Pulmonary lymphangitis sarcomatosis: a rare cause of severe progressive dyspnoea

Abstract

Pulmonary lymphangitis carcinomatosis is a complication of malignancy with a poor prognosis. We describe an unusual case in which it caused ventilatory failure and unfortunately death in a previously well male in his 70s. Abnormal chest imaging led to a wide differential diagnosis with Bronchoscopy confirming malignant cells. MRI of his pelvis and biopsy was done diagnosis of metastatic leiomyosarcoma, a particularly aggressive malignancy. Sarcoma-related lymphangitis carcinomatosis is rarely described in the literature and this is the first case to our knowledge of its association with leiomyosarcoma.

Keywords: lung cancer (oncology); radiology; respiratory system.

Figure 1

The most striking feature is diffuse, irregular, nodular interlobular septal thickening (black arrows), which outlines the secondary pulmonary nodule. This is considered a hallmark CT finding in LC. Bilateral pleural effusions, another common finding in LC, are demonstrated (black asterisks). LC, lymphangitis carcinomatosis.

Figure 2

Selected axial image demonstrating widespread bilateral pulmonary nodules (black chevrons), another finding typical of LC. bilateral pleural effusions again demonstrate (black asterisks). Ground glass opacification which was visualised in a scattered distribution throughout the lungs (black figure X) was thought to most likely represent superimposed infection and is unrelated to the LC.

Figure 3

Coronal section through the thorax once again demonstrates the findings of LC: interlobular septal thickening (black arrows) and pulmonary nodules (black chevrons), as well as a superimposed area of infection, demonstrated by ground glass opacification (black figure X).

Figure 4

Contrast-enhanced MRI of the pelvis was performed. This axial image shows a 5 cm peripherally enhancing mass in the posterior left psoas muscle (white arrow). A second focal 1.5 cm peripherally enhancing lesion is seen posterior to the right psoas at the L3 level (black arrow).

Figure 5

This coronal image reveals an ill-defined enhancing mass is seen at the inferomedial aspect of the right iliopsoas (white arrow).

Figure 6

This coronal image reveals an ill-defined enhancing mass is seen at the inferomedial aspect of the right iliopsoas (white arrow).

Figure 7

Desmin immunostain (Ventana DE-R-1clone) ×100.