Lymphangitis carcinomatosa: Difference between revisions
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==Overview== | ==Overview== | ||
'''Lymphangitis carcinomatosa''' is an inflammation of the [[lymph vessel]]s secondary to a [[malignancy]]. Lymphangitis carcinomatosa was first discovered by Gabriel Andral, a French pathologist, in 1829. The pathogenesis of lymphangitis carcinomatosa is characterized by the tumoral spread into the [[lymphatics]], following hematogenous seeding of the lungs. The pathogenesis is also characterized by retrograde spread into [[lymphatics]] from the [[mediastinal]] and [[hilar]] [[lymph nodes]] in the lungs. Common causes of lymphangitis carcinomatosa include [[breast cancer]] (most common), [[lung cancer]], [[Colorectal cancer|colon cancer]], [[stomach cancer]], [[prostate cancer]], [[cervical cancer]], and [[thyroid cancer]]. Lymphangitis carcinomatosa is a rare disease. The prevalence of lymphangitis carcinomatosa is approximately 0.03 per 100,000 individuals worldwide. Lymphangitis carcinomatosa is more commonly observed among patients aged between 40 to 49 years old. [[Computed tomography]] is the imaging modality of choice for lymphangitis carcinomatosa. On CT, characteristic findings of lymphangitis carcinomatosa include subpleural nodules, thickening on the interlobar fissures, [[pleural effusion]], and hilar and mediastinal nodal enlargement (40-50%). The mainstay therapy for lymphangitis carcinomatosa is systemic chemotherapy (chemotherapeutic regimen depends on the tumor histology). | '''Lymphangitis carcinomatosa''' is an [[inflammation]] of the [[lymph vessel]]s [[secondary]] to a [[malignancy]]. [[Lymphangitis]] carcinomatosa was first discovered by Gabriel Andral, a French [[Pathologists|pathologist]], in 1829. The [[pathogenesis]] of [[lymphangitis carcinomatosa]] is characterized by the [[tumoral]] [[Spread of the cancer|spread]] into the [[lymphatics]], following hematogenous seeding of the [[lungs]]. The [[pathogenesis]] is also characterized by [[Retrograde infection|retrograde]] [[Spread of the cancer|spread]] into [[lymphatics]] from the [[mediastinal]] and [[hilar]] [[lymph nodes]] in the lungs. Common [[causes]] of [[lymphangitis]] carcinomatosa include [[breast cancer]] (most common), [[lung cancer]], [[Colorectal cancer|colon cancer]], [[stomach cancer]], [[prostate cancer]], [[cervical cancer]], and [[thyroid cancer]]. [[Lymphangitis carcinomatosa]] is a [[rare]] [[disease]]. The [[prevalence]] of [[lymphangitis carcinomatosa]] is approximately 0.03 per 100,000 individuals worldwide. [[Lymphangitis carcinomatosa]] is more commonly observed among [[patients]] [[Age|aged]] between 40 to 49 years old. [[Computed tomography]] is the [[imaging]] modality of choice for [[lymphangitis carcinomatosa]]. On [[CT-scans|CT]], characteristic findings of [[lymphangitis carcinomatosa]] include subpleural [[nodules]], thickening on the interlobar [[fissures]], [[pleural effusion]], and [[hilar]] and [[mediastinal]] [[Lymph node|nodal]] enlargement (40-50%). The mainstay [[therapy]] for [[lymphangitis carcinomatosa]] is [[systemic]] [[chemotherapy]] ([[Chemotherapeutic agent|chemotherapeutic]] regimen depends on the [[tumor]] [[histology]]). | ||
==Historical Perspective== | ==Historical Perspective== | ||
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*The pathogenesis is also characterized by retrograde spread into [[lymphatics]] from the [[mediastinal]] and [[hilar]] [[lymph nodes]] in the lungs. | *The pathogenesis is also characterized by retrograde spread into [[lymphatics]] from the [[mediastinal]] and [[hilar]] [[lymph nodes]] in the lungs. | ||
*There are no specific genetic mutations associated with the development of lymphangitis carcinomatosa. | *There are no specific genetic mutations associated with the development of lymphangitis carcinomatosa. | ||
*On gross pathology, characteristic findings of lymphangitis carcinomatosa include:<ref name="lymph"> Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis Accesed on April 29, 2016</ref> | *On gross pathology, characteristic findings of lymphangitis carcinomatosa include:<ref name="lymph">Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis Accesed on April 29, 2016</ref> | ||
:*No remarkable findings | :*No remarkable findings | ||
*On microscopic histopathological analysis, characteristic findings of lymphangitis carcinomatosa include:<ref name="lymph"> Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis Accesed on April 29, 2016</ref> | *On microscopic histopathological analysis, characteristic findings of lymphangitis carcinomatosa include:<ref name="lymph">Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis Accesed on April 29, 2016</ref> | ||
:*Carcinoma in multiple the lymphatic channels | :*Carcinoma in multiple the lymphatic channels | ||
==Causes== | ==Causes== | ||
*Common causes of lymphangitis carcinomatosa include:<ref name="lymph"> Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis Accesed on April 29, 2016</ref> | *Common causes of lymphangitis carcinomatosa include:<ref name="lymph">Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis Accesed on April 29, 2016</ref> | ||
**[[Breast cancer]] (most common) | **[[Breast cancer]] (most common) | ||
**[[Lung cancer]] | **[[Lung cancer]] | ||
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==Epidemiology and Demographics== | ==Epidemiology and Demographics== | ||
*Lymphangitis carcinomatosa is a rare disease. | *Lymphangitis carcinomatosa is a rare disease. | ||
*The prevalence of lymphangitis carcinomatosa is approximately 0.03 per 100,000 individuals worldwide.<ref name="radio"> Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | *The prevalence of lymphangitis carcinomatosa is approximately 0.03 per 100,000 individuals worldwide.<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | ||
===Age=== | ===Age=== | ||
*Patients of all age groups may develop lymphangitis carcinomatosa. | *Patients of all age groups may develop lymphangitis carcinomatosa. | ||
*Lymphangitis carcinomatosa is more commonly observed among patients aged 40–49 years old.<ref name="radio"> Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | *Lymphangitis carcinomatosa is more commonly observed among patients aged 40–49 years old.<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | ||
*Lymphangitis carcinomatosa is more commonly observed among middle aged adults. | *Lymphangitis carcinomatosa is more commonly observed among middle aged adults. | ||
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==Risk Factors== | ==Risk Factors== | ||
*Common risk factors in the development of lymphangitis carcinomatosa include:<ref name="radio"> Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | *Common risk factors in the development of lymphangitis carcinomatosa include:<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | ||
:*Personal history of cancer | :*Personal history of cancer | ||
:*Preexistent malignant cancer | :*Preexistent malignant cancer | ||
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=== Symptoms === | === Symptoms === | ||
*Lymphangitis carcinomatosa is usually asymptomatic. | *Lymphangitis carcinomatosa is usually asymptomatic. | ||
*Symptoms of lymphangitis carcinomatosa may include the following:<ref name="radio"> Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | *Symptoms of lymphangitis carcinomatosa may include the following:<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | ||
:* [[Hemoptysis]] | :* [[Hemoptysis]] | ||
:* Chronic [[coughing]] | :* Chronic [[coughing]] | ||
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=== Physical Examination === | === Physical Examination === | ||
*Patients with lymphangitis carcinomatosa usually appear pale and malnourished. | *Patients with lymphangitis carcinomatosa usually appear pale and malnourished. | ||
*Physical examination may be remarkable for:<ref name="radio"> Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | *Physical examination may be remarkable for:<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | ||
'''Auscultation''' | '''Auscultation''' | ||
*Present [[pleural friction rub]] | *Present [[pleural friction rub]] | ||
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===Imaging Findings=== | ===Imaging Findings=== | ||
*Computed tomography is the imaging modality of choice for lymphangitis carcinomatosa.<ref name="radio"> Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | *Computed tomography is the imaging modality of choice for lymphangitis carcinomatosa.<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | ||
*The most important feature about CT scan is the detection of peripheral and central changes. | *The most important feature about CT scan is the detection of peripheral and central changes. | ||
*On CT, characteristic findings of lymphangitis carcinomatosa include:<ref name="radio"> Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | *On CT, characteristic findings of lymphangitis carcinomatosa include:<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | ||
:*Subpleural nodules, and thickening on the interlobar fissures | :*Subpleural nodules, and thickening on the interlobar fissures | ||
:*[[Pleural effusion]] | :*[[Pleural effusion]] | ||
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== Treatment == | == Treatment == | ||
=== Medical Therapy === | === Medical Therapy === | ||
*The mainstay therapy for lymphangitis carcinomatosa is systemic chemotherapy (chemotherapeutic regimen depends on the tumor histology).<ref name="radio"> Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | *The mainstay therapy for lymphangitis carcinomatosa is systemic chemotherapy (chemotherapeutic regimen depends on the tumor histology).<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016</ref> | ||
=== Surgery === | === Surgery === | ||
Revision as of 15:42, 21 May 2019
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Swathi Venkatesan, M.B.B.S.[2]
Synonyms and keywords: Lymphangitis carcinomatosis; Pulmonary lymphangitic carcinomatosis
Overview
Lymphangitis carcinomatosa is an inflammation of the lymph vessels secondary to a malignancy. Lymphangitis carcinomatosa was first discovered by Gabriel Andral, a French pathologist, in 1829. The pathogenesis of lymphangitis carcinomatosa is characterized by the tumoral spread into the lymphatics, following hematogenous seeding of the lungs. The pathogenesis is also characterized by retrograde spread into lymphatics from the mediastinal and hilar lymph nodes in the lungs. Common causes of lymphangitis carcinomatosa include breast cancer (most common), lung cancer, colon cancer, stomach cancer, prostate cancer, cervical cancer, and thyroid cancer. Lymphangitis carcinomatosa is a rare disease. The prevalence of lymphangitis carcinomatosa is approximately 0.03 per 100,000 individuals worldwide. Lymphangitis carcinomatosa is more commonly observed among patients aged between 40 to 49 years old. Computed tomography is the imaging modality of choice for lymphangitis carcinomatosa. On CT, characteristic findings of lymphangitis carcinomatosa include subpleural nodules, thickening on the interlobar fissures, pleural effusion, and hilar and mediastinal nodal enlargement (40-50%). The mainstay therapy for lymphangitis carcinomatosa is systemic chemotherapy (chemotherapeutic regimen depends on the tumor histology).
Historical Perspective
- Lymphangitis carcinomatosa was first discovered by Gabriel Andral, a French pathologist, in 1829.
Classification
- There is no classification for lymphangitis carcinomatosa.
Pathophysiology
- The pathogenesis of lymphangitis carcinomatosa is characterized by the tumoral spread into the lymphatics, following hematogenous seeding of the lungs.
- The pathogenesis is also characterized by retrograde spread into lymphatics from the mediastinal and hilar lymph nodes in the lungs.
- There are no specific genetic mutations associated with the development of lymphangitis carcinomatosa.
- On gross pathology, characteristic findings of lymphangitis carcinomatosa include:[1]
- No remarkable findings
- On microscopic histopathological analysis, characteristic findings of lymphangitis carcinomatosa include:[1]
- Carcinoma in multiple the lymphatic channels
Causes
- Common causes of lymphangitis carcinomatosa include:[1]
Differentiating Lymphangitis Carcinomatosa from Other Diseases
- Lymphangitis carcinomatosa must be differentiated from other diseases that cause thickened interlobular septae (on imaging finding), dyspnea, fatigue, and weight-loss, such as:
- Sarcoidosis
- Viral pneumonia
- Pulmonary edema
- Radiation pneumonitis
- Lymphocytic interstitial pneumonitis
Epidemiology and Demographics
- Lymphangitis carcinomatosa is a rare disease.
- The prevalence of lymphangitis carcinomatosa is approximately 0.03 per 100,000 individuals worldwide.[2]
Age
- Patients of all age groups may develop lymphangitis carcinomatosa.
- Lymphangitis carcinomatosa is more commonly observed among patients aged 40–49 years old.[2]
- Lymphangitis carcinomatosa is more commonly observed among middle aged adults.
Gender
- Females are more commonly affected with lymphangitis carcinomatosa than males.
Race
- There is no racial predilection for lymphangitis carcinomatosa.
Risk Factors
- Common risk factors in the development of lymphangitis carcinomatosa include:[2]
- Personal history of cancer
- Preexistent malignant cancer
Natural History, Complications and Prognosis
- The majority of patients with lymphangitis carcinomatosa are symptomatic at the time of diagnosis.
- Early clinical features include dyspnea, fatigue, and weight-loss.
- If left untreated, patients with lymphangitis carcinomatosa may progress to develop acute respiratory failure.
- Common complications of lymphangitis carcinomatosa include:
- Prognosis is generally poor, and the mean survival rate of patients after diagnosis of lymphangitis carcinomatosa is approximately 6 months.
Diagnosis
Symptoms
- Lymphangitis carcinomatosa is usually asymptomatic.
- Symptoms of lymphangitis carcinomatosa may include the following:[2]
- Hemoptysis
- Chronic coughing
- Chest pain
- Loss of appetite
- Fatigue
Physical Examination
- Patients with lymphangitis carcinomatosa usually appear pale and malnourished.
- Physical examination may be remarkable for:[2]
Auscultation
- Present pleural friction rub
- Present egophony
- Crackling or bubbling noises
- Present whispered pectoriloquy
- Decreased/absent breath sounds
Percussion
- Dull percussion
- Reduced chest expansion
Laboratory Findings
- There are no specific laboratory findings associated with lymphangitis carcinomatosa.
Imaging Findings
- Computed tomography is the imaging modality of choice for lymphangitis carcinomatosa.[2]
- The most important feature about CT scan is the detection of peripheral and central changes.
- On CT, characteristic findings of lymphangitis carcinomatosa include:[2]
- Subpleural nodules, and thickening on the interlobar fissures
- Pleural effusion
- Hilar and mediastinal nodal enlargement (40-50%)
- Relatively little destruction of overall lung architecture
- Involvement of the peripheral (interlobular septa) and central lymphatic system
- Distribution of changes is variable, but most are asymmetric and patchy
- Usually bilateral (may be unilateral especially in cases of lung and breast cancer)
Treatment
Medical Therapy
- The mainstay therapy for lymphangitis carcinomatosa is systemic chemotherapy (chemotherapeutic regimen depends on the tumor histology).[2]
Surgery
- Surgery is not recommended for patients with lymphangitis carcinomatosa.
Prevention
- There are no primary preventive measures available for lymphangitis carcinomatosa.
- Once diagnosed and successfully treated, patients with lymphangitis carcinomatosa are followed-up periodically.
- Follow-up testing may include respiratory function tests and disease progression monitorization.
References
- ↑ 1.0 1.1 1.2 Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis Accesed on April 29, 2016
- ↑ 2.0 2.1 2.2 2.3 2.4 2.5 2.6 2.7 Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis Accessed on April 28,2016