Pulmonary lymphangitis sarcomatosis: a rare cause of severe progressive dyspnoea
- 1 Respiratory, Saint James's Hospital, Dublin, Ireland
- 2 Saint James's Hospital, Dublin, Ireland
- Correspondence to Dr David Quigley; quigledj@tcd.ie
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.
Case presentation
A man in his 70s with no medical history was admitted with right-sided pelvic and thigh pain with mild right lower limb weakness of 3 weeks duration. On examination, there was mild tenderness over his right iliac crest with reduced range of movement in his right lower limb without neurological signs. Respiratory rate was normal, oxygen saturation was 97% on room air but crackles were audible in his upper zones bilaterally despite having no respiratory symptoms. Renal profile, B-natriuretic peptide level and white cell count were normal. C reactive protein was elevated at 57.1 mg/L. lactate dehydrogenase (LDH) was normal. Three successive COVID-19 PCR nasopharyngeal swabs in addition to interferon gamma release assay (IGRA) were negative.
Investigations
Renal profile, B-natriuretic peptide level and white cell count were normal. C reactive protein was elevated at 57.1 mg/L. LDH was normal. Three successive COVID-19 PCR nasopharyngeal swabs in addition to IGRA were negative. Chest X-ray, and subsequent CT thorax revealed bilateral lung infiltrates (figures 1–3) with images discussed at multi-disciplinary meeting (MDT). Arterial blood gas result showed a borderline normal pa02 at rest of 12.8 with a pco2 of 4.7 and PH of 7.42. CT abdomen and pelvis, and subsequent MRI (figures 4 and 5), revealed bilateral psoas masses. CT-guided core biopsies were taken from the right psoas lesion on day 5 of admission.
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.
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.
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).
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).
This coronal image reveals an ill-defined enhancing mass is seen at the inferomedial aspect of the right iliopsoas (white arrow).
Cytological analysis of bronchoalveolar lavage (BAL) showed scant malignant cells of smooth muscle origin (figures 6 and 7). Bronchoalveolar lavage samples were negative for micro-organisms including bacteria, Pneumocystis pneumonia (PCP), tuberculosis (TB) and fungi. Transbronchial biopsy was not performed given the severity of the patient’s respiratory compromise.
This coronal image reveals an ill-defined enhancing mass is seen at the inferomedial aspect of the right iliopsoas (white arrow).
Desmin immunostain (Ventana DE-R-1clone) ×100.
Differential diagnosis
There was numerous differential diagnoses in this case. Infectious causes of concern included viral (not limited to COVID-19), bacterial, mycobacterial and fungal. Rapidly progressive forms of interstitial lung disease can present with a similar clinical picture. Acute decompensated heart failure is always considered in a patient with dyspnoea and bilateral infiltrates. Lymphangitis carcinomatosis was determined most likely based on clinical, radiological and biochemical investigations at this time.
Outcome and follow-up
Unfortunately, the patient’s respiratory status deteriorated further. High flow oxygen therapy settings were fio2 90% with flow rates of 60 L/minute. Repeat arterial blood gas at this time indicated significant hypoxaemia with a pa02 of 7.6 on room air. Respiratory alkalosis with a PH 7.52 and pco2 of 3.1 was consistent with the patients level of tachypnoea. A trial of continuous positive airway pressure was trialled for a number of hours but was poorly tolerated by the patient. Histological confirmation of malignancy was provisionally made at day 10 of admission. Intubation was deemed inappropriate, palliative measures were undertaken, and he passed away.
Discussion
Pulmonary lymphangitis carcinomatosis occurs most commonly in the setting of breast, lung and gastric adenocarcinoma.1 Dyspnoea and dry cough are the most common initial symptoms but may be absent. Lymphangitic spread of sarcomas (pulmonary lymphangitic sarcomatosis) is extremely rare, as sarcomas tend to disseminate haematogenously and only 2%–5% metastasize to lymph nodes.2
The presence of bilateral lung infiltrates (figures 1–3) in the absence of infection, is strongly suggestive of lymphangitis carcinomatosis.1 3–5 CT chest was discussed at our pulmonary/radiology/oncology pathology and cardiothoracic surgeon multi centre MDT. Our radiology colleagues supported the diagnosis. We acknowledge, however, that other lung pathology could not be definitively out ruled without histological confirmation. Organising pneumonia, diffuse alveolar damage and acute interstitial fibrosis may all present with a similar radiological appearance but the findings in this case were not typical for any of these but instead were felt morphologically to be most in keeping with lymphangitis. MRI findings of bilateral soft tissue masses were highly suspicious for malignancy (figures 4 and 5).
Histology from the psoas lesion showed a fascicular spindle cell tumour comprising atypical spindle cells with hyperchromatism (figure 6). Mitoses numbered 2 per 10 HPF. Tumour cells were strongly and diffusely positive for the muscle markers desmin and smooth muscle actin (figure 7) and were negative for the skeletal muscle marker MYF4, in addition to S100, caldesmon, CD117, DOG-1, melan A, HMB45 and cytokeratin (MNF116). Features were in keeping with leiomyosarcoma. The appearance of a radiologically similar contralateral lesion was felt to indicate metastatic leiomyosarcoma although whether the right or left lesion was the primary remains uncertain.
Other authors have identified Pulmonary lymphangitis Sarcamatosis in cutaneous angiosarcoma,6 epitheliod haemangioendothelioma,2 osteosarcoma,7 Kaposi’s sarcoma8 and Ewings sarcoma.9 This, to our knowledge, is the first described case of radiological pulmonary lymphangitis associated with leiomyosarcoma.
Over the following 7 days, the patient deteriorated. He was treated with high dose hydrocortisone 100 mg intravenously four times a day, piperacillin–tazobactam antibiotics 4.5 g three times a day and high flow oxygen.
Learning points
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Extensive causes of dyspnoea and bilateral infiltrates—radiological imaging is critical to diagnosis.
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Interdiscipline discussion regarding diagnostic uncertainties can prevent invasive measures, that is, intubation.
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During the COVID-19 pandemic, it is important to recognise the close mimics and out rule the rarer causes.
Ethics statements
Patient consent for publication
Footnotes
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Contributors DQ was involved in the management of the case and bronchscopy and discussion of histopathology. ROD was involved in the management of the case and in the bronchoscopy. CM was involved in the radiological discussion of this case.
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Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
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Case reports provide a valuable learning resource for the scientific community and can indicate areas of interest for future research. They should not be used in isolation to guide treatment choices or public health policy.
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Competing interests None declared.
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Provenance and peer review Not commissioned; externally peer reviewed.
- © BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.
References
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