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 [2]. Non-hematolymphoid tumors arise from the red pulp of the spleen and include hemangiomas, hamartomas, lymphangiomas, and SANT [2]. Vascular splenic lesions with variable biological behavior include littoral cell angioma, hemangioendothelioma, and hemangiopericytoma, whereas angiosarcoma is a primary, frankly malignant, vascular splenic neoplasm [2].
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. [3]. 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 [4].
Clinical, pathological, and histopathological features of splenic SANT
Only a few reports have detailed the clinicopathological aspects of splenic SANT [3–6]. 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 [4]. 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 [3–10]. 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 [4]. 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 [4], 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)
Little is known of the typical cross-sectional imaging features of splenic SANT as fewer than 30 cases have been reported in the medical literature [7, 11–13], and most published reports have concentrated on the pathological aspects of this lesion. The published reports detailing the cross sectional imaging appearance of splenic SANT are summarized in Table 1. Li et al. [6] were the first to describe the CT appearance of SANT. These investigators indicated that CT showed a lesion with a small focus of central calcification, initially hypodense on portal phase imaging, that became progressively homogeneous and approaching the attenuation of surrounding enlarged spleen in the late portal venous phase [6]. Subsequent reports by other investigators have repeatedly described a lesion that shows a comparatively hypovascular center with an enhancing rim and radiating vascularized tissue penetrating from the periphery toward the center of the lesion; this morphology has been seen on contrast-enhanced ultrasound [11], CT [7, 12], and MR examinations. When mutiphasic imaging is performed, splenic SANT lesions show progressive central enhancement with delayed imaging, thought to be the result of contrast penetrating the center of the lesion from the vascular rim. This morphological pattern has been referred to as a “spoke-wheel” pattern [11, 12]. In those reports detailing radiological and pathological correlation for splenic SANT lesions, this “spoke-wheel” morphology corresponds with a central, stellate fibrous stroma with fibrous septa separating angiomatoid nodules [7, 11–14].
Table 1 Sclerosing angiomatoid nodular transformation of the spleen: summary of cross-sectional imaging reports 2005–2009
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 [13]. These authors [13] 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 [11]. 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 [15]. 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, 11–13] 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.