lesion. Re-excision is the commonly preferred treatment option once the pathological diagnosis is established. In this article, we present a 12-year-old male patient with a PILA treated with re-excision, skin grafting, and sentinel lymph node biopsy. The patient was symptom-free at annual follow-up. A sentinel lymph node biopsy during re-excision might be a good option in the surgical management of PILA. Frequent examination of lymph nodes and skin lesion also might be beneficial during the follow-ups.

Locally Invasive Papillary Intralymphatic Angioendothelioma Arising Within a Lymphatic/Venous Malformation

“…This rare tumor occurs predominantly in infants, children, and young adults, but it can also occur in the elderly. There is a slight female predilection (M: F ratio: ~0.7: 1) [ 4 ]. The disease typically affects the dermis and subcutaneous tissues of the extremities and, to a lesser extent, the trunk, head, and neck.…”

Oropharyngeal Invasive Papillary Intralymphatic Angioendothelioma (Dabska’s Tumor): About an Unusual Location of an Exceptional Tumor

“…In 1969, Maria Dabska described PILA as a malignant childhood tumor in six children [1]. The tumor was described as a low-grade angiosarcoma characterized by papillary endovascular proliferations of atypical endothelial cells and anastomosing vascular channels [3]. In 2013, the latest edition of World Health Organization tumor classification (4th edition) revised the tumor description to rarely metastasizing lymphatic vascular neoplasm [3].…”

“…The tumor was described as a low-grade angiosarcoma characterized by papillary endovascular proliferations of atypical endothelial cells and anastomosing vascular channels [3]. In 2013, the latest edition of World Health Organization tumor classification (4th edition) revised the tumor description to rarely metastasizing lymphatic vascular neoplasm [3]. It is generally observed in the dermis and soft tissues; however, few cases were described in deeper locations such as spleen, tongue, testis and bone.In this paper, we report the first case (to our knowledge) of PILA occurring in the tonsil and extending to the oropharynx.…”

“…Mitotic figures may be seen. Many intraluminal lymphocytes may also be evident, often attached to the endothelial cells [1][2][3][4][5]. Immunohistochemically the tumor cells are positive for Von-Willebrand factor, CD31, CD34, ERG and vascular endothelial growth factor receptor-3 (VEGFR-3) [6].In deeper locations, it is often misdiagnosed and confused in particular with retiform hemangioendothelioma, or angiosarcoma.…”

“…To the best of our knowledge, less than 40 PILA cases have been reported in the literature [2]. It is generally observed in the dermis and soft tissues; however, few cases were described in deeper locations such as spleen, tongue, testis and bone [1][2][3]. In such cases, correct diagnosis and management can be challenging.…”