Selected case from the Arkadi M. Rywlin international pathology slide series: Lymphangiomatosis of the spleen associated with ipsilateral abdominopelvic and lower extremity venolymphatic malformations: A case report and review of the literature

Michele Bisceglia, Antonio Chiaramonte, Walter Lauriola, Carlos A. Galliani, Pellegrino Musto, Giovanni Colacchio

Research output: Contribution to journalArticle

Abstract

Herein, we report a 26-year-old patient with lymphangiomatosis of the spleen associated with multiple lymphatic and venous malformations. This patient underwent excision of a large lymphatic malformation of the left abdominal wall during childhood. A venous malformation of her left lower limb was excised during adolescence. Additional lymphatic malformations were found in the soft tissue of her left thigh at the age of 20. During hospitalization for a huge vulvar hemangioma at the age of 26, she was incidentally found to have asymptomatic splenomegaly, for which she underwent splenectomy. Examination of the spleen revealed diffuse involvement by a lymphatic anomaly predominantly forming small cystic spaces. Lymphangiomatosis of the spleen is rare and is classically separated into an isolated or pure form and a generalized form when it is associated with involvement of other viscera and/or multiple soft-tissue planes. This patient was affected by a borderline form of splenic lymphangiomatosis with limited somatic involvement of the superficial soft tissues and blood vessels. Notably, all the additional vascular malformations in this patient were left sided, and at this time there was no additional involvement of internal organ. No hereditary or known syndrome was identified.

Original languageEnglish
Pages (from-to)291-299
Number of pages9
JournalAdvances in Anatomic Pathology
Volume21
Issue number4
DOIs
Publication statusPublished - 2014

Keywords

  • lymphangiomatosis
  • lymphatic malformation
  • multiple cysts of spleen
  • splenomegaly
  • venous malformation

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Anatomy
  • Medicine(all)

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