Sclerosing Angiomatoid Nodular Transformation (SANT) of the spleen: Case report and review of the literature

Department of General Surgery, Cleveland Clinic Foundation, Digestive Disease Institute, 9500 Euclid Avenue, Cleveland, OH 44195, United States.
International Journal of Surgery Case Reports 07/2012; 3(10):492-500. DOI: 10.1016/j.ijscr.2012.06.003
Source: PubMed


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.

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    • "SANT is composed of multiple angiomatoid nodules derived from the brown-red pulp separated by interspersed bands of fibrous tissue which unite to form a central stellate fibrous scar. The size of these tumors is reported to range between 3 and 17 cm, and the male-to-female ratio is approximately 1:1 [1] [3]. Microscopically, SANT has numerous capillaries and sinusoidal spaces separated by a fibrous stroma. "
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    ABSTRACT: Sclerosing angiomatoid nodular transformation (SANT) is a recently recognized and rare, non-neoplastic lesion of the spleen. It has a distinct gross appearance. Some papers have reported an increased SANT diameter during the follow-up period. This makes the diagnosis between benign and malignant difficult. We present 2 cases of SANT whose size increased during the follow-up period. Diffusion weighted MR imaging (DWI) findings correlated well with the pathological findings of SANT, the multinodular high-intensity area as the angiomatoid nodules and the peripheral low-intensity area as fibrous tissues. Therefore, DWI may be a useful imaging tool for diagnosing SANT preoperatively.
    Clinical Imaging 10/2014; 39(2). DOI:10.1016/j.clinimag.2014.10.015 · 0.81 Impact Factor
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    • "A diagnosis of malignant lymphoma was initially made in a number of the IPTS cases reported in the literature, as occurred in the present case prior to surgery. The differential diagnosis for IPTS also considers other lesions, including hamartomas, hemangiomas, hemangioendotheliomas, angiosarcomas, infectious granulomatous processes and sarcoidosis (4–7,11–15,21,22). "
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    ABSTRACT: Inflammatory pseudotumor of the spleen (IPTS) is an extremely rare condition. To the best of our knowledge, only ∼113 cases have been reported in the literature since the first 2 cases were reported in 1984. The present study reports the case of an IPTS in a 72-year-old male patient. The splenic tumor was identified incidentally 1 year prior to the patient being admitted to the Second Affiliated Hospital of Dalian Medical University (Dailan, China). There were no specific clinical symptoms. The initial diagnosis was of splenic lymphoma based on the pre-operative radiological findings. However, the patient underwent a splenectomy and the final pathological diagnosis of IPTS was declared. The present study also highlighted the difficulty of forming accurate pre-operative diagnoses, even when using modern imaging techniques. A partial resection of the spleen or splenectomy was considered to be the required treatment to form a definitive diagnosis and exclude malignancy. The prognosis of IPTS is generally considered to be favorable following splenectomy. The clinical and pathological features of previously reported cases are also briefly reviewed in the present study to aid in improving the accuracy of the diagnosis of this rare disease.
    Oncology letters 06/2013; 5(6):1955-1957. DOI:10.3892/ol.2013.1286 · 1.55 Impact Factor
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    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.
    Archives of pathology & laboratory medicine 09/2013; 137(9):1309 - 1312. DOI:10.5858/arpa.2012-0601-RS · 2.84 Impact Factor
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