Navigating Dąbska tumour with intra-abdominal cases: a 50-year surgical journey
摘要
Dąbska Tumour (DT), or Papillary Intralymphatic Angioendothelioma (PILA), is a rare lymphovascular neoplasm of intermediate malignancy first described in 1969. Initially identified in children, but it has since been reported in adults. PILA typically presents as slow-growing intradermal nodules with variable colour and sizes, ranging from small nodules to masses up to 400 mm in diameter. Although PILA predominantly affect the skin and subcutaneous tissues, but can invade deeper structures such as fascia, muscles, spleen, tongue, testis, and heart. PILA may arise de novo or in association with chronic lymphedema or preexisting vascular malformations. While generally carrying a favourable prognosis, it remains clinically challenging due to its potential for local recurrence and low-grade metastases. Given the rarity of PILA and the paucity of standardised management protocols, this review aims to consolidate existing knowledge, with emphasis on abdominal presentations, and outlines diagnostic and management strategies. improve