Sclerosing Angiomatoid Nodular Transformation of the Spleen
- 1 September 2013
- journal article
- review article
- Published by Archives of Pathology and Laboratory Medicine in Archives of Pathology & Laboratory Medicine
- Vol. 137 (9), 1309-1312
- https://doi.org/10.5858/arpa.2012-0601-rs
Abstract
Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen is a rare benign lesion of the spleen with unknown etiology. SANT is classically considered to be a female-predominant disease, with most of the patients in the 30- to 60-year age group. Most lesions are found incidentally on imaging. Although SANT has specific imaging findings, the differential diagnosis from other splenic tumors or malignant lesions is very difficult. Histopathologically, these tumors reveal multiple confluent angiomatoid nodules; these nodules are surrounded by concentric collagen fibers exhibiting an inflammatory and myofibroblastic response and are accompanied by numerous erythrocytes and siderophages. The nodules are populated by endothelial cells, phenotypically recapitulating normal splenic vasculature, such as sinusoids, capillaries, and small veins. Nuclear atypia is minimal, mitotic figures are extremely rare, and necrosis is consistently absent. This lesion has a unique immunohistochemical profile characterized by CD34−CD31+CD8+ sinusoids, CD34+CD31+CD8− capillaries, and CD34−CD31+CD8− small veins. CD68 is positive in macrophages. Splenectomy is a useful and effective technique for the management of SANT. SANT patients have a good prognosis, with no recurrence after splenectomy. In this review, we discuss the current knowledge of SANT of the spleen and its clinical relevance.Keywords
This publication has 24 references indexed in Scilit:
- Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen: Case report and review of the literatureInternational Journal of Surgery Case Reports, 2012
- Sclerosing angiomatoid nodular transformation of the spleen masquerading as a sarcoma metastasisRare Tumors, 2010
- Sclerosing angiomatoid nodular transformation of the spleen in an adolescent with chronic abdominal painJournal of Pediatric Surgery, 2010
- Case report and magnetic resonance images of sclerosing angiomatoid nodular transformation (SANT) of the spleenBMJ Case Reports, 2009
- Sclerosing angiomatoid nodular transformation (SANT) of the spleen: Sonographic findingJournal of Clinical Ultrasound, 2009
- Predominance of IgG4+ plasma cells and CD68 positivity in sclerosing angiomatoid nodular transformation (SANT)Histopathology, 2008
- Detection of Epstein–Barr virus-encoded small RNA-expressed myofibroblasts and IgG4-producing plasma cells in sclerosing angiomatoid nodular transformation of the spleenVirchows Archiv, 2008
- F-18 FDG-avid sclerosing angiomatoid nodular transformation (SANT) of the spleen: case study and literature reviewPathology, 2007
- Littoral-cell angioma of the spleen: A case reportWorld Journal of Gastroenterology, 2007
- Fine‐needle aspiration cytology of splenic hamartomaDiagnostic Cytopathology, 2003