Sclerosing angiomatoid nodular transformation of the spleen - An uncommon splenic pseudotumorus variant

Abstract

Introduction Sclerosing angiomatoid nodular transformation is a benign splenic pseudotumorous multinodular vascular proliferation. In the past, it was usually reported as splenic hamartoma, multinodular hemangioma or splenic hemangioendothelioma. Since it was defined in 2004, a total of 150 cases have been reported. We will present our experience with a 58-year-old female patient who underwent splenectomy due to the tumorous change in the upper pole of the spleen, histopathologically characterized as sclerosing angiomatoid nodular transformation of the spleen. Case outline A 58-year-old women presented with abdominal pain, anemia, elevated C-reactive protein and fibrinogen level. Abdominal ultrasound and MDCT scan found a well-circumscribed, homogeneous, low-density tumor in the upper pole of the spleen. As the nature of the tumorous change could not be accurately determined and malignancy could not be excluded, splenectomy was performed. Histological findings showed multiple similar nodular foci, hardly discernible from splenic parenchyma, angiomatoid nodules surrounded and separated by partly collagenized fibroblastic areas admixed with mononuclear inflammatory infiltrate in various proportions. All findings were characterized as the coexistence of sclerosing angiomatoid nodular transformation of the spleen and splenic inflammatory pseudotumor. Conclusion Splenectomy, laparoscopic or open, is an acceptable therapeutic and at the same time diagnostic method. Considering the important role of the spleen in the immune system, partial splenectomy is also an option, especially in children. However, the coexistence of sclerosing angiomatoid nodular transformation of the spleen and inflammatory pseudotumor indicates a careful treatment decision, given the tendency of inflammatory pseudotumor to relapse, and, rarely, the possibility of malignant transformation.

Report on four cases of the rarest benign splenic tumor - myoid angioendothelioma, with literature review

“…The definitive and only authoritative diagnosis is made by histopathological and immunohistochemical examination [2,5,12].…”