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      A case of sclerosing angiomatoid nodular transformation of the spleen with increased accumulation of fluorodeoxyglucose after 5-year follow-up

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          Highlights

          • Although sclerosing angiomatoid nodular transformation (SANT) of the spleen is a new entity defined as a benign pathologic lesion, it is very difficult for ruling out the malignancy in preoperative imaging studies.

          • Even when accumulation of fluorodeoxyglucose increased slightly during the follow-up period and suggested the possibility of malignant diseases, it is necessary to make a diagnosis of splenic SANT comprehensively.

          Abstract

          Introduction

          Sclerosing angiomatoid nodular transformation (SANT) of spleen is a new entity defined as a benign pathologic lesion. Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT) shows weak accumulation, thereby ruling out the malignancy in preoperative diagnosis is difficult. Herein, we reported a case of shrinking SANT with increased FDG accumulation during a 5-year follow-up period, which was treated by laparoscopic splenectomy.

          Presentation of case

          A 64-year-old female had been referred to our hospital for the evaluation of a splenic tumor. Initial contrast-enhanced computed tomography (CT) showed a well-defined, and ovoid hypoattenuating lesion, measuring 52 mm in diameter in the spleen. Initial PET/CT revealed accumulation of FDG in the tumor (maximum standardized uptake value [SUVmax]: 2.8). The mass was diagnosed as SANT, and the patient was followed-up every 6–12 months for 5 years. Follow-up PET/CT revealed increased accumulation of FDG (SUVmax: 3.5). As it was suspicious considering the differential diagnosis, including malignant lymphoma and inflammatory pseudotumor, she underwent laparoscopic splenectomy. The pathological results showed three types of vessels including capillaries, ectatic small veins, and sinusoids-like vessels, consistent with the features of SANT.

          Discussion

          A SANT may have features that resemble those of malignancy, including the growing mass and the increase of FDG accumulation.

          Conclusion

          Although the preoperative diagnosis of SANT is difficult, it is necessary to make a diagnosis of SANT comprehensively, even when accumulation of FDG increased slightly during the follow-up period and suggested the possibility of malignant diseases.

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          Most cited references18

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          The SCARE Statement: Consensus-based surgical case report guidelines.

          Case reports have been a long held tradition within the surgical literature. Reporting guidelines can improve transparency and reporting quality. However, recent consensus-based guidelines for case reports (CARE) are not surgically focused. Our objective was to develop surgical case report guidelines.
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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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              CD30 and Epstein-Barr virus RNA expression in sclerosing angiomatoid nodular transformation of spleen.

              Sclerosing angiomatoid nodular transformation (SANT) is a splenic lesion composed of angiomatoid/vascular nodules surrounded by hyalinized/sclerotic stroma, fibroblasts, myofibroblasts, and inflammatory cells. The endothelium within the nodules has a phenotype resembling splenic sinusoids, capillaries, and small veins. Martel et al. (Am J Surg Pathol 28:1268-1279, 2004) suggested that SANT may represent the final pathway of a variety of splenic lesions including inflammatory pseudotumors (IPTs). Epstein-Barr virus (EBV) has a role in the genesis of some splenic IPTs, but its presence in SANT has not been investigated. Six cases of SANT are reported. All were stained for CD31, CD34, CD8, CD68, smooth muscle actin, muscle-specific actin, and CD30 and were tested for EBV by in situ hybridization (EBER). All cases showed angiomatoid nodules with complex expression of CD31, CD34, and CD8, with focal CD68. Expression of CD30 by endothelial cells was also seen. One case had small diffuse areas lacking nodules resembling an IPT and was positive for EBV. The inflammatory cells and the normal spleen were negative for CD30 and EBER. In conclusion, SANT shows upregulation of CD30 with respect to normal spleen. The presence of EBV in the stromal cells of a case supports the notion that a subset of SANT may be related to IPT.
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                Author and article information

                Contributors
                Journal
                Int J Surg Case Rep
                Int J Surg Case Rep
                International Journal of Surgery Case Reports
                Elsevier
                2210-2612
                21 July 2017
                2017
                21 July 2017
                : 39
                : 9-13
                Affiliations
                [a ]Department of Gastroenterological Surgery, Hiroshima Prefectural Hospital, Japan
                [b ]Department of Gastroenterological and Transplant Surgery, Applied Life Sciences, Institute of Biomedical and Health Sciences, Hiroshima University, Japan
                [c ]Department of Pathology Clinical Laboratory, Hiroshima Prefectural Hospital, Japan
                [d ]Department of Internal Medicine, Hiroshima Prefectural Hospital, Japan
                Author notes
                [* ]Corresponding author at: Department of Gastroenterological Surgery, Hiroshima Prefectural Hospital, 1-5-54, Ujina-Kanda, Minami-ku, Hiroshima 7348530, Japan.Department of Gastroenterological SurgeryHiroshima Prefectural HospitalJapan oshita-akihiko@ 123456umin.ac.jp
                Article
                S2210-2612(17)30358-9
                10.1016/j.ijscr.2017.07.035
                5545820
                28783523
                929442bd-f2b8-4841-a1b9-49feab701ddf
                © 2017 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 3 June 2017
                : 17 July 2017
                : 17 July 2017
                Categories
                Case Report

                sant, sclerosing angiomatoid nodular transformation,ct, computed tomography,fdg, fluorodeoxyglucose,pet/ct, positron emission tomography/computed tomography,fna, fine-needle aspiration,mri, magnetic resonance imaging,suvmax, maximum standardized uptake value,sclerosing angiomatoid nodular transformation,fluorodeoxyglucose positron emission tomography/computed tomography

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