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Srpski arhiv za celokupno lekarstvo 2012 Volume 140, Issue 11-12, Pages: 777-781
https://doi.org/10.2298/SARH1212777S
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Combined lymphangioma and hemangioma of the spleen in a patient with Klippel–Trénaunay syndrome

Spasić Marko ORCID iD icon (Klinika za hirurgiju, Klinički centar „Kragujevac“, Kragujevac)
Radovanović Dragče (Klinika za hirurgiju, Klinički centar „Kragujevac“, Kragujevac)
Čanović Dragan (Klinika za hirurgiju, Klinički centar „Kragujevac“, Kragujevac)
Azanjac Goran (Klinika za hirurgiju, Klinički centar „Kragujevac“, Kragujevac)
Đurđević Predrag (Interna klinika, Centar za hematologiju, Klinički centar „Kragujevac“, Kragujevac)
Mitrović Slobodanka ORCID iD icon (Služba za patologiju, Klinički centar „Kragujevac“, Kragujevac)

Introduction. Klippel–Trénaunay syndrome (KTS) is a very rare congenital anomaly of blood vessels, characterized by the following clinical triad: varicose superficial veins, port-wine stain and usually bony and soft tissue hypertophy of extremities, most often located in the lower extremities. It is often accompanied by visceral manifestations, and rarely combined with splenomegaly. Case Outline. A 30-year-old female patient came to the Surgery Clinic because of occasional left hypochondrial pain. After she was diagnosed with KTS combined with splenomegaly, splenectomy was performed. Macroscopic and microscopic spleen examination indicated the presence of tumor of vascular origin, presenting a combination of lymphangioma and hemangioma. Conclusion. Diagnosed KTS demands a thorough clinical examination of the patient because of the potential presence of visceral manifestations. When splenomegaly is present, even though being often benign, splenectomy is usually performed to alleviate accompanying symptoms which occur as a result of organ enlargement and compression, to prevent rupture and consequential bleeding when the vascular spleen tumor is large, and finally to avoid a possibility of malignant transformation.

Keywords: Klippel–Trénaunay syndrome, splenomegaly, hemangioma, lymphangioma