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      Sclerosing angiomatoid nodular transformation of the spleen: A case report and literature review

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          Abstract

          Sclerosing angiomatoid nodular transformation (SANT) is a rare benign splenic vascular lesion. Since it was first defined in 2004, a total of 132 cases of SANT have been reported in ~50 studies in the English literature. However, it remains difficult to form a definitive pre-operative differential diagnosis of SANT compared with other splenic tumors or malignant lesions. The present study reports a pathologically proven case of SANT in a 29-year-old man who initially presented with left upper quadrant and back discomfort. The study also provides a review of the current knowledge on the condition, including the clinical profile, imaging features, cytological features, differential diagnosis and treatment of SANT. The most important distinguishing features of SANT are its typical vascular character and lack of other features that are typical of a granuloma. A splenectomy is required and the diagnosis is based on pathological analysis.

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          Sclerosing angiomatoid nodular transformation (SANT): report of 25 cases of a distinctive benign splenic lesion.

          Twenty-five cases of a morphologically distinctive vascular lesion of the spleen are described. The patients were 17 women and 8 men, ranging in age from 22 to 74 years (mean, 48.4 years; median, 56 years). The most common presentations were incidental finding of an asymptomatic splenic mass (13 patients), abdominal pain or discomfort (6 patients), and splenomegaly (4 patients). None of the patients had evidence of recurrent disease after splenectomy. The splenic lesion was solitary, measuring 3 to 17 cm, and sharply demarcated from the surrounding parenchyma. The cut surface revealed a mass of coalescing red-brown nodules embedded in a dense fibrous stroma. All cases showed a remarkably consistent multinodular appearance at low-power examination. The individual nodules had an angiomatoid appearance, in the sense that they were composed of slit-like, round or irregular-shaped vascular spaces lined by plump endothelial cells and interspersed by a population of spindly or ovoid cells. Some of the nodules (particularly the smaller ones) were surrounded by concentric rings of collagen fibers. Numerous red blood cells were present, as well as scattered inflammatory cells. Nuclear atypia was minimal, mitotic figures were extremely rare, and necrosis was consistently absent. The internodular stroma consisted of variably myxoid to dense fibrous tissue with scattered plump myofibroblasts, plasma cells, lymphocytes, and siderophages. Immunostaining revealed 3 distinct types of vessels in the angiomatoid nodules: CD34+/CD8-/CD31+ capillaries, CD34-/CD8+/CD31+ sinusoids, and CD34-/CD8-/CD31+ small veins, recapitulating the composition of the normal splenic red pulp. These features are therefore different from those of littoral cell angioma, conventional hemangioma, and hemangioendothelioma of the spleen. We interpret these angiomatoid nodules as altered red pulp tissue that had been entrapped by a nonneoplastic stromal proliferative process. The characteristic morphologic appearance, immunophenotype, and benign clinical course suggest that this is a distinctive nonneoplastic vascular lesion of the spleen that we propose to designate as sclerosing angiomatoid nodular transformation (SANT).
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            Tumours of the spleen

            The spleen has been considered a ‘forgotten organ’ even if it is included and well demonstrated on every CT and MRI of the abdomen. Tumours of the spleen are rare; however, radiologists need to be aware of the main tumoral features and patterns in order to try to distinguish between benign and malignant masses often discovered incidentally. The principal tumoral masses, benign (cysts, haemangiomas, litteral cell angioma, lymphangioma) and malignant (lymphoma, metastases haemagiosarcoma), are described.
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              Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen: Case report and review of the literature.

              Sclerosing Angiomatoid Nodular Transformation of the spleen (SANT) is a rare benign vascular lesion of the spleen with extensive sclerosis and unknown etiology. We report a new case of SANT of the spleen found in a 53-year-old female following detection of a splenic mass on a routine computed tomography (CT). The patient underwent an uncomplicated laparoscopic splenectomy and the specimen was sent for histopathologic examination. A review of the 97 reported cases of SANT found in the literature was undertaken. There were 43 males and 54 females with a median age of 46 years (range: 11-82 years). SANT is classically considered to be a female predominant disease, however 44.3% of reported case were male and the gender predilection may soon be neutralized as more cases are reported. 65 of the 97 (67%) patients were in 30-60 year age group. The majority of lesions (n=50) were incidentally found on imaging, and for those patients presenting with symptoms, abdominal pain (n=18) was the predominant symptom. The diagnosis of SANT should be considered in any patient presenting with a splenic lesion that contains an angiomatoid or inflammatory component. As the differential diagnosis for SANT includes malignant pathologies, and currently no reliable diagnostic radiological feature has been identified to differentiate between these conditions, SANT will continue to be diagnosed on the basis of surgical histopathology. Copyright © 2012 Surgical Associates Ltd. Published by Elsevier Ltd. All rights reserved.
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                Author and article information

                Journal
                Oncol Lett
                Oncol Lett
                OL
                Oncology Letters
                D.A. Spandidos
                1792-1074
                1792-1082
                August 2016
                15 June 2016
                15 June 2016
                : 12
                : 2
                : 928-932
                Affiliations
                [1 ]Department of Surgery, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong 510080, P.R. China
                [2 ]Department of Gastrointestinal Surgery, The Affiliated Hospital, Binzhou Medical College, Binzhou, Shandong 256603, P.R. China
                [3 ]Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong 510080, P.R. China
                [4 ]Department of Radiology, The First Affiliated Hospital, Sun Yat-sen University, Guangzhou, Guangdong 510080, P.R. China
                Author notes
                Correspondence to: Professor Tian-Bao Wang, Department of Surgery, The First Affiliated Hospital, Sun Yat-sen University, 58 Zhongshan Second Road, Guangzhou, Guangdong 510080, P.R. China, E-mail: zsdxwtb@ 123456163.com
                [*]

                Contributed equally

                Article
                OL-0-0-4720
                10.3892/ol.2016.4720
                4950498
                27446372
                e09bfc04-4312-4093-b51b-9d3f4a5434de
                Copyright: © Wang et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivs License, which permits use and distribution in any medium, provided the original work is properly cited, the use is non-commercial and no modifications or adaptations are made.

                History
                : 02 April 2015
                : 17 May 2016
                Categories
                Articles

                Oncology & Radiotherapy
                sclerosing angiomatoid nodular transformation,spleen,diagnosis,treatment

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