Splenic papillary angioendothelioma in a 6-year-old girl

J Pediatr Hematol Oncol. 2007 Dec;29(12):808-10. doi: 10.1097/MPH.0b013e31815814b1.

Abstract

Papillary angioendothelioma is a rare, low-grade neoplasm of lymphatic channels that usually presents intradermally. We report the case of a 6-year-old girl with isolated splenomegaly and symptoms of early satiety and weight loss, whom was found to have a splenic papillary angioendothelioma. Preoperative abdominal computed tomography scan showed an irregular, heterogeneous mass; a tagged red cell scan ruled out a hemangioma, whereas a positron emission tomography scan showed mildly increased uptake. Subsequent surgery and pathologic assessment revealed a papillary angioendothelioma (Dabska tumor) within lymphatic spaces. The child has no evidence of recurrence or metastases 1 year postoperatively.

Publication types

  • Case Reports

MeSH terms

  • Child
  • Female
  • Hemangioendothelioma / diagnostic imaging*
  • Hemangioendothelioma / pathology
  • Hemangioendothelioma / surgery*
  • Humans
  • Positron-Emission Tomography
  • Splenic Neoplasms / diagnostic imaging*
  • Splenic Neoplasms / pathology
  • Splenic Neoplasms / surgery*
  • Tomography, X-Ray Computed
  • Treatment Outcome