A 13-year-old boy was admitted with abdominal pain. On physical examination, the spleen was palpated under the costal arch. There was no other abnormal finding detected at examination. All the laboratory parameters were within normal limits.
In abdominal ultrasound (USG) (Figure 1), a lesion with well-defined borders near the lower pole of the spleen, which was isoechoic relative to the spleen was seen. Minimal peripheral and central vascularizations were observed in the lesion at doppler USG (Figure 2). An encapsulated 110*90*85 mm lesion, which was hypointense on T1 weighted and heteregeneous hyperintense on T2 weighted images (Figure 3), was detected in abdominal Magnetic resonance imaging (MRI). The lesion had a progressive persistent contrast enhancement on postcontrast images (Figure 4). There were few linear hyperintense foci alining from periphery to the centre of the lesion on T2 (Figure 3b, 3d) weighted images and there was a central millimetric hypointense focus in the lesion on T2 weighted images (Figure 3b, 3d). Also, there was a diffusion restriction at the periphery of the lesion in Diffusion-weighted imaging (DWI) (Figure 5 a, 5b).
Littoral cell angioma (LCA) is a primary vascular tumour of the spleen. The tumour originates from the red pulp of the splenic sinuses [1,2]. LCA was first described at the beginning of 1990. Although there is no age predilection, most of the LCA cases reported in the literature were adults and few pediatric cases have been reported in the literature [1,3,4].
The symptoms are related to hypersplenisms such as anaemia, thrombocytopenia and splenomegaly. Incidental findings like abdominal pain and fever can be the only presenting symptoms of patients with LCA . LCAs are primarily benign tumours, but may rarely show malignant transformation .
Although USG, Computed Tomography (CT) and MRI are used in the diagnosis of the lesion, all of them exhibit some nonspecific features [2,7]. Radiologically, the most common form of LCA is multiple nodules but can also be solitary . The appearance of the lesion at USG is variable and can be seen as isoechoic, hypoechoic and hyperechoic . Peripheral and central vascularity is usually observed at colour Doppler USG examination. Our lesion was isoechoic relative to the spleen at USG. The most common CT findings of LCA are splenomegaly and numerous nodular lesions in the spleen . These lesions are generally hypodense and have a size ranging from 0.2 to 6 cm. The lesion in our case was different from the general literature, which was presented as a large solitary mass. Contrast-enhanced sequences have shown increased enhancement of the lesion in the portal venous phase . In MRI, lesions typically appear mildly hypointense on T1-weighted images and hyperintense on T2-weighted images. Bhatt et al. stated that the accumulation of hemosiderin in the lesion may help in the diagnosis of the signal properties of the T1 and T2 sequences in MRI. Therefore, they mentioned the superiority of MRI in diagnosis . Our lesion was hypointense on T1 weighted and heterogeneously hyperintense on T2 weighted images and had a central hypointense millimetric focus on T2-weighted sequences. The linear hyperintense foci alining from periphery to the centre of the lesion on T2 weighted images were thought to be the reflections of the histopathologic characteristics of the lesion such as anastomosing vascular channels of different sizes and focal papillary fronds extending into these channels.
The histopathologic diagnosis of our lesion was ‘’littoral cell angioma of the spleen’’ after splenectomy. There was no residual mass or relapse in the control MRI at the postoperative first year.
Although LCAs are generally seen as multiple millimetric lesions, they may appear as a single, large lesion as in our case. It should be considered in differential diagnosis among the lesions of the spleen by radiologists with radiological examinations, especially MRI.
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