Diagnosis of Papillary Intralymphatic Angioendothelioma in the Abdominal Viscera: A Case Report

Papillary Intralymphatic Angioendothelioma (PILA), or Dąbska Tumour, is an exceedingly rare lymphovascular neoplasm of intermediate malignancy, predominantly affecting cutaneous and soft tissue sites. This case report highlights an unusual presentation of PILA in the mesentery of a 38-year-old male,...

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Main Authors: Abdus Salam Raju, Dinuke de Silva, Yasser Farooque, Tristan Rutland
Format: Article
Language:English
Published: Tehran University of Medical Sciences 2025-07-01
Series:Case Reports in Clinical Practice
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Online Access:https://crcp.tums.ac.ir/index.php/crcp/article/view/1032
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author Abdus Salam Raju
Dinuke de Silva
Yasser Farooque
Tristan Rutland
author_facet Abdus Salam Raju
Dinuke de Silva
Yasser Farooque
Tristan Rutland
author_sort Abdus Salam Raju
collection DOAJ
description Papillary Intralymphatic Angioendothelioma (PILA), or Dąbska Tumour, is an exceedingly rare lymphovascular neoplasm of intermediate malignancy, predominantly affecting cutaneous and soft tissue sites. This case report highlights an unusual presentation of PILA in the mesentery of a 38-year-old male, a site not previously documented. He initially underwent a splenectomy for symptomatic splenic hemangioma. His symptoms recurred two years later, presenting with haemoperitoneum and extensive abdominal involvement, including the colon, small bowel mesentery, and diaphragms. An en-bloc extended right hemicolectomy and partial hepatectomy achieved complete resection. Histopathology revealed hobnail endothelial cells and characteristic papillary structures, confirming PILA. Despite its rare visceral occurrence and diagnostic challenges, the patient showed no recurrence at six months post-surgery. This case underscores the importance of histopathological analysis and awareness of atypical presentations. Wider surgical excision remains the cornerstone of treatment, with vigilant follow-up essential for detecting recurrence and guiding future management protocols for this rare entity.
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series Case Reports in Clinical Practice
spelling doaj-art-ddce176e28a04a2c8b0facf9edf763972025-08-20T04:02:32ZengTehran University of Medical SciencesCase Reports in Clinical Practice2538-26832538-26912025-07-019610.18502/crcp.v9i6.18946Diagnosis of Papillary Intralymphatic Angioendothelioma in the Abdominal Viscera: A Case ReportAbdus Salam Raju0Dinuke de Silva1Yasser Farooque2Tristan Rutland3Department of General Surgery, Upper GI Surgery Liverpool Hospital, Sydney, NSW Australia. AND Western Sydney University, Sydney, NSW Australia.Department of Anatomical Pathology, NSW Health Pathology, Sydney, NSW Australia.Department of General Surgery, Upper GI Surgery Liverpool Hospital, Sydney, NSW Australia.Western Sydney University, Sydney, NSW Australia. AND Department of Anatomical Pathology, NSW Health Pathology, Sydney, NSW Australia.Papillary Intralymphatic Angioendothelioma (PILA), or Dąbska Tumour, is an exceedingly rare lymphovascular neoplasm of intermediate malignancy, predominantly affecting cutaneous and soft tissue sites. This case report highlights an unusual presentation of PILA in the mesentery of a 38-year-old male, a site not previously documented. He initially underwent a splenectomy for symptomatic splenic hemangioma. His symptoms recurred two years later, presenting with haemoperitoneum and extensive abdominal involvement, including the colon, small bowel mesentery, and diaphragms. An en-bloc extended right hemicolectomy and partial hepatectomy achieved complete resection. Histopathology revealed hobnail endothelial cells and characteristic papillary structures, confirming PILA. Despite its rare visceral occurrence and diagnostic challenges, the patient showed no recurrence at six months post-surgery. This case underscores the importance of histopathological analysis and awareness of atypical presentations. Wider surgical excision remains the cornerstone of treatment, with vigilant follow-up essential for detecting recurrence and guiding future management protocols for this rare entity. https://crcp.tums.ac.ir/index.php/crcp/article/view/1032PILAIntralymphatic angioendotheliomaDabska tumour
spellingShingle Abdus Salam Raju
Dinuke de Silva
Yasser Farooque
Tristan Rutland
Diagnosis of Papillary Intralymphatic Angioendothelioma in the Abdominal Viscera: A Case Report
Case Reports in Clinical Practice
PILA
Intralymphatic angioendothelioma
Dabska tumour
title Diagnosis of Papillary Intralymphatic Angioendothelioma in the Abdominal Viscera: A Case Report
title_full Diagnosis of Papillary Intralymphatic Angioendothelioma in the Abdominal Viscera: A Case Report
title_fullStr Diagnosis of Papillary Intralymphatic Angioendothelioma in the Abdominal Viscera: A Case Report
title_full_unstemmed Diagnosis of Papillary Intralymphatic Angioendothelioma in the Abdominal Viscera: A Case Report
title_short Diagnosis of Papillary Intralymphatic Angioendothelioma in the Abdominal Viscera: A Case Report
title_sort diagnosis of papillary intralymphatic angioendothelioma in the abdominal viscera a case report
topic PILA
Intralymphatic angioendothelioma
Dabska tumour
url https://crcp.tums.ac.ir/index.php/crcp/article/view/1032
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AT dinukedesilva diagnosisofpapillaryintralymphaticangioendotheliomaintheabdominalvisceraacasereport
AT yasserfarooque diagnosisofpapillaryintralymphaticangioendotheliomaintheabdominalvisceraacasereport
AT tristanrutland diagnosisofpapillaryintralymphaticangioendotheliomaintheabdominalvisceraacasereport