Lymphangitic Carcinomatosis: Some HRCT Findings
Akochi Agunwamba, Harvard Medical School Year III and Gillian Lieberman, MD
Lymphangitic Carcinomatosis: Overview
Also known as “lymphangiatic spread of tumors”, “lymphangitis carcinomatosa”, or “lymphangiosis carcinomatosa”
LC refers to the infiltration of pulmonary parenchymal lymphatic channels by tumor cells
Usually spread to lungs is hematogenous, while spread within lungs is lymphangitic
Unilateral (primary lung cancer and breast carcinoma) involvement is less common than bilateral (Stomach, Pancreas, Prostate, Cervical, Thyroid, Colon, or Adenocarcinoma from an unknown site) ~80% of the mettastases are adenocarcinomas
Herold CJ, et al. “Lung metastases”. Eur. Radiol. 6, 596-606 (1996)
Menu of Radiological Tests
Chest X-ray (CXR) – ~50% of lymphangitic carcinomatosis patients appear normal on CXR
High Resolution Computed Tomography (HRCT) – Currently appears to offer the best combination of cost and sensitivity. 8-74% better than CXR for lymphangitic carcinomatosis
Positron emission tomography with radio-labeled [18F]-2-fluorodeoxy-D-glucose (PET-FDG) – Very expensive. Sensitivity appears to be identical to HRCT though experience with PET-FDG in diagnosing lymphangitic carcinomatosis is limited
Khan AN, et al. “Lymphangitic Carcinomatosis”. eMedicine - http://www.emedicine.com/Radio/topic416.htm. Accessed 7/19/2008
HRCT findings in Lymphangitic Carcinomatosis
Irregular, smooth or nodular thickening of interlobular septa
Irregular and nodular thickening of peribronchovascular sheets
Thickening of centrilobular structures
Peripherally located wedge shaped densities representing edema from lymphatic destruction
Pleural disease
Correct first diagnosis 40% of the time
Herold CJ, et al. “Lung metastases”. Eur. Radiol. 6, 596-606 (1996); Webb, RW et al. Thoracic Imaging: Pulmonary and Cardiovascular Radiology (2005)
Lymphangitic Carcinomatosis : Clinical Symptoms
The clinical symptoms often precede radiographic abnormalities and include: – Breathlessness – Dry cough – Hemoptysis (unusual unless there is primary lung cancer)
Screening for lymphangitic carcinomatosis occurs in the context of prior malignancy
British Thoracic Society and Standards of Care Committee, Thorax 1999;54;S1-S28
Anatomy of the Secondary Lobule and Acinus
Webb, W. R. Radiology 2006;239:322-338 Copyright ©Radiological Society of North America, 2006
Radiograph: 1-mm lung slice taken from peripheral lower lobe – showing secondary lobule
S Æ Lobules are marginated by thin interlobular septa
V Æ Pulmonary vein branches B Æ Bronchioles A Æ Centrilobular Pulmonary arteries
Webb, W. R. Radiology 2006;239:322-338 Copyright ©Radiological Society of North America, 2006
Pathology: Interlobular septal and peribronchovascular thickening in lymphangitic carcinomatosis
On cut lung surface, small white arrows point to thickened interlobular septa and large arrow points to thickened peribronchovascular interstitium Copyright ©Radiological Society of North America, 2006
H&E specimen (10x). Black arrows point to nodules of tumor in interlobular septa & centrilobular peribronchovascular region.
Webb, W. R. Radiology 2006;239:322-338
Our Patient: 35 y/o woman with 2 lung nodules. Rest of history withheld for now
nodule Below level of lesion At level of lesion NOTE: Inspecting level above and below suspected level of lesion helps distinguish between nodule and vessel.
Above level of lesion
Axial C+ CT of the chest Images from PACS BIDMC
Courtesy Dr. Ferris
Differential for solitary nodule
Malignancy (adenocarcinoma - 40%, squamous cell carcinoma - 20%, large cell carcinoma - 15%, bronchoalveolar carcinoma - 10%, solitary metastases)
Benign neoplasms (hamartomas, lipomas, and fibromas)
Vascular lesions - Arteriovenous malformation
Infectious granulomas - Tuberculosis, atypical mycobacterial infection, histoplasmosis, coccidioidomycosis, and blastomycosis
Other infections - Aspergilloma, ascaris, dirofilariasis, echinococcal cyst, and bacterial abscess
Noninfectious granulomas - Rheumatoid arthritis, Wegener granulomatosis, and sarcoidosis
Developmental lesions - Bronchogenic cyst
1.
Other conditions - Hematoma, bronchiolitis obliterans-organizing pneumonia, pseudotumor, pulmonary infarction, amyloidoma, rounded atelectasis, and mucoid impaction
Manocha S, “Solitary Pulmonary Nodule.” eMedicine – accessed 7/21/2008
Our Patient: At 8 mth follow up, presents with tachycardia and dyspnea Enlarged hilum Filling defects Peripheral opacities (probably cellular or fluid infiltrates)
Reformatted HR CTA Axial View of the chest Images from PACS BIDMC
Courtesy Dr. Ferris
Our Patient: Also had diffuse pulmonary disease Enlarged Hilum Thickened Septa Thickened centrilobar structures Peripheral opacities (probably fluid or cellular infiltrates)
Axial C+ CT of the chest Images PACS BIDMC
Courtesy Dr. Ferris
Our Patient: Diffuse interstitial disease on reconstructed Sagittal C+ HRCT of the Chest
Diffuse nodular and septal infiltrates Major fissure
Serial Slices from Reconstructed Sagittal C+ HRCT of the chest PACS BIDMC
Courtesy Dr. Ferris
Our Patient: Interstitial infiltrates on CXR and HRCT
Left Lateral CXR
AP Frontal CXR
Scapula SVC Porta catheter Mediastinal enlargement
Axial C- HRCT
Increased interstitial markings
Images from PACS BIDMC
Courtesy Dr. Ferris
Differential for septal and centrilobar thickening
Smooth interlobar septal thickening:
– pulmonary edema, hemorrhage, or veno-occlusive disease; Lymphangitic carcinomatosis; lymphangiomatosis; amyloidosis, pneumonia, alveolar proteinosis
Nodular interlobar septal thickening:
– Lymphangitic carcinomatosis; lymphoproliferative disease (e.g. lymphocytic interstitial pneumonia); sarcoidosis; silicosis and coal workers pneumoconiosis; amyloidosis
Centrilobular nodules due to perilymphatic disease:
– Lymphangitic carcinomatosis; sarcoidosis; silicosis, coal workers pneumoconiosis; lymphocytic interstitial pneumonia
Ikezoe J, et al. AJR 1995;165:49-52. Webb, W. R. Radiology 2006;239:322-338
Our Patient: Complete History
The patient is s/p right colectomy for poorly differentiated signet cell mucinous carcinoma of the terminal ileum; b/l salpingo-oopherectomies for mets. Mets to hemidiaphragm, bladder, small bowel, and pelvic side wall also observed. She is currently undergoing chemo. This combined with the CT findings makes the suspicion for metastasis to the lung and for lymphangitic carcinomatosis very high
Prognosis for Lymphangitic carcinomatosis
Usually very poor – survival in months In a small 8yr study of 10 pts treated with surgical resection of primary, chemo, and/or radiation tx, initially: – – – –
Pulmonary Sxs regressed in 6 Progressed in 2 Unchanged in 2 Median survival post diagnosis was 13 mths (range: 1130mths)
Ikezoe J, et al. “Pulmonary Lymphangitic Carcinomatosis: Chronicity of Radiographic Findings in Long-Term Survivors”. AJR 1995; 165:49-52.
Conclusions
Lymphangitic carcinomatosis spreads hematogenously to the lungs and then invades the lymphatic vessels The accumulation of tumor cells causes thickening of the secondary lobule interstitium HRCT is currently the modality of choice for screening lymphangitic carcinomatosis patients The diagnosis of lymphangitic carcinomatosis requires a clinical context that includes malignancy. The prognosis for lymphangitic carcinomatosis is poor
References
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Acknowledgements
Dr. Clare Horken Dr. Gethin Williams Dr. Diana Litmanovich Dr. Diana Ferris Dr. Gillian Lieberman Ms. Maria Levantakis
