Sonographic features of lymphoma of the major salivary glands diagnosed with ultrasound-guided core needle biopsy in Sjögren's syndrome

Abstract

Objective. To identify ultrasound (US) features of lymphomas (L) of major salivary glands (SGs) in primary Sjögren's syndrome (pSS) patients and to differentiate US pattern of L and non-L. Methods. Prospectively, from September 2019 to March 2021, 27 pSS-patients with clinical findings suspicious for L of the SGs underwent US evaluation followed by US-guided core-needle biopsy (CNB). For each patient, we assessed the OMERACT score, dichotomised (0/1 "lower", 2/3 "higher"), and we compared it between L-pSS and nonL-pSS groups. For focal lesions, echogenicity, inner appearance, shape, margins, presence of septa, vascularisation and posterior acoustic features were also assessed and compared between the two groups; we planned to consider as "suspicious" features more frequently associated with L. We expected to compare frequencies at which two or more "suspicious" features were simultaneously present between L-pSS and nonL-pSS. p<0.05 were considered statistically significant. Results. L-pSS showed more inhomogeneous glandular pattern (100% vs. 69.2% higher OMERACT; p=0.0407). For focal lesions, the "suspicious" features identified were: OMERACT grade 3, very hypoechoic, homogenous, oval shape, well-defined margins, presence of septa, colour-Doppler vascularisation, posterior acoustic enhancement. 6/8 and 7/8 simultaneous suspicious features were significantly higher among p=0.034 for 6/8 features; 77.8% vs. 14.3%, p=0.040 for 7/8 features). Conclusions. L of the major SGs in pSS was always associated with OMER-ACT scores 2 or 3 and presented with diffuse or focal patterns. For focal lesions, the association of more "suspicious" features made the diagnosis of L increasingly more likely. This information can help to improve planning of US-guided CNB.

Salivary Gland Ultrasound in Primary Sjögren’s Syndrome: Current and Future Perspectives

“… 66 In pSS patients with inhomogeneous glandular patterns, especially those with higher OMERACT scores (2 and 3), the presence of a newly diagnosed focal lesion is suggestive of lymphoma. 33 However, in this clinical scenario, other focal benign and malignant lesions typically found in the general population must first be ruled out ( Figure 4 ). 67 , 68 In the general population, focal lesions in SGs are rare.…”

“… 29 , 33 However, recent studies highlighted that NHL of major SGs can also present as well-defined focal abnormalities in SGs with no diffuse background lymphomatous infiltration. 19 , 33 It has been hypothesized that such areas may represent foci of actively expanding B-cell clones, 33 in keeping with the currently accepted model of lymphomagenesis in pSS. 66 Research has also demonstrated that focal NHL exhibits recurrent, distinctive sonographic features and tends to present as a very hypoechoic, oval, well-defined, homogenous lesion, with posterior acoustic enhancement, which frequently contains thin, hyperechoic septa and signs of hypervascularity on SGUS and color Doppler imaging.…”

“… 66 Research has also demonstrated that focal NHL exhibits recurrent, distinctive sonographic features and tends to present as a very hypoechoic, oval, well-defined, homogenous lesion, with posterior acoustic enhancement, which frequently contains thin, hyperechoic septa and signs of hypervascularity on SGUS and color Doppler imaging. 33 For these reasons, when NHL of major SGs is suspected in pSS, it is highly advisable to prioritize assessing the presence of focal lesions, as this could potentially aid the selection of the best target and guide the resulting biopsy. 33 …”

Sjögren syndrome: looking forward to the future

“…The SG ultrasonographic pattern might represent another predictor, due to the proven association with clinical, serological and histological features of lymphoproliferative risk and its ability to define the ESSDAI glandular domain in pSS, 21 , 108 , 124 , 126 , 185 as recently reported by Lorenzon et al 186 …”

Sjögren Syndrome: New Insights in the Pathogenesis and Role of Nuclear Medicine

“…Promising innovations are ultrasound elastography (with algorithms to evaluate quantitatively the tissue stiffness of the salivary gland tissue [ 50 ], the application of artificial intelligence to automatically score for biopsy purposes [ 51 ], and the ultrasound-guided core needle biopsy of major salivary glands which could represent an alternative to surgical biopsy [ 52 ].…”