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Malignant Pleural Effusion

Neoplastic Pleural Effusion

Malignant pleural effusion refers to an excess of pleural fluid caused by pleural neoplasms, either primary tumors or metastases, that interfere with the equilibrium between pleural fluid formation and reabsorption.


Presentation

MPE is usually associated with a severe increase of pleural fluid and many patients carry several liters of such liquid in their thoracic cavities. This amount of pleural fluid interferes with lung expansion and causes dyspnea and dull chest pain [7]. Of note, in case of unilateral MPE, a mediastinal shift may occur and cause compression of the contralateral lung. Therefore, breathing difficulties are not necessarily less severe in these patients.

Large amounts of pleural fluid dampen breathing sounds that are heard during auscultation and result in dullness to percussion.

Because MPE is generally diagnosed in patients suffering from metastasizing cancer, they often present with malaise, loss of appetite, weight loss or cachexia, anemia, leukocytosis and other symptoms considered part of the paraneoplastic syndrome.

Depending on the primary tumor and tumor staging, additional symptoms may be present. They may range from hoarseness, chronic cough and hemoptysis in case of bronchogenic carcinoma to fever, lymphadenopathy and recurrent infections in patients suffering from lymphoproliferative disorders.

Mediastinal Lymphadenopathy
  • 3 LC SQ Right 45 Ischemic heart diease, Atrial fibrillation Extensive mediastinal lymphadenopathies 4 * LC LC Right 73 Extensive mediastinal lymphadenopathies Enlargement of right hilar lymph nodes 5 * LC LC Both 57 Tamponade by massive pericardial effusion[kjim.org]
  • Both of these patients had extensive supraclavicular and mediastinal lymphadenopathy with distortion of the airways and bilateral effusions suggestive of lymphoma.[doi.org]
  • lymphadenopathy, or effusion secondary to pulmonary embolism.[dovepress.com]
Recurrent Pleural Effusion
  • Unfortunately, he died for recurrent pleural effusion and pulmonary complication 6 months later. Identifying the origin of malignant pleural effusion is important to provide treatment guidance.[ncbi.nlm.nih.gov]
  • Twenty-one patients, 12 with normal pleural fluid pH and 9 with low pleural fluid pH, were treated with tube thoracostomy and intrapleural tetracycline for symptomatic, recurrent pleural effusions.[ncbi.nlm.nih.gov]
  • Intervention: Twenty-one patients, 12 with normal pleural fluid pH and 9 with low pleural fluid pH, were treated with tube thoracostomy and intrapleural tetracycline for symptomatic, recurrent pleural effusions.[doi.org]
  • Recurrent pleural effusion: who benefits from a tunneled pleural catheter? Thorac Cardiovasc Surg 2009;57:42-6. Crossref 18. Al-Halfawy A, Light R.[hkmj.org]
  • This type of indwelling pleural drain is recommended in particular for absent lung expansion, recurrent pleural effusion, and compromised overall condition ( 17 , e9 ).[aerzteblatt.de]
Hyperthermia
  • We assessed the benefit of simple intrapleural hyperthermia (SIH) during thoracoscopic exploration for MPE.[ncbi.nlm.nih.gov]
Hypoxemia
  • […] of reversible diseases, and pleuroscopy may be contraindicated in situations such as highly loculated PF, inability of a lung to collapse due to an underlying disease, and existence of cardiovascular instability, pulmonary hypertension, or untreated hypoxemia[thoracicmedicine.org]
Pleural Effusion
  • BACKGROUND: Lung adenocarcinoma can easily cause malignant pleural effusion which was difficult to discriminate from benign pleural effusion. Now there was no biomarker with high sensitivity and specificity for the malignant pleural effusion.[ncbi.nlm.nih.gov]
  • Clinical factors predicting the diagnosis of malignant pleural effusions are symptoms lasting more than 1 month and the absence of fever. This is needed to confirm the presence of a pleural effusion.[en.wikipedia.org]
  • BACKGROUND: Malignant pleural effusions are one of the leading causes of exudative pleural effusions.[ncbi.nlm.nih.gov]
  • However, there is still no very effective treatment to control malignant pleural effusion. Here we report that malignant pleural effusion in one patient was completely relieved for 15 months by the anti-tuberculosis therapy.[ncbi.nlm.nih.gov]
  • Convert to ICD-10-CM : 511.81 converts directly to: 2015/16 ICD-10-CM J91.0 Malignant pleural effusion Approximate Synonyms Neoplastic pleural effusion Pleural effusion due to malignancy Clinical Information A collection of fluid in the pleural cavity[icd9data.com]
Dyspnea
  • A 73-year-old man presented with dyspnea, right-sided pleural effusion, and bilateral pulmonary infiltrates.[ncbi.nlm.nih.gov]
  • As a result, the patient's dyspnea was relieved, and her ECOG performance status improved from 4 to 2. However, the thoracotomy tube was not removed due to subsequent iatrogenic pneumothorax.[ncbi.nlm.nih.gov]
  • The dyspnea symptoms disappeared in 90 % of the patients in the fibrinolytic group and in 55 % of the patients in the control group (P 0.03). Recurrence rate was 11 % in fibrinolytic group and 45 % in control group (P 0.07).[ncbi.nlm.nih.gov]
  • Therefore, MPE strongly interferes with respiration and causes severe dyspnea. Most MPE patients also claim chest pain.[symptoma.com]
  • A 66-year-old woman was diagnosed as having pulmonary adenocarcinoma with malignant pleural effusion following investigations for cough and dyspnea. After drainage of the effusion she received combination chemotherapy with CDDP and VDS.[ncbi.nlm.nih.gov]
Pleural Disorder
  • BACKGROUND: Malignant pleural effusion (MPE) represents a very common cause of pleural exudates, and is one of the most challenging pleural disorders to manage.[ncbi.nlm.nih.gov]
  • Pleural Disorders Your pleura is a large, thin sheet of tissue that wraps around the outside of your lungs and lines the inside of your chest cavity. Between the layers of the pleura is a very thin space.[icdlist.com]
Mediastinal Disease
  • Weissberg et al. 86 performed medical thoracoscopy in 45 patients with lung cancer and a pleural effusion, and found pleural invasion in 37, mediastinal disease in three, and no metastatic disease in five (11%) and therefore, no contraindication to resection[doi.org]
Amnesia
  • The use of sedation may be helpful to allay such fears and induce amnesia. The level of sedation should be appropriate to relieve anxiety but sufficient to maintain patient interaction.[doi.org]

Workup

Anamnestic data, particularly in patients with a medical history of malignancies, should prompt the suspicion of MPE in patients presenting with dyspnea and diminished breathing sounds.

Quantities of pleural fluid that exceed 250 ml are generally well visible on plain radiographs obtained in lateral decubitus position. Even smaller amounts may be detected applying sonography [8]. The latter technique may also guide the aspiration of a fluid sample by thoracocentesis. This sample should then be examined chemically and cytologically. MPE is an exudative type of pleural effusion and thus, each µl contains more than 30 µg of protein and significantly more than 1,000 cells. Its specific weight is > 1018. Cytologic analysis may reveal important hints as to the underlying cancerous process. If a precise diagnosis cannot be made, a pleural biopsy may be required and should be obtained under sonographic guidance or via thoracoscopy [9]. Of note, severe pleural effusions may be caused by a variety of other pathologies that should be considered as possible differential diagnoses:

Some of these conditions may be caused by a malignant neoplasm and in these cases, the patient should be diagnosed with paramalignant pleural effusion instead of MPE [10]. Some patients may present both MPE and paramalignant pleural effusion.

Although radiography and/or sonography may be helpful in assessing the overall condition of the thoracic cavity, magnetic resonance imaging and computed tomography scans are usually applied to detect pulmonary, cardiac and pleural pathologies as well as neoplasms located in extrathoracic organs.

The result of diagnostic imaging should be used to prepare treatment, i.e., to evaluate the severity, extent and localization of pleural effusion.

Mediastinal Shift
  • A patient presented to the emergency department with a malignant pleural effusion associated with shortness of breath, and radiographic evidence of mediastinal shift and hypotension.[ncbi.nlm.nih.gov]
  • There may not be a mediastinal shift if the mediastinum is fixed secondary to a large lung mass or lymphadenopathy. A decubitus film may show layering of the fluid.[clevelandclinicmeded.com]
  • However, it is indicated when endobronchial lesions are suspected because of haemoptysis, atelectasis, or large effusions without contralateral mediastinal shift.[doi.org]
  • Of note, in case of unilateral MPE, a mediastinal shift may occur and cause compression of the contralateral lung. Therefore, breathing difficulties are not necessarily less severe in these patients.[symptoma.com]
  • Only symptomatic patients having massive and recurrent collection with mediastinal shift to opposite side responds best to pleurodesis. Successful pleurodesis requires apposition of the visceral and parietal pleura.[lungindia.com]
Bilateral Pulmonary Infiltrate
  • A 73-year-old man presented with dyspnea, right-sided pleural effusion, and bilateral pulmonary infiltrates.[ncbi.nlm.nih.gov]
Wuchereria Bancrofti
  • Pleural fluid examination revealed malignant cells, along with microfilaria of Wuchereria bancrofti.[ncbi.nlm.nih.gov]
Pleural Effusion
  • BACKGROUND: Lung adenocarcinoma can easily cause malignant pleural effusion which was difficult to discriminate from benign pleural effusion. Now there was no biomarker with high sensitivity and specificity for the malignant pleural effusion.[ncbi.nlm.nih.gov]
  • Clinical factors predicting the diagnosis of malignant pleural effusions are symptoms lasting more than 1 month and the absence of fever. This is needed to confirm the presence of a pleural effusion.[en.wikipedia.org]
  • BACKGROUND: Malignant pleural effusions are one of the leading causes of exudative pleural effusions.[ncbi.nlm.nih.gov]
  • However, there is still no very effective treatment to control malignant pleural effusion. Here we report that malignant pleural effusion in one patient was completely relieved for 15 months by the anti-tuberculosis therapy.[ncbi.nlm.nih.gov]
  • Convert to ICD-10-CM : 511.81 converts directly to: 2015/16 ICD-10-CM J91.0 Malignant pleural effusion Approximate Synonyms Neoplastic pleural effusion Pleural effusion due to malignancy Clinical Information A collection of fluid in the pleural cavity[icd9data.com]

Treatment

In general, treatment for MPE is palliative. Removal of fluid may be realized by means of thoracocentesis, thoracostomy or implantation of an indwelling pleural catheter. The latter options allow for continuous drainage of accumulating pleural fluid, while thoracocentesis is an acute measure that may have to be repeated if reaccumulation of fluid is not prevented effectively.

Reduction of the pleural space by means of pleurodesis may be required to avoid renewed pleural effusion. In order for pleural adhesion to occur, excess pleural fluid needs to be drained previously by any of the aforementioned methods. Otherwise, the accumulated fluid in the pleural space may impede contact of visceral and parietal pleura. Distinct methods are available: Instillation of talc or other mediators of sterile inflammation causes adhesion of visceral and parietal pleura in what is known as chemical pleurodesis. On the other hand, surgical or mechanical pleurodesis requires roughening of the pleura, i.e., mechanical irritation to induce an inflammatory reaction.

Prognosis

The average life expectancy of people diagnosed with MPE is six months and the majority of patients does not survive for more than a year [7]. Because MPE is associated with malignancies, often with metastasizing tumors, morbidity and mortality do not only result from respiratory insufficiency due to pleural effusion but also from the underlying disease.

Etiology

MPE results from the presence of neoplastic cells within the pleural space. Tumor cells may reach the pleura via the lymphatic system - which is indeed the most common way of metastatic spread into the pleura -, via blood vessels or by direct infiltration from adjacent tissues.

Carcinoma of the respiratory tract, namely lung adenocarcinoma and bronchogenic carcinoma, is most frequently diagnosed in MPE patients [3]. Breast carcinoma and distinct types of lymphoproliferative disease, both Hodgkin's disease and non-Hodgkin's lymphoma, tend to metastasize into the pleura, too. According to current estimates, mesothelioma accounts for less than 10% of all MPE cases. In some patients diagnosed with MPE, no primary tumor can be identified.

Epidemiology

MPE is a common complication of advanced malignancy and it has been estimated that 10-20% of all patients who die from cancer present some degree of MPE. Thus, the annual incidence might approximate 5 per 10,000 inhabitants.

The risk for malignancies and consequently the risk for MPE increase with age. However, the disease may also be diagnosed in young patients [4].

Sex distribution
Age distribution

Pathophysiology

The entirety of the mesothelial lining of the thoracic cavity is designated pleura and is composed of a visceral layer that mainly covers the lungs and a parietal layer that covers the thoracic wall. There is virtually no room between both layers and a healthy adult disposes of about 15 ml of pleural fluid that greatly facilitate lung movement.

Both visceral and parietal pleura produce up to 10 liters of pleural fluid daily, whereby this fluid is essentially an ultrafiltrate of capillary blood [5]. Lymph capillaries, principally those of the parietal pleura, reabsorb that liquid. Under physiological conditions, filtration and reabsorption are in equilibrium, a state that avoids accumulation of excess pleural fluid that would interfere with lung expansion. This equilibrium may be altered by a variety of factors not necessarily related with malignant neoplasms. An increased hydrostatic pressure, possibly caused by cardiac disorders, a reduced oncotic pressure, maybe related to hypoalbuminemia, and an elevated capillary permeability due to vascular pathologies may augment pleural fluid formation. Depending on the severity of these conditions, lymphatic capillaries may not be able to completely reabsorb excess fluid. With regards to the lungs, atelectasis may diminish the intrathoracic space occupied by the lungs, may reduce intrathoracic pressure and increase the pull on capillaries. Additionally, pleural effusion may result from limitations in lymphatic reabsorption itself. In this scenario, even normal filtration may lead to an accumulation of fluid within the pleural space. The lymphatic system may be disturbed at the level of pleural capillaries, larger vessels or even the thoracic duct.

Malignancies may alter the above described equilibrium of forces by means of altering the function of any component of the system. For instance, pleural tumors may directly interfere with capillary permeability and lymph capillary function, thus increasing pleural fluid formation and reducing reabsorption [6]. They may also interrupt lymph flow at more distal points, likely by local mass effects. Of note, chemotherapeutic or radiation cancer therapy may induce tissue fibrosis and excess connective tissue may affect reabsorption of pleural fluid and subsequent lymph drainage in a very similar manner.

Any one of these pathogenetic mechanisms or a combination thereof may lead to an accumulation of several liters of pleural fluid within the pleural space. This great amount of liquid strongly reduces the space at the lungs' disposal and thus causes dyspnea and possibly fatal respiratory failure. Additionally, venous return to the heart is largely restricted by the increased intrathoracic pressure and this condition may eventually cause heart failure.

Prevention

As per definition, MPE is associated with malignant neoplasms. Thus, any measure that decreases the individual risk of cancer may also be considered as a preventive measure for MPE. In this context, avoidance of exposure to carcinogens like cigarette smoke and asbestos, reduction of exposure to radiation and preventive medical examinations may be mentioned as examples.

Summary

Malignant pleural effusion (MPE) refers to a cancer-related accumulation of excess fluid in the pleural space [1]. It is a rather common complication of advanced cancer and may cause the amount of pleural fluid to multiply from physiological 15 ml to various liters. Therefore, MPE strongly interferes with respiration and causes severe dyspnea. Most MPE patients also claim chest pain. Because of the severity of the underlying disease and MPE, prognosis is generally poor and the average life expectancy of patients diagnosed with this disease is about half a year.

Few cases of MPE result from primary pleural tumors, i.e., from mesothelioma. Commonly, malignant neoplasms of the respiratory tract, of breast, gastrointestinal or urogenital tract metastasize into the pleura. Lymphoproliferative disorders are also frequently associated with pleural metastases. Some thoracic tumors, mainly those originating from the respiratory tract and the breast, may also directly infiltrate the mesothelial lining of the thoracic cavity. However, virtually every neoplasm may affect the pleura and cause MPE [2].

Treatment is palliative and primarily consists in tube thoracostomy or implantation of tunneled pleural catheters and pleurodesis. These procedures aim at reducing renewed accumulation of pleural fluid, dyspnea and discomfort, and may thus augment life quality.

Of note, MPE needs to be distinguished from paramalignant pleural effusion, which is generally caused by other pathologies. The latter should, however, be considered as differential diagnoses. Please refer to chapter Workup for details.

Patient Information

Pleura is the name of the inner lining of the thoracic cavity, i.e., the pleura coats lungs and inner chest wall. Between the lung-coating visceral pleura and the chest wall-lining parietal pleura, there is a very tiny room that is filled with a few milliliters of pleural fluid and it's called the pleural space. This fluid serves as a lubricant and allows for lung movement. Under physiological conditions, formation and reabsorption of pleural fluid are in a tightly regulated equilibrium. If that balance is disturbed and more pleural fluid is produced or less liquid is absorbed, the pleural space fills with fluid, possibly with several liters of it. This may happen if there are tumor cells in pleura and pleural space. This condition is called malignant pleural effusion (MPE).

Causes

Virtually every malignant tumor may metastasize into the pleura. Neoplasms growing within the thoracic cavity - lung cancer, for instance - may also infiltrate the pleural space directly. In some cases, a malignant tumor may directly originate from the pleura. This type of cancer is known as mesothelioma.

Lung adenocarcinoma, bronchogenic carcinoma, breast carcinoma and lymphoproliferative disorders like Hodgkin's disease and non-Hodgkin's lymphoma are most commonly related with MPE.

Symptoms

Fluid accumulation within the pleural space interferes with lung expansion and complicates breathing. Thus, MPE generally manifests in form of breathing difficulties. Chest pain may also be claimed.

Additional symptoms are mainly caused by the underlying malignancy.

Diagnosis

If a patient presents with symptoms characteristic for pleural effusion, this condition is diagnosed by sonography or plain radiography. A fluid sample will need to be obtained in order to identify the source of pleural effusion. If tumor cells are found within that sample, the patient may be diagnosed with MPE.

Further workup will aim at assessing the overall condition of the patient and consists in identification of the primary tumor and localization of possible metastasis.

Treatment

Therapy of MPE is generally palliative. Removal of the accumulated fluid may improve life quality by relieving breathing difficulties. Thoracocentesis, thoracostomy and implantation of a pleural catheter are possible therapeutic approaches. Some methods allow for continuous drainage of pleural fluid and thus avoid reaccumulation of great amounts of liquid. Pleurodesis, i.e., adhesion of visceral and parietal pleura, is an alternative treatment option. Pleurodesis reduces the pleural space and thus leaves no room for pleural fluid to reaccumulate.

References

Article

  1. Egan AM, McPhillips D, Sarkar S, Breen DP. Malignant pleural effusion. Qjm. 2014; 107(3):179-184.
  2. Tomoda C, Ogimi Y, Saito F, et al. Outcome and characteristics of patients with malignant pleural effusion from differentiated thyroid carcinoma. Endocr J. 2016; 63(3):257-261.
  3. Wu SG, Yu CJ, Tsai MF, et al. Survival of lung adenocarcinoma patients with malignant pleural effusion. Eur Respir J. 2013; 41(6):1409-1418.
  4. Afghani R, Hajimohammadi A, Azarhoush R, Kazemi-Nejad V, Yari B, Rezapour Esfahani M. Massive malignant pleural effusion due to lung adenocarcinoma in 13-year-old boy. Asian Cardiovasc Thorac Ann. 2016; 24(4):389-392.
  5. Zarogoulidis K, Zarogoulidis P, Darwiche K, et al. Malignant pleural effusion and algorithm management. J Thorac Dis. 2013; 5 Suppl 4:S413-419.
  6. Damianovich M, Hout Siloni G, Barshack I, et al. Structural basis for hyperpermeability of tumor vessels in advanced lung adenocarcinoma complicated by pleural effusion. Clin Lung Cancer. 2013; 14(6):688-698.
  7. Ried M, Hofmann HS. The treatment of pleural carcinosis with malignant pleural effusion. Dtsch Arztebl Int. 2013; 110(18):313-318.
  8. Yousefifard M, Baikpour M, Ghelichkhani P, et al. Screening Performance Characteristic of Ultrasonography and Radiography in Detection of Pleural Effusion; a Meta-Analysis. Emerg (Tehran). 2016; 4(1):1-10.
  9. Rahman NM, Gleeson FV. Image-guided pleural biopsy. Curr Opin Pulm Med. 2008; 14(4):331-336.
  10. Na MJ. Diagnostic tools of pleural effusion. Tuberc Respir Dis (Seoul). 2014; 76(5):199-210.

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Last updated: 2019-07-11 20:26