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Related Terms: Lymphangitis, lymphedema, Lymphangitic carcinomatosa, Carcinomatosa, Nebulization Chemotherapy, Squamous cell lung cancer, Transbronchial lung biopsy, broncoscopy, transbronchial biopsy or thoracoscopic wedge resection.
This is rapidly progressive and is a very rare “cancer” where there is an extensive lymphatic permeation by tumor cells. The cancer has surrounding fibrosis and produces visable cords. The condition is most commonly seensecondary to adenocarcinomas such as: breast cancer, gastrointestinal tract adenocarcinomas, bronchogenic adenocarcinoma, prostate cancer, ovarian adenocarcinoma. The most commonly affected organs are reported to be breasts, lungs, colon and stomach.
Some patients experience dyspnoea, others abnormal pulmonary functions tests.
Because it does involve the lymphatics (lymphatic dilatation), pleural effusions are common.
Chest radiography is the preferred examination for the diagnoses of pulmonary metastases. Other radiology tests mayh include a CT Scan, PET scan or the use of an ultrasound. For diagnosing early stages of lymphangitis carcinomatosa, a high resolution CT (HRCT) may be used. A broncoscopy may also be a diagnostic tool.
Others useful diagnostic tools include a transbronchial biopsy or thoracoscopic wedge resection. While it is useful, transthoracic needle aspiratons yield (85-90%) in evaluating pulmonary nodules, the yield is lower with lymphangitis tumour spread in lymphangitis carcinomatosa.
Diagnostic radiography may have these specific findings: thickening of the peribronchovascular interstitium surrounding vessels and bronchi in the parahilar lung; interlobular septal thickening (Fig.1 a,b) and subpleural interstitial thickening that is smooth, or nodular; thickening of the peribronchovascular axial interstitium in the centrilobular regions; and a preservation of normal lung architecture at the lobular level, despite the presence of these findings.
Hilar lymphadenopathy is visible on CT in only 50% of patients with lymphangitic carcinoma. Lymph node enlargement can be symmetrical or asymmetrical. pleural effusion may also be present. (3)
CT findings may include: Irregular, nodular, and/or smooth interlobular septal thickening; Thickening of the fissures from infiltration of lymphatics in the subpleural space; Peribronchovascular thickening which predominates over interlobular septal thickening in a minority of patients; Polygonal arcades or polygons with prominence of the centrilobular bronchovascular bundle in association with interlobular septal thickening (50%); Mediastinal and/or hilar lymphadenopathy (30-50%); Pleural effusions (30-50%); Laminar pleural effusions are common; Nodular opacities.
The differential diagnosis of LC includes sarcoidosis and other chronic interstitial lung diseases such as silicosis, coal worker’s pneumoconiosis, extrinsic allergic alveolitis (hypersensitivity pneumonitis), and cryptogenic fibrosing alveolitis, as well as other neoplasms such as lymphoma and Kaposi sarcoma. (2)
Treatment may include the standard chemotherapy - both systemic and nebulization. Nebulization chemotherpy involves the inhalation of 5-Flourouracil (5-FU).
The patient may require treatment for the pleural effusions as well.
For the lymphedema, the patient should be referred to a certified lymphedema therapist for an evaluation and subsequent manual decongestive therapy.
One study indicated that 50% of patients die within three months after their first respiratory symptoms, but lymphangitis carcinomatosa in other organs may have good remission with prompt hormonal therapy. (1)
Lymphangitis Carcinomatosis as a Complication of Malignant Pleural Effusion April 2011
Lymphangitis carcinomatosis (LC) is a rare and often fatal form of pulmonary metastases. Author describes this as a case of left sided malignant pleural effusion with unknown primary which after follow up of two months developed lymphangitis carcinomatosis on right side. Despite extensive search of literature author could not find any information to explain the occurrence of lymphangitis carcinomatosis as in this case where the primary could not be traced. There has been literature on the association of lymphangitis carcinomatosis with pleural effusion, but there has been no literature reporting the occurrence of lymphangitis carcinomatosis after or as a complication of malignant pleural effusion.
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Case report : Nebulization chemotherapy for lymphangitis carcinomatosa 1999
S Ramkumar, Donald J Fernandes; Department of Radiotherapy and Oncology, Kasturba Medical College, Manipal-576719, Karnataka, India
Keywords: Lymphangitis Carcinomatosa, Nebulization Chemotherapy
Lymphangitis carcinomatosa is a rare, aggressive and often fatal form of pulmonary metastases . Although treatment modalities used for lymphangitis vary, no form of treatment has consistently had any impact on survival.
We report on a primary breast cancer patient with lymphangitis carcinomatosa who was treated using a unique technique: nebulization chemotherapy, in addition to systemic chemotherapy and supportive care in order to achieve rapid, sustainable improved response.
Nebulization chemotherapy involves inhalation of ultrasonically aerosolized particles of a chemotherapeutic drug 5-Fluorouracil (5-FU), using an ultrasonic nebulizer. This technique was first used in inoperable lung cancer  and allows direct and prolonged action of high concentration 5- FU on tumor tissue. Since 5-FU is directly incorporated and metabolized in the respiratory tract, it is possible to deliver a localized high concentration of the drug to the tumor tissue with minimal systemic absorption, resulting in higher tumor response, with minimal systemic side effects.
FDG PET/CT in assessment of pulmonary lymphangitic carcinomatosis. Jan. 2010
Prakash P, Kalra MK, Sharma A, Shepard JA, Digumarthy SR.
Source Department of Radiology, Massachusetts General Hospital and Harvard Medical School, 55 Fruit St., 202 Founders, Boston MA 02114, USA.
The purpose of this study was to assess the role of PET/CT in the diagnosis of pulmonary lymphangitic carcinomatosis.
MATERIALS AND METHODS: Integrated PET/CT images of 35 patients (15 men, 20 women; mean age, 64.5 years) with pulmonary lymphangitic carcinomatosis confirmed at follow-up chest CT or histopathologic examination were analyzed retrospectively. Standardized uptake value based on body weight and the initial injected activity was measured in the affected lung, the normal lung, and the mediastinal blood pool. Two radiologists independently assessed abnormal PET activity in the lungs. Both radiologists reviewed the CT images to determine the presence, size, location, and extent of pulmonary lymphangitic carcinomatosis. The data were analyzed to determine the sensitivity and specificity of PET for pulmonary lymphangitic carcinomatosis.
RESULTS: Among the 35 patients with pulmonary lymphangitic carcinomatosis, 17 (49%), 13 (37%), and five (14%) patients had diffuse, focal, and bilateral pulmonary lymphangitic carcinomatosis, respectively. Thirty of the 35 patients had nodular septal thickening as the chief CT finding of pulmonary lymphangitic carcinomatosis. Subjective assessment showed a visually identifiable increase in uptake in the region of pulmonary lymphangitic carcinomatosis in 30 of the 35 patients (86%). Four of the other five patients had focal pulmonary lymphangitic carcinomatosis, and one patient had diffuse pulmonary lymphangitic carcinomatosis of the right lung. The specificity of PET/CT for pulmonary lymphangitic carcinomatosis was 100%, and the sensitivity was 86%. The mean standardized uptake value in the region of pulmonary lymphangitic carcinomatosis (1.37+/-0.64) was significantly greater than that in normal lung (0.51+/-0.29) (p<0.0001). The standardized uptake ratio of mediastinal blood pool to lymphangitic lung was 1.26+/-0.45, and that of blood pool to normal lung was 3.78+/-1.37.
CONCLUSION: FDG PET/CT has high specificity in the detection of pulmonary lymphangitic carcinomatosis. Focal pulmonary lymphangitic carcinomatosis close to a primary malignant tumor, however, can be missed at PET.
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Asymptomatic Lymphangitis Carcinomatosis due to Squamous Cell Lung Carcinoma 2005
P.R. Gupta, Narayani Joshi, R.C. Meena and M. Ali Departments of Chest Diseases and TB and Pathology, S.M.S. Medical College, Jaipur (Rajasthan), India
Lymphangitis carcinomatosa most commonly due to primary malignancy originating in the breast, stomach, pleura and prostate but may also originate from the lung itself. It is clinically characterised by progressing dyspnoea with or without cough even at an early stage. We report the case of a patient with squamous cell lung cancer presenting with asymptomatic lymphangitis carcinomatosa.
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Lymphangitis carcinomatosa in thin section computed tomography.
Pasławski M, Krzyzanowski K, Złomaniec J.
2nd Department of Radiology, Skubiszewski Medical University of Lublin.
High resolution computed tomography is a diagnostic method of choice in the evaluation of lung parenchyma. HRCT enables the evaluation of small interstitial changes, invisible on plain chest radiographs, and their assessment at the level of the lung lobule. The aim of the study was the assessment of typical findings in HRCT in lymphangitis carcinomatosa, enabling differential diagnosis. Material comprises a group of 18 patients with lymphangitic spread of carcinoma, in whom HRCT examination was performed. Nodular thickening of the peribronchovascular interstitium and interlobular septa are typical in lymphangitic spread of carcinoma. Smooth peribronchovascular and septal thickenings are typical in sarcoidosis, and are only seen in some patients in the lymphangitic spread of carcinoma. In lymphangitis carcinomatosa lung architecture remains unchanged, which allows differentiating from sarcoidosis.
Lymphangitic Carcinomatosis Imaging
A Case of Prostate Carcinoma Discovered With Pulmonary Lymphangitis Carcinomatosa April 2011
Pulmonary lymphangitic carcinomatosis as a primary manifestation of colon cancer in a young adult 2008
Lymphangitis carcinomatosa as an unusual presentation of renal cell carcinoma: a case report Jan. 2008
Pulmonary lymphangitic carcinomatosis (PLC): spectrum of FDG-PET findings. Nov 2006
Generalised lymphatic carcinosis (“lymphangitis carcinomatosa”) of the lungs Jan 2005
Respiratory failure due to pulmonary lymphangitis carcinomatosis. Mar 1993
Lymphangitis carcinomatosa as an unusual presentation of renal cell carcinoma: a case report 1993
Radiological Appearances of Lymphangitis Carcinomatosa of the Lung 1964