An Immunohistochemical Study of Angiokeratomas of Children

作者：Trindade Felicidade; Torrelo Antonio; Kutzner Heinz; Requena Luis; Tellechea Oscar; Colmenero Isabel^*

来源：American Journal of Dermatopathology, 2014, 36(10): 796-799.

DOI：10.1097/dad.0000000000000224

• angiokeratoma
• Wilms tumor 1
• GLUT1
• D2-40
• podoplanin
• Prox1
• ERG1
• vascular neoplasms
• vascular malformations

Background: Angiokeratomas are vascular anomalies in which the clinical, histological, and immunohistochemical characteristics are insufficient to determine whether the lesion is a vascular neoplasm or vascular malformation, and their exact origin is also uncertain. To further clarify the nosology of angiokeratomas, we studied 14 cases.
Objective: To analyze immunohistochemical characteristics of angiokeratomas to gain further insight into its histogenesis.
Methods: We carried out a retrospective review of the histopathology files. Immunohistochemical expression for Wilms tumor 1 (WT1), GLUT1, D2-40, podoplanin, Prox1, and ERG1 was performed in 14 cases.
Results: None of the lesions showed cytoplasmic immunoreactivity for WT1. GLUT1 resulted to be negative in 13 cases. All 14 cases in our series showed some expression with at least 1 lymphatic marker. In 12 cases, the positivity was diffuse and strong for ERG1.
Conclusions: Angiokeratomas are complex lesions with difficult classification. Angiokeratomas are best considered vascular malformations in children, according to the WT1-negative stain. The lymphatic component of angiokeratoma is demonstrated by positivity and/or focal expression for lymphatic markers (podoplanin and Prox1); however, a blood capillary component within the malformation cannot be excluded.

• 出版日期2014-10