Hepatosplenic sarcoidosis: contrast-enhanced ultrasound findings and implications for clinical practice

Abstract

Sarcoidosis is a complex granulomatous disease that affects virtually every organ and tissue, with a prevalence that varies significantly among the sites involved. The role of conventional imaging, such as computed tomography and magnetic resonance imaging, in the assessment of hepatosplenic sarcoidosis is well established by revealing organ enlargement, multiple discrete nodules, and lymphadenopathy. In this review, we aim to describe contrast-enhanced ultrasound (CEUS) findings in liver and spleen involvement by sarcoidosis, reporting evidence from the literature and cases from our experience, after a brief update on safety profile, cost-effectiveness, and clinical indications of this novel technique. Furthermore, we highlight potential advantages of CEUS in assessing hepatosplenic sarcoidosis that may be useful in the clinical practice.

Figure 1

A 51-year-old woman with diagnosis of pulmonary sarcoidosis, who presented with dyspepsia. (a) B-mode US showed diffuse liver hyperechogenicity suggestive of fatty liver disease and a hypoechoic lesion in the hepatic segment I (arrow). The lesion was in close contiguity with inferior vena cava and had a maximum size of 51 mm. Imaging findings were suggestive of focal fatty sparing, but histopathological examination revealed noncaseating granulomas, suggesting liver involvement by sarcoidosis. Spleen was normal. (b) Color Doppler US showed no flow inside the nodule (arrow). (c) After six months of steroid therapy, the lesion was significantly reduced (arrow).

Figure 2

A 24-year-old female with histopathological diagnosis of hepatic sarcoidosis that resembled advanced stage of cirrhosis on US. (a) The liver was almost completely subverted by multiple more diffuse and also more circumscribed hypoisoechoic nodules (numbers 1 to 4); the lesions did not demonstrate vascularity on Color Doppler US. (b) Contrast-enhanced US in the late phase showed almost isoenhancing lesions.

Figure 3

A 53-year-old male with history of pulmonary sarcoidosis. (a) B-mode US showed a rounded and hypoechoic lesion located in the lower pole of the spleen; the nodule did not show flow on Color Doppler US and had a maximum size of 1 cm (arrow, caliper 1). (b) Contrast-enhanced US confirmed the lesion and showed other progressively hypoenhancing nodules of few mm (in median 5 mm, arrowheads) that were not evident on conventional US. Histopathological examination of the spleen revealed infiltration by sarcoidosis.

Figure 4

A 64-year-old female with sarcoidosis. (a) B-mode US documented typical prominent perihepatic lymphadenopathy (maximum size of 3 cm, caliper). Contrast-enhanced US showed (b) homogenous enhancement during the arterial phase (arrow) and (c) prominent wash-out (caliper).

Figure 5

A 45-year-old woman with history of colon cancer, polycystic ovary syndrome, and migraine, who presented with fatigue, weight loss, and headache. No changes in bowel habits were reported. Physical examination revealed only laterocervical lymphadenopathy. (a) B-mode US documented splenomegaly, with parenchyma subverted by multiple and rounded hypoechoic lesions (arrows). The nodules had maximum size of 22 mm and showed no flow on Color Doppler US. Contrast-enhanced US documented (b) rim-like enhancement of the lesions in the arterial phase (7 seconds, arrows) and hypoenhancement in the parenchymal (c) (1 min 20 sec, arrows) and late phases. In view of the patient history, this pattern was first suggestive of malignancy. However, other organs were normal on second imaging, and histopathological examination revealed noncaseating granulomas, suggesting the diagnosis of splenic sarcoidosis (reprinted with permission from [10]).