Sclerosing angiomatoid nodular transformation of the spleen: CT, MR, PET, and 99mTc-sulfur colloid SPECT CT findings with gross and histopathological correlation
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- Thacker, C., Korn, R., Millstine, J. et al. Abdom Imaging (2010) 35: 683. doi:10.1007/s00261-009-9584-x
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Sclerosing angiomatoid nodular transformation (SANT) is a benign, proliferative vascular lesion affecting the spleen. Few reports detailing the cross sectional and PET appearance of this lesion are available, and the lesion’s behavior with 99mTc-sulfur colloid scintigraphy is previously unreported. Sclerosing nodular transformation of the spleen shows increased tracer accumulation on positron emission tomography, and a central scar-like appearance with an enhancing capsule and radiating septae on CT and MR studies that reflects the gross and histopathological features of the lesion may be visible. An understanding of this pathological finding may allow prospective recognition of the sclerosing nodular transformation of the spleen on cross sectional imaging studies.
KeywordsSclerosing angiomatoid nodular transformation Spleen Proliferation Mass Vascular lesion Sulfur colloid Positron emission tomography
Splenic lesions are commonly incidentally encountered during abdominal cross sectional imaging studies. The majority of such lesions are benign. Occasionally solid, vascular splenic lesions may be identified and can prove difficult to characterize with non-invasive imaging. Awareness of the imaging appearance and differential diagnosis of rare vascular splenic lesions, and, in particular, recognition of certain imaging features that may suggest a specific diagnosis for these uncommon lesions, may facilitate proper patient management.
Report of case
The incidental detection of a splenic lesion is not an uncommon clinical occurrence, and differentiation of indolent from aggressive splenic lesions on imaging studies can occasionally be difficult. Fortunately, most cystic and solid lesions within the spleen in patients without known primary malignancy are benign, and neoplasms within the spleen are less common than benign processes and are often encountered in a known clinical context, commonly patients with lymphoma or metastatic disease.
Vascular splenic lesions include both benign and malignant etiologies, but most of these lesions are non-hematolymphoid tumors . Non-hematolymphoid tumors arise from the red pulp of the spleen and include hemangiomas, hamartomas, lymphangiomas, and SANT . Vascular splenic lesions with variable biological behavior include littoral cell angioma, hemangioendothelioma, and hemangiopericytoma, whereas angiosarcoma is a primary, frankly malignant, vascular splenic neoplasm .
SANT of the spleen is a recently recognized, rare, benign vascular splenic neoplasm of uncertain etiology, first described in 2004 as a distinct, non-neoplastic vascular splenic lesion by Martel et al. . Prior to 2004, isolated reports of splenic SANT were described under various other terms, including splenic hamartomas, multinodular hemangiomas, cord-capillary hemangioma, or splenic hemangioendotheliomas .
Clinical, pathological, and histopathological features of splenic SANT
Only a few reports have detailed the clinicopathological aspects of splenic SANT [3, 4, 5, 6]. The few reports detailing this lesion suggest that splenic SANT is most commonly encountered in middle-aged adults, with a mean age of presentation approximating 50 years [4, 7], although a fairly wide age range for presentation of 22–74 years has been noted . A female preponderance of cases is described, with a female-to-male ratio of 2:1 suggested in several sources [4, 7]. Most reports indicate that splenic SANT is clinically silent and is detected incidentally, usually during laparotomy or imaging studies for unrelated reasons.
At gross inspection, patients with splenic SANT often have a normal-sized or mildly enlarged spleen. The lesion itself usually appears as solitary, unencapsulated but circumscribed mass consisting of red or brown nodules of variable size. The histopathological and immunohistochemical findings of splenic SANT have been the subject of several reports [3, 4, 5, 6, 7, 8, 9, 10]. At low-power examination, splenic SANT is composed of multiple, variably sized, circumscribed and confluent angiomatoid nodules within a fibrosclerotic stroma containing hemosiderin-laden macrophages, myofibroblasts, lymphocytes, and plasma cells . The nodules have a slit-like, vascular morphology lined with endothelial cells, pericytes, and red blood cells. Minimal cellular atypia may be present within splenic SANT nodules but mitotic figures are rare. Immunohistochemical analysis of splenic SANT shows staining with various markers for splenic sinusoidal lining cells, capillary-like, and venule-like elements , including CD34, CD21, and CD8. CD68 staining is also typical in splenic SANT, whereas CD21 staining is characteristically absent.
Imaging features of splenic SANT (Table 1)
Sclerosing angiomatoid nodular transformation of the spleen: summary of cross-sectional imaging reports 2005–2009
Li et al. 
Hypodense, central calcification, progressive central enhancement
Lee et al. 
Lee et al. 
Two lesions, hypodense, central enhancement
↓ T1, ↓ T2, no enhancement
Karaosmanaglu et al. 
Centrally hypodense, peripheral enhancement extending centrally
Central ↑ T1, Peripherally ↑, and centrally ↓ T2, peripheral enhancement extending centrally
Gutziet et al. 
Isoechoic with hypoechoic rim. Peripheral enhancement filling centrally with “spoke-wheel” pattern
Hypodense, some central enhancement
Zeeb et al. 
36/female/left upper quadrant pain
Hypodense on unenhanced images and arterial and portal venous phase images with peripheral and septal enhancement. Delayed imaging isodense to spleen, with persistent hypodense stellate center
Thacker et al.
Iso- to mildly hypodense on unenhanced images and arterial and portal venous phase images with peripheral and septal enhancement. Delayed imaging isodense to spleen, with persistent hypodense stellate center
Predominantly ↓ T1 and T2 signal with small central foci of ↑ T1 and T2 signal. Iso- to mildly hypointense on unenhanced images and arterial and portal venous phase images with peripheral and septal enhancement. Delayed imaging is isointense to spleen, with persistent hypointense stellate center. Opposed-phase imaging may show presence of iron
The presence of signal changes on MR imaging suggesting the presence of iron initially supported the impression of extramedullary hematopoiesis as the etiology of the splenic lesion in this patient. However, iron deposition may occur in a number of conditions unrelated to extramedullary hematopoiesis, and it is likely that the hemorrhagic nature of splenic SANT accounts for this MR imaging appearance.
Only one prior published report of the FDG-PET behavior of splenic SANT is known . These authors  indicated that splenic SANT shows hypermetabolic activity on FDG-PET, simulating neoplasm; our lesion showed similar behavior. The abundance of cells, including hemosiderin-laden macrophages, myofibroblasts, lymphocytes, and plasma cells, may account for splenic SANT’s FDG avidity.
The 99mTc-sulfur colloid SPECT CT imaging findings of splenic SANT have not been previously reported. The absence of reticuloendothelial cells within the SANT lesion accounts for the lack of uptake of 99mTc-sulfur colloid.
Diagnosis of splenic SANT
Most published reports of SANT of the spleen have established the diagnosis following splenectomy, although one investigation has shown that the diagnosis of SANT may be made with percutaneous core biopsy . Nevertheless, percutaneous biopsy of the spleen must be undertaken with caution as a higher frequency of complication is noted with percutaneous biopsy of vascular splenic lesions . The gross pathological and radiological correlation of splenic SANT in this report as well as the published descriptions of the cross-sectional imaging findings of splenic SANT [7, 11, 12, 13] suggest that the diagnosis of SANT of the spleen may be specifically offered when contrast-enhanced CT or MR shows a centrally hypovascular lesion with peripheral enhancement progressively extending toward the center of the lesion through septae on delayed imaging- the so-called “spoke-wheel” appearance [11, 12]. However, it is likely that when a vascular splenic lesion is encountered, particularly when large and bulging the capsular surface of the spleen, concerns for malignancy and the potential for splenic rupture will render splenectomy the primary means by which splenic SANT will be diagnosed and treated.
SANT of the spleen is a recently described, non-neoplastic benign vascular lesion. Few cross-sectional imaging reports have detailed the radiological appearance of SANT of the spleen, but the literature suggests that a variably sized lesion, often large, with peripheral enhancement that progressively fills in toward the center of the lesion through radiating septae is a typical appearance on enhanced ultrasound, CT, and MR studies. Possibly due to the presence of various inflammatory cells within the lesion, FDG avidity is expected on FDG-PET studies of SANT of the spleen, whereas, due to the absence of reticuloendothelial elements, absence of 99mTc-sulfur colloid uptake is observed. The diagnosis of SANT of the spleen may be established by percutaneous biopsy, but, due to the potential for an increased rate of complications when vascular lesions of the spleen undergo percutaneous biopsy and concerns regarding the possibility of spontaneous rupture of a large vascular splenic lesion, splenectomy will likely be the means by which SANT of the spleen is diagnosed and managed.
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