Angiomatoid Nodular Transformation Research Articles

Sclerosing angiomatoid nodular transformation of spleen (SANT) – case report and literature review

Introduction Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular lesion of spleen of unknown aetiology. Case history A 74-year-old female presented with a 6 month history of iron deficiency anaemia and raised C-reactive protein. A full body CT scan showed an exophytic mass involving the anterior pole of the spleen measuring up to 6 cm in maximum dimension. No other abnormalities or relevant past history was noted. The clinical differential diagnoses were lymphoma or metastatic tumour, and thus a splenectomy was performed. Macroscopically, the spleen showed a well-demarcated tumour with red-brown nodules and central speckled area of fibrosis. The histology revealed a lesion composed of complex slit- like vascular spaces interspersed with spindle to ovoid cells and numerous inflammatory cells. On immunohistochemistry, all the nodules expressed CD31, CD34, SMA and CD30. EBER and IgG4 stains were negative. The lesion was finally diagnosed as a SANT. Discussion SANT is thought to be a reactive process rather than a true neoplasm. They may represent the final common pathway of a variety of splenic lesions including inflammatory pseudotumours. SANT may be related to IgG4 disease or EBV virus. They show upregulation of CD30 with respect to normal spleen. Splenectomy is usually the standard curative treatment.

Nonneoplastic, benign, and malignant splenic diseases: cross-sectional imaging findings and rare disease entities.

Splenic lesions are commonly encountered and are often incidental in nature. Benign splenic vascular neoplasms include hemangioma, hamartoma, lymphangioma, extra-medullary hematopoiesis (EMH), and sclerosing angiomatoid nodular transformation (SANT). Uncommonly encountered entities of the spleen include focal EMH, focal myeloma, angiomyolipoma, and SANT. Primary splenic angiosarcoma is the most common malignant nonhematolymphoid malignancy of the spleen. Lymphoma, myeloma, and metastases are the other malignant entities involving the spleen. The clinical presentation, key imaging findings, and associations of benign, neoplastic, and malignant diseases that can involve the spleen will be discussed. Radiologists can use multimodality imaging to diagnose entities involving the spleen by recognizing key imaging features and considering patient characteristics. However, biopsy may be warranted for definitive diagnosis when imaging findings are nonspecific.

Contrast sonographic appearance of sclerosing angiomatoid nodular transformation of the spleen.

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular tumour whose pathogenesis is unclear. Defined as a distinct entity for the first time in 2004 [1], SANT consists of multiple angiomatoid nodules surrounded by fibrous tissue that often forms a central scar. Typical features at computed tomography and magnetic resonance are characterized by a solitary mass with early peripheral enhancing radiating lines [2].

Differentiation of an Unclear Splenic Lesion in a Patient With Cholangiocarcinoma

Here we report on a 51-year-old man with the primary diagnosis of cholangiocarcinoma. Workup with CT and contrast-enhanced ultrasound revealed an additional lesion in the spleen, raising the concern for metastasis. Combined FDG PET/CT revealed a different metabolic pattern, making a metastasis unlikely. Histopathology of the splenic lesion confirmed sclerosing angiomatoid nodular transformation, a rare benign lesion of the spleen.

Sclerosing angiomatoid nodular transformation of the spleen: a new entity or a new name?

Sclerosing angiomatoid nodular transformation of the splenic red pulp has been described quite recently; many of the lesions previously diagnosed as splenic exuberant granulation tissue, multinodular hemangioma, and inflammatory pseudotumor could actually belong to this category. The lesion has been well reported intermittently in the past, but new cases with still newer associations keep appearing from time to time. There are no known risk factors and no inciting triggers have been proven. We report two such cases- one of which has extensive extramedullary haematopoiesis; a feature that has never been reported earlier. Clinico-morphological and radiological features along with pathogenesis are discussed in detail.

An unusual case of sclerosing angiomatoid nodular transformation: radiological and histopathological analyses

Sclerosing angiomatoid nodular transformation (SANT) is a rare benign primary vascular lesion of the spleen. Its etiology is still debated. Radiological characteristics are less known, although there are some reports regarding histopathological features ofSANT. A 21-year-old male patient was admitted to our hospital with fatigue, weight loss, and abdominal pain for 4 months. Physical examination, complete blood count, andbiochemical parameters were unremarkable. Dynamic contrast-enhanced computed tomography (CT) of the abdomen was performed. A heterogeneous well-defined hyperdense nodular lesion 3 cm in diameter was detected during the arterial phase. The detected lesion was seen as isodense with the spleen parenchyma during the portal venous and late venous phases. Magnetic resonance imaging (MRI) showed an isointense-hypointense nodular lesion on T1- and T2-weighted images. Intraabdominal LAM or splenomegaly was not detected. Microscopically, it was composed of angiomatoid nodules separated by central stellate fibrous stroma and fibrous septa. The contrast enhancement pattern was described as centrally hypovascular, radially progressive centripetal vascular contrast enhancement, called a spoke-wheel pattern in previously reported cases. We present CT and MRI findings and their correlation with histopathological findings of a case of unusual symptomatic SANT.

14. Sclerosing angiomatoid nodular transformation (SANT) of the spleen: A perth experience

Background Sclerosing angiomatoid nodular transformation of the spleen (SANT) is an uncommon, usually incidental, nonneoplastic vascular splenic lesion first described in 20041. Recent articles have suggested association of SANT with IgG4-related sclerosing disease and with Epstein-Barr virus RNA expression2. Aim A retrospective review of SANT cases reported in three Perth metropolitan hospitals over the past 20 years. Methods A computer-assisted search of all reported SANT cases from the Perth metropolitan hospital Anatomical Pathology database for the period 1992-2012 was performed. Slides were reviewed from the identified cases and, where formalin-fixed paraffin-embedded (FFPE) tissue was available, further immuno-histochemical stains were performed including CD8, CD30, CD31, CD34, D2-40, ERG, CD138, IgG, IgG4 and EBV-encoded small RNA (EBER). Results Four cases were identified, including three splenectomy specimens and one post-mortem specimen. All had the characteristic CD31/CD34/CD8 vascular immunophenotype1. Endothelial expression of CD30 was found in two of the cases. Architectural derangement was highlighted by an altered pattern of ERG immu-noperoxidase nuclear expression in the sinusoidal epithelium of SANT angiomatoid nodules. EBER was negative in the three cases with available FFPE material and no convincing evidence for IgG4-related sclerosing disease was identified. Conclusion Four cases of SANT are described, including immunohistochemical adjuncts to their diagnosis. No evidence for an association with EBV infection or IgG4-related sclerosing disease is identified in these cases.

Sclerosing angiomatoid nodular transformation of the spleen - a case report

Sclerosing angiomatoid nodular transformation of the spleen (SANT) is a rare, relatively new, and benign splenic lesion, which mostly affects middle-aged females. It is characterized by multiple angiomatoid nodules in fibrosclerotic stroma comprising myofibroblasts and infiltrating chronic inflammatory cells. The etiology is unknown. The diagnosis is based on the histopathologic examination of the splenectomy specimen. We report a classic case of SANT in a 52-year-old woman with abdominal discomfort and mild splenomegaly. The immunohistochemical profile and updated clinicopathological data of the disease are discussed.

A Case of Sclerosing Angiomatoid Nodular Transformation of the Spleen: Spoke Wheel Pattern on Computed Tomography

Sclerosing angiomatoid nodular transformation (SANT) is a rare, benign vascular neoplasm. Most patients have no clinical symptoms, and the tumors are usually discovered incidentally on abdominal computed tomography or ultrasonography. Some studies have reported the clinical features and imaging findings of SANT, but the diagnosis is based on histopathologic examination of a tissue specimen obtained at splenectomy. We report herein an incidentally discovered case of SANT and review the related literature. (Korean J Med 2014;86:357-361)

Distinctive PET/CT Features of Splenic SANT

A 44-year-old woman suffered from epigastralgia for 1 month. An abdominal sonography revealed a space-occupying lesion, about 6 cm, in the spleen. Contrast-enhanced CT revealed enhanced splenic lesions. The PET/CT showed FDG-avid multiple splenic nodules with a "prunes on bread" appearance in the maximum-intensity-projection image (MIP image). In sectional PET/CT images, a central cold area with peripheral increased FDG uptake in the splenic nodule is visible. Because splenic malignancy was suspected, laparoscopic splenectomy was performed. Histology revealed multiple nodules with angiomatoid appearance, CD31(+), CD34(+) and HHV-8(-) in the vascular space, typical for the rare sclerosing angiomatoid nodular transformation (SANT).

Sclerosing Angiomatoid Nodular Transformation of the Spleen

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.

A case of sclerosing angiomatoid nodular transformation of the spleen: Correlations between contrast‐enhanced ultrasonography and histopathologic findings

Sclerosing angiomatoid nodular transformation (SANT) is a recently recognized benign vascular lesion of the spleen. Detection of SANT as an incidentaloma has increased due to improvements in imaging techniques. However, a definitive diagnosis of SANT on CT or MRI remains difficult. We report the use of contrast-enhanced ultrasonography with Sonazoid in a case of SANT in a 50-year-old woman, with gross and microscopic pathologic correlations.

Sclerosing Angiomatoid Nodular Transformation of the Spleen: CT and MRI Features With Pathologic Correlation

The objective of this study was to describe the CT and MRI features of sclerosing angiomatoid nodular transformation of the spleen with pathologic correlation. Nine patients with surgically resected and pathologically confirmed sclerosing angiomatoid nodular transformation were included in the study. Clinical history was reviewed to determine patient demographics and symptoms at presentation. Gross pathologic, histologic, and immunohistochemical findings were recorded. CT (n = 9) and MRI (n = 4) examinations were evaluated for lesion shape and margins, intrinsic characteristics, and enhancement pattern. Patients included were six women and three men, with a mean age of 41.2 years. Pathologic features of sclerosing angiomatoid nodular transformation included multiple angiomatous nodules in a radiating pattern with a central stellate fibrous scar and evidence of hemosiderin deposition. On imaging, the lesions were solitary and round, 78% having a lobulated margin. They were heterogeneously hypoenhancing during the arterial and portal venous phases of contrast-enhanced CT or MRI, with peripheral enhancing radiating lines in 88% of lesions. They showed progressive enhancement and were isoenhancing or hyperenhancing in the delayed phase. A hypoenhancing central scar was shown on imaging in 22% of lesions. All lesions were hypointense on T2-weighted images. Sclerosing angiomatoid nodular transformation shows characteristic CT and MRI findings reflecting the underlying pathology. Typical features are a solitary, round, lobulated mass with early peripheral enhancing radiating lines and progressive enhancement of the angiomatous nodules; delayed enhancement of the fibrous tissue; and hypo-intense T2 signal intensity from hemosiderin deposition.

Sclerosing angiomatoid nodular transformation of the spleen related to IgG4-associated disease: report of a case

Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a rare benign vascular mass, with fewer than 100 cases documented. It is generally recognized as a vascular lesion that develops in the red pulp of the spleen; however, its pathogenesis is not clearly defined. We report a case of SANT of the spleen, which presents evidence to support the hypothesis that this disease entity is associated with IgG4-associated disease. Microscopically, the tumor was composed of multiple vascular structures separated by fibrous connective tissue and immunohistochemical examination revealed positive staining for CD31, CD34, factor VIII, and IgG4. Further research based on large number of cases is warranted to clarify the pathogenesis of this tumor.

腹腔鏡下に切除したsclerosing angiomatoid nodular transformationの1例

症例は52歳女性で，検診にて脾臓に5cm大の境界明瞭な腫瘤を指摘された．単純CTで低濃度，造影CTでは腫瘍周囲にのみ淡い造影効果を示した．MRIではT1・T2とも低信号で，腹部超音波検査では豊富な血流信号を伴う低濃度腫瘤でペルフルブタンによる造影では腫瘍辺縁部に造影効果を認め，後期相では腫瘤へのペルフルブタンの取り込みを認めた．炎症性偽腫瘍または炎症性筋線維芽細胞腫瘍の術前診断のもと，腹腔鏡下脾臓摘出術を施行した．腫瘤は病理組織学的にsclerosing angiomatoid nodular transformation （SANT）と診断された．SANTは脾の血管腫類似病変で，本邦では会議録を除き7症例が報告されているまれな疾患で症例の集積が必要と考えられ，本邦報告例の検討とともに報告する．

Sclerosing angiomatoid nodular transformation of the spleen: clinical and radiologic characteristics

Sclerosing angiomatoid nodular transformation (SANT) is a rare benign primary vascular lesion of the spleen. Although there have been many reports about the pathology of SANTs of the spleen, there have been no comprehensive descriptions of the imaging findings of SANTs of the spleen. To determine the clinical characteristics and imaging findings of SANTs of the spleen. We retrospectively evaluated seven patients with pathologically confirmed SANT, who underwent CT (n = 7), MRI (n = 4), ultrasonography (n = 4), and PET/CT (n = 3). Follow-up CT examinations were obtained in five patients. Clinical characteristics such as symptoms and concurrent disease were assessed. CT and MRI findings were evaluated by two radiologists, including the number, border, signal intensity, enhancement pattern, hemorrhage, and cystic change or necrosis. The longest diameter of each tumor was measured on CT. Echogenicity on ultrasonography and standardized uptake value on PET/CT were also evaluated. No specific symptom was associated with SANT. Two patients had a history of malignancy, one with cervical cancer and the other with early gastric cancer. Tumor growth was observed in four of five patients. On CT, all seven SANTs appeared as single, well-demarcated masses. CT showed a heterogeneous enhancement in seven patients. MRI showed centripetal progressive enhancement and absence of cystic change or necrosis in four patients, with three of these patients showing evidence of old hemorrhage. Ultrasonography showed heterogeneous hypoechoic mass in four patients. PET/CT showed increased standardized uptake value, ranging from 2.0 to 2.8, in three patients. SANT of the spleen is a single, well-demarcated solid mass without cystic change or necrosis. Increased FDG activity and tumor growth on follow-up imaging are common.

Sclerosing angiomatoid nodular transformation of the spleen (SANT): multimodality imaging appearance of five cases with radiology–pathology correlation

Sclerosing angiomatoid nodular transformation (SANT) is an extremely rare splenic lesion first reported in 2004, representing an unusual reaction of splenic red pulp to stromal inflammation or vascular injury. There are very few descriptions of the imaging appearance of SANT in the literature. We present five pathologically proven cases of SANT, with a description of the imaging appearance using multiple different modalities, as well as correlation with the histopathologic features of the lesion. While there are several imaging features of SANT which have been described in the literature, it is not routinely possible to make a prospective diagnosis based on the imaging features alone. Moreover, it may not be possible to exclude malignancy based on the imaging features, and splenectomy may be required in certain cases.

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

INTRODUCTIONSclerosing Angiomatoid Nodular Transformation of the spleen (SANT) is a rare benign vascular lesion of the spleen with extensive sclerosis and unknown etiology. PRESENTATION OF CASEWe 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. DISCUSSIONA 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. CONCLUSIONThe 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.

Laparoscopic splenectomy for splenic sclerosing angiomatoid nodular transformation: A case report

Introduction: Sclerosing angiomatoid nodular transformation (SANT) is a rare benign and proliferative vascular lesion arising within the red pulp of the spleen. The lesion is often identified incidentally on imaging and the diagnosis is confirmed on pathologic assessment of the resected spleen. The lesion can be cured by splenectomy. Until now, little has been published on the laparoscopic approach for treatment of SANT. Case Report: A 5­cm splenic tumor was discovered during a routine examination of an asymptomatic 38­ year­old man. We were initially suspicious of fibrous hamartoma, but could not rule out malignancy. Consequently, laparoscopic splenectomy was performed, and histopathological findings showed that the tumor was a sclerosing angiomatoid nodular transformation. Conclusion: We report a rare case of sclerosing angiomatoid nodular transformation, completely resected by laparoscopy. In such cases, laparoscopic splenectomy seems to be a feasible, safe, and effective treatment.

Sclerosing angiomatoid nodular transformation of the spleen treated by laparoscopic partial splenectomy.

The authors describe a case of sclerosing angiomatoid nodular transformation (SANT) of the spleen treated at the 2nd Department of Surgery, Jagiellonian University, Medical College. The patient was a 23-year-old woman. Clinically she presented with 2-year history of recurrent mild fever, diffuse joint pain, abdominal discomfort and iron deficiency anaemia of chronic disease. The laboratory tests revealed a non-characteristic chronic inflammatory response. A splenic solid lesion 9 cm in diameter was found on abdominal computed tomography. The patient underwent uneventful laparoscopic resection of the upper half of the splenic parenchyma. The resected tumour showed characteristic histological and immunophenotypical findings of SANT as previously described in the literature. In long-term follow-up, improvement of preoperative symptoms and abnormalities in the blood tests was documented.