Diagnostic experience of intravenous leiomyomatosis with emphasis on conventional ultrasonography imaging: a single-center study

Abstract

Objective: Intravenous leiomyomatosis (IVL) is a rare and aggressive tumor type that has the potential to extend into the inferior vena cava (IVC) and is susceptible to be misdiagnosed and neglected. Despite its clinical significance, there is a paucity of research that has focused on the specific manifestations of IVL on ultrasonography. Therefore, this study aims to systematically analyze the specific ultrasound features of IVL and augment its diagnostic accuracy.

Materials and method: Prospective inclusion was granted to patients admitted to our hospital between December 2016 and March 2021 for an IVC-occupying lesion. Multi-modal ultrasonography, encompassing gray-scale and color Doppler, was conducted. Lesions were categorized as IVL or non-IVL based on pathological or follow-up data. Two ultrasound sonographers with over 5 years of experience read and recorded ultrasound data for all lesions, which were subsequently comparatively analyzed to identify specific signs of IVL.

Results: A total of 284 patients diagnosed with IVC-occupying lesions were included in the study. The lesion types comprised of IVL (n=67, 23.6%), IVC thrombus (n=135, 47.5%), tumor thrombus of renal carcinoma involving the IVC (n=35, 12.4%), tumor thrombus of liver carcinoma involving the IVC (n=24, 8.5%), leiomyosarcoma of the IVC (n=14, 4.9%), and tumor thrombus of adrenocortical adenocarcinoma (n=9, 4.1%). The presence of "sieve hole" and "multi-track" signs was observed in 20 IVL lesions under the grey-scale modality, while both signs were absent in the non-IVL group (P<0.01). The study found no statistically significant differences in the presentation of "sieve hole" and "multi-track" signs under the grey-scale and color Doppler modalities in cases of intravascular lithotripsy (IVL) (P>0.05). Using these two signs as diagnostic criteria for IVL, the sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), miss rate, misdiagnosis rate, and accuracy were determined to be 29.9%, 100%, 100%, 82.2%, 70.1%, 0, and 83.5%, respectively (AUC ROC=0.649; 95%CI: 0.537-0.761).

Conclusion: IVL exhibits distinct ultrasound presentations, including "sieve hole" and "multi-track" signs, which demonstrate high specificity and accuracy as diagnostic indicators. Furthermore, these signs are corroborated by pathological evidence and effectively distinguish IVL from other lesions occupying the IVC.

Keywords: gynecological tumor; intravenous leiomyomatosis (IVL); rare disease; ultrasonic characteristics; ultrasonography.

Figure 1

Transverse section of IVL showed the “sieve hole sign” in gray scale mode and color Doppler mode in figure (A, B) respectively (red star). Longitudinal section of IVL showed the “multi-track sign” in gray scale mode and color Doppler mode in figure (C, D) respectively (red star). Figure (E) showed transverse section of IVL with “sieve hole sign” and figure (F) showed longitudinal section with “multi-track sign” in enhanced MRI (red star).

Figure 2

A descriptive diagram showed IVL with “sieve hole sign” in transverse section (A) and “multi-track sign” in longitudinal section (B).

Figure 3

ROC curve of IVL diagnosed by “sieve hole sign” and “multi-track sign”, AUC=0.649 (95% CI: 0.537-0.761).

Figure 4

A 51-year-old female patient with a surgical pathology result of IVL. Figure (A, B) showed the “sieve hole sign” under conventional ultrasound and enhanced CT respectively (red arrow). The cross section in figure (C) showed multiple circular cavities within the gross specimen lesion (red arrow), while figure (D) showed the presence of small cavity structures under the microscope (red arrow).

Figure 5

A 47-year-old female with a surgical pathology result of IVL. Figure (A) showed a conventional ultrasound grayscale image, with no “sieve hole sign” on the transverse section (red star). Transverse section of enhanced CT in figure (B) showed uneven enhancement of the lesion, presenting a small tiny “sieve hole sign”(red arrow). Figure (C) showed a fissure like appearance in the transverse section of the lesion (red arrow), while figure (D) showed the internal cavity structure of the lesion under a microscope (red star).