Rare vulvar tumors with their aggressive clinical course

Objective: To improve clinical diagnosis in patients with rare aggressive vulvar tumors on the basis of the authors’ observations and literature data. Materials and methods: The investigation enrolled 9 patients with rare aggressive vulvar tumors. Among them, there were 6 patients with Paget's cancer, 2 with desmoid fibroma, and 1 with aggressive angiomyxoma. Based on the international experience presented in the literature, the authors critically analyze their materials and present the most important points of clinical observations. Results: Patient complaints, the type of a primary lesion, and disease recurrences, the focus and relapses of the disease, and the features of a clinical course are described. The delayed diagnosis and high frequency of local relapses of the disease are given. Attention is focused on the shortcomings of surgical treatment planning, which often cause local relapses of the disease. The authors come to the conclusion that the patients should be treated in the clinics where the technique of combined, reconstructive-and-plastic, and expansion surgery are mastered. Conclusion: All over the world, there is an increase in the number of patients with rare tumors, including those localized in the vulvar area. The aggressive nature of tumors consists in the destruction of nearby organs and tissues, but metastases are extremely rare. To optimize the diagnosis and treatment, it is necessary to further accumulate clinical experience and to investigate the immunohistochemical characteristics and molecular genetic features of tumors.

Paget’s cancer, desmoid fibroma, aggressive angiomyxoma, vulva, tumor