Sclerosing Angiomatoid Nodular Transformation of the Spleen Diagnosed After Laparoscopic Splenectomy

Young Hoe Hur, MD, Ho Hyun Kim, MD, Hee Joon Kim, MD, Byung Gwan Choi, MD, Eun Kyu Park, MD, Yang Seok Koh, PhD, Jung Chul Kim, PhD, Chol Kyoon Cho, PhD, Hyun Jong Kim, PhD. Department of Surgery, Chonnam National University Medical School

(Introduction) Vascular tumor or tumor like lesion of the spleen are rare and mainly incidentally on radiologic studies. Among them, sclerosing angiomatoid nodular transformation (SANT) of the spleen is a benign vascular splenic mass, which has been reported in less than 100 cases since it was first described by Martel et al. in 2004. Specifically, SANT is a vascular lesion of the red pulp of the spleen. Here, we report on a case of SANT of the spleen, which was diagnosed after laparoscopic splenectomy.
(Methods) A 51-year-old woman visited our hospital complaining of abdominal pain. Abdominal computed tomography revealed a 5.1 × 4.6 cm well-defined heterogenously enhanced nodular mass in the spleen. The laboratory data revealed that liver and renal function were within normal limits, and tumor markers including CEA and CA 19-9 were normal. The initial impression was benign lesion such as hamartoma or hemangioma. Laparoscopic splenectomy was performed.
(Result) The macroscopic examination of the specimen showed a 4 × 4-cm tumor with red-brown multi-nodular surface. Microscopically, the tumor was composed of multiple vascular structures separated by fibrous connective tissue. An immunohistochemical examination showed positive staining for CD31, CD34, factor VIII (<1%), and IgG4 (<1%). Based on these results, the tumor was diagnosed as SANT of the spleen. The patient had an uneventful postoperative course.
(Conclusion) Although the etiology and pathogenesis of SANT is still known, some report about the connection SANT to IgG4-related sclerosing disease. In our case, immunohistochemical staining for IgG4 was positive. Thus, our data support the possibility that SANT is connected to IgG4-related sclerosing disease. But further studies based on large populations are necessary to clarify its pathogenesis. Here, we present a case of SANT of the spleen treated with surgical resection.

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