Combined lymphangioma and hemangioma of the spleen in a patient with Klippel–Trénaunay syndrome

Spasić, Marko; Radovanovic, Dragce; Čanović, Dragan; Azanjac, Goran; Djurdjević, Predrag; Mitrović, Slobodanka

doi:10.2298/sarh1212777s

Cited by 8 publications

(6 citation statements)

References 20 publications

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“…Splenic hemangioma is the most common benign tumor and the most uncommon is hemolymphangioma. There were only 3 cases reported on spleen previously (PubMed), 2 , 17 , 18 and the characteristics are shown in Table 2 . Histologically, hemangioma distributed throughout the organ parenchyma and filled with endothelial cells expressing vimentin, factor VIII, and CD 31, but not CD8.…”

Section: Discussionmentioning

confidence: 99%

Multiple Hemolymphangioma of the Visceral Organs

Zhang

Wang

et al. 2015

Medicine

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Hemolymphangioma is a rare disease with malformation of both lymphatic and vascular vessels. Few cases of hemolymphangioma occurring in the rectum, small intestine, pancreas, esophagus, and other organs have been reported. Nevertheless, multiple hemolymphangioma of the visceral organs are extremely rare. We report a 25-year-old female with a significantly enlarged spleen full of multiple-rounded lesions. Curiously, the splenic flexure and even retroperitoneum had many lesions. The patient recovered well after splenectomy and the pathologic diagnosis of spleen was hemolymphangioma with abnormal lymphatic and blood vessels with polycystic spaces.Usually, it is hard to cure this disease. We should take much more consideration into the diagnosis, treatment, and even pathogenesis, even though it is a benign lesion.

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Section: Discussionmentioning

confidence: 99%

Multiple Hemolymphangioma of the Visceral Organs

Zhang

Wang

et al. 2015

Medicine

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“…40,52,53 Two additional patients affected by Klippel-Trenaunay-Weber syndrome were also observed in whom multiple hepatic hemangiomas 54 or mixed form of splenic hemangio-lymphangiomatosis 55 have also been reported. 40,52,53 Two additional patients affected by Klippel-Trenaunay-Weber syndrome were also observed in whom multiple hepatic hemangiomas 54 or mixed form of splenic hemangio-lymphangiomatosis 55 have also been reported.…”

Section: Authors' Commentmentioning

confidence: 97%

Selected Case From the Arkadi M. Rywlin International Pathology Slide Series

Bisceglia

Chiaramonte²,

Lauriola

et al. 2014

Advances in Anatomic Pathology

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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.

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“…Both occurred in patients with KTS, a rare congenital anomaly of blood vessels, characterised by a triad of superficial varicose veins, port-wine stain lesions, and bony and soft tissue hypertrophy. It is often accompanied by visceral manifestations, which rarely includes splenomegaly and splenic vascular lesions,6 7 though neither of these cases contained bone marrow lesions.…”

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confidence: 90%