Lymphangitis carcinomatosa: Difference between revisions

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{{SI}}                                                                 
{{SI}}                                                                 
{{CMG}} {{AE}} {{MV}}
{{CMG}}; {{Swathi}} {{S.G.}}
   
   
{{SK}} Lymphangitis carcinomatosis
{{SK}} Lymphangitis carcinomatosis; Pulmonary lymphangitic carcinomatosis
   
   
==Overview==
==Overview==


'''Lymphangitis carcinomatosa''' is inflammation of the [[lymph vessel]]s secondary to a [[malignancy]]. Breast, lung, stomach, pancreas, and prostate cancers are the most common tumors that result in lymphangitis.
[[Lymphangitis|Lymphangitis carcinomatosa]] also known as carcinomatous [[lymphangitis]], 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 [[Lung|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==
*Lymphangitis carcinomatosa was first discovered by Gabriel Andral, a French pathologist, in 1829.
*[[Lymphangitis]] carcinomatosa was first discovered by Gabriel Andral, a distinguished French [[pathologist]] and professor at the University of Paris in 1829.<ref name="Doyle2018">{{cite journal|last1=Doyle|first1=L|title=Gabriel Andral (1797-1876) and the First Reports of Lymphangitis Carcinomatosa|journal=Journal of the Royal Society of Medicine|volume=82|issue=8|year=2018|pages=491–493|issn=0141-0768|doi=10.1177/014107688908200814}}</ref>
*Andral's [[case report]] on [[postmortem]] findings of [[lymphangitis]] carcinomatosa followed after a [[case report]] on a [[Women|woman]] who had died from [[cancer]] of the [[uterus]]; there had been extensive [[Spread of the cancer|spread]] of the [[cancer]] in the [[pelvis]], [[mesentery]] and [[thoracic duct]]-there was no mention of [[pulmonary]] or [[pleural]] involvement.<ref name="Doyle20182">{{cite journal|last1=Doyle|first1=L|title=Gabriel Andral (1797-1876) and the First Reports of Lymphangitis Carcinomatosa|journal=Journal of the Royal Society of Medicine|volume=82|issue=8|year=2018|pages=491–493|issn=0141-0768|doi=10.1177/014107688908200814}}</ref>
*Andral is remembered for his pioneer investigations of [[blood]] [[chemistry]].
*Gabriel Andral is considered to be the founder of [[scientific]] [[hematology]], and is credited with its integration into [[clinical]] and [[Analytical chemistry|analytical]] [[medicine]].


==Classification==
==Classification==
*Lymphangitis carcinomatosa may be classified according to [classification method] into [number] subtypes/groups:
*There is no established system for the classification of lymphangitis carcinomatosa.<ref name="pmid31245208">{{cite journal |vauthors=Aslam HM, Zhi C, Nadeem M, Arsalan M, Wallach SL |title=A Case of Rapidly Deteriorating Lymphangitic Carcinomatosis in a Patient with Stage IV Pancreatic Cancer |journal=Cureus |volume=11 |issue=4 |pages=e4421 |date=April 2019 |pmid=31245208 |pmc=6559437 |doi=10.7759/cureus.4421 |url=}}</ref>
:*[group1]
 
:*[group2]
:*[group3]
*Other variants of lymphangitis carcinomatosa include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
==Pathophysiology==
==Pathophysiology==
*The pathogenesis of lymphangitis carcinomatosa is characterized by the tumoral spread into the lymphatics, following hematogenous seeding of the lungs.
*It is understood that lymphangitis carcinomatosa is the result of the [[tumoral]] [[Spread of the cancer|spread]] into the [[lymphatics]], following [[Hematogen|hematogenous]] seeding of the [[lungs]].<ref name="BiswasSriram2015">{{cite journal|last1=Biswas|first1=Abhishek|last2=Sriram|first2=Peruvemba S.|title=Getting the Whole Picture: Lymphangitic Carcinomatosis|journal=The American Journal of Medicine|volume=128|issue=8|year=2015|pages=837–840|issn=00029343|doi=10.1016/j.amjmed.2015.04.007}}</ref>
*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 of the cancer|spread]] into [[lymphatics]] from the [[mediastinal]] and [[hilar]] [[lymph nodes]] in the [[lungs]].<ref name="Beattie1956">{{cite journal|last1=Beattie|first1=J.W.|title=Lymphangitis carcinomatosa|journal=British Journal of Tuberculosis and Diseases of the Chest|volume=50|issue=2|year=1956|pages=120–129|issn=03660869|doi=10.1016/S0366-0869(56)80046-4}}</ref>
*There are no specific genetic mutations associated with the development of lymphangitis carcinomatosa.  
*Both the peripheral [[lymphatics]] coursing in the interlobular [[septa]] and beneath the [[pleura]], and the central [[lymphatics]] coursing in the bronchovascular [[interstitium]] are involved.<ref name="urlLymphangitic carcinomatosis | Radiology Reference Article | Radiopaedia.org">{{cite web |url=https://radiopaedia.org/articles/lymphangitic-carcinomatosis?iframe=true&lang=us |title=Lymphangitic carcinomatosis &#124; Radiology Reference Article &#124; Radiopaedia.org |format= |work= |accessdate=}}</ref>
*On gross pathology, characteristic findings of lymphangitis carcinomatosa, include:  
*There are no specific [[genetic]] [[mutations]] associated with the [[development]] of lymphangitis carcinomatosa.<ref name="MoubaxWuyts2012">{{cite journal|last1=Moubax|first1=Kim|last2=Wuyts|first2=Wim|last3=Vandecaveye|first3=Vincent|last4=Prenen|first4=Hans|title=Pulmonary lymphangitic carcinomatosis as a primary manifestation of gastric carcinoma in a young adult: a case report and review of the literature|journal=BMC Research Notes|volume=5|issue=1|year=2012|pages=638|issn=1756-0500|doi=10.1186/1756-0500-5-638}}</ref>
:*
*On [[gross pathology]], characteristic findings of [[lymphangitis]] carcinomatosa include:<ref name="lymph">Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis</ref>
:*
:*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>
:*
:*[[Carcinoma]] in multiple the [[lymphatic]] channels
*On microscopic histopathological analysis, characteristic findings of lymphangitis carcinomatosa, include:  
[[File:Metastatic gastric adenocarcinoma-lymphangitic carcinomatosis (7261944992).jpg|300px|thumb|none|Poorly differentiated adenocarcinoma within lymphatics surrounding an artery.  Yale Rosen from USA [CC BY-SA 2.0 (https://creativecommons.org/licenses/by-sa/2.0)],https://upload.wikimedia.org/wikipedia/commons/3/34/Metastatic_gastric_adenocarcinoma-lymphangitic_carcinomatosis_%287261944992%29.jpg,https://commons.wikimedia.org/wiki/File:Metastatic_gastric_adenocarcinoma-lymphangitic_carcinomatosis_(7261944992).jpg]]
:*
:*
:*
:*


==Causes==
==Causes==
* Common causes of lymphangitis carcinomatosa, include:
*Common [[causes]] of lymphangitis carcinomatosa include:<ref name="lymph">Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis</ref>
**Breast cancer (most common)
**[[Breast cancer]] (most common)
**Lung cancer  
**[[Lung cancer]]
**Colon cancer
**[[Colon cancer]]
**Stomach cancer
**[[Stomach cancer]]
**Prostate cancer
**[[Prostate cancer]]
**Cervical cancer
**[[Cervical cancer]]
**Thyroid cancer
**[[Thyroid cancer]]


==Differentiating Lymphangitis Carcinomatosa from Other Diseases==
==Differentiating Lymphangitis Carcinomatosa from Other Diseases==
*Lymphangitis carcinomatosa must be differentiated from other diseases that cause dyspnea, fatigue, and weight loss, such as:
*[[Lymphangitis]] carcinomatosa must be [[Differentiate|differentiated]] from other [[diseases]] that cause thickened [[interlobular]] [[septa]]e (on imaging finding), [[dyspnea]], [[fatigue]], and [[weight-loss]], such as:<ref name="ThomasLenox2008">{{cite journal|last1=Thomas|first1=A.|last2=Lenox|first2=R.|title=Pulmonary lymphangitic carcinomatosis as a primary manifestation of colon cancer in a young adult|journal=Canadian Medical Association Journal|volume=179|issue=4|year=2008|pages=338–340|issn=0820-3946|doi=10.1503/cmaj.080142}}</ref><ref name="AslamZhi2019">{{cite journal|last1=Aslam|first1=Hafiz M|last2=Zhi|first2=Cassandra|last3=Nadeem|first3=Muhammad|last4=Arsalan|first4=Mohammad|last5=Wallach|first5=Sara L|title=A Case of Rapidly Deteriorating Lymphangitic Carcinomatosis in a Patient with Stage IV Pancreatic Cancer |journal=Cureus|year=2019|issn=2168-8184|doi=10.7759/cureus.4421}}</ref>
:*[Differential dx1]
 
:*[Differential dx2]
:*[[Sarcoidosis]]
:*[Differential dx3]
:*[[Pulmonary]] [[Alveolar]] [[Protein|Proteinosis]]
:*[[Viral pneumonia]]; [[pneumocystis jirovecii]]
:*[[Pulmonary edema]]
:*[[Pneumonitis|Radiation pneumonitis]]
:*[[Lymphocytic]] [[interstitial pneumonitis]]
*[[Pulmonary]] lymphangitic carcinomatosis occurs in 6%–8% of [[Patient|patients]] with [[pulmonary]] [[Metastasis|metastases]].<ref name="ThomasLenox20082">{{cite journal|last1=Thomas|first1=A.|last2=Lenox|first2=R.|title=Pulmonary lymphangitic carcinomatosis as a primary manifestation of colon cancer in a young adult|journal=Canadian Medical Association Journal|volume=179|issue=4|year=2008|pages=338–340|issn=0820-3946|doi=10.1503/cmaj.080142}}</ref>


==Epidemiology and Demographics==
==Epidemiology and Demographics==
* The prevalence of lymphangitis carcinomatosa is approximately [number or range] per 100,000 individuals worldwide.
*Lymphangitis carcinomatosa is a [[rare]] [[disease]].<ref name="KashiharaKanai2018">{{cite journal|last1=Kashihara|first1=Eriko|last2=Kanai|first2=Osamu|last3=Okamura|first3=Misato|last4=Mio|first4=Tadashi|title=Cutaneous lymphangitis carcinomatosa made cervicofacial oedema intractable in a patient with superior vena cava syndrome|journal=BMJ Case Reports|year=2018|pages=bcr-2018-224206|issn=1757-790X|doi=10.1136/bcr-2018-224206}}</ref>
* In [year], the incidence of lymphangitis carcinomatosa was estimated to be [number or range] cases per 100,000 individuals in [location].
*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</ref>
 
===Age===
===Age===
*Patients of all age groups may develop lymphangitis carcinomatosa.
*[[Patients]] of all [[age]] groups may [[Development|develop]] [[lymphangitis]] carcinomatosa.
*Lymphangitis carcinomatosa is more commonly observed among patients aged [age range] years old.
*Lymphangitis carcinomatosa is more commonly observed among [elderly patients/young patients/children].
   
   
*[[Lymphangitis]] carcinomatosa is more commonly observed among [[patients]] [[Age|aged]] 40–49 [[Year|years]] old.<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis</ref>
*[[Lymphangitis]] carcinomatosa is more commonly observed among middle [[Age|aged]] [[Adult|adults]]. 
===Gender===
===Gender===
*Lymphangitis carcinomatosa affects men and women equally.
*The condition affects [[males]] more than the [[Female|females]]
*Affects a younger [[population]] than that affected with most [[malignancies]]
*[Gender 1] are more commonly affected with lymphangitis carcinomatosa than [gender 2].
 
* The [gender 1] to [Gender 2] ratio is approximately [number > 1] to 1.
===Race===
===Race===
*There is no racial predilection for lymphangitis carcinomatosa.
*There is no [[racial]] predilection for lymphangitis carcinomatosa.
 
*Lymphangitis carcinomatosa usually affects individuals of the [race 1] race.
*[Race 2] individuals are less likely to develop lymphangitis carcinomatosa.
==Risk Factors==
==Risk Factors==
*Common risk factors in the development of lymphangitis carcinomatosa are [risk factor 1], [risk factor 2], [risk factor 3], and [risk factor 4].
*Common [[risk factors]] in the [[development]] of lymphangitis carcinomatosa include:<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis</ref>
:*Personal [[History and Physical examination|history]] of [[cancer]]
:*Preexistent [[malignant]] [[cancer]]
 
== Natural History, Complications and Prognosis==
== Natural History, Complications and Prognosis==
*The majority of patients with lymphangitis carcinomatosa remain asymptomatic for [duration/years].  
*The majority of [[patients]] with [[lymphangitis]] carcinomatosa are [[symptomatic]] at the time of [[diagnosis]].<ref name="RajaSeshadri2011">{{cite journal|last1=Raja|first1=Anand|last2=Seshadri|first2=Ramakrishnan Ayloor|last3=Sundersingh|first3=Shirley|title=Lymphangitis Carcinomatosa: Report of a Case and Review of Literature|journal=Indian Journal of Surgical Oncology|volume=1|issue=3|year=2011|pages=274–276|issn=0975-7651|doi=10.1007/s13193-011-0047-9}}</ref>
*Early clinical features include dyspnea, fatigue, and weight-loss.  
*There are two [[Theory|theories]] as to how this [[condition]] occurs<ref name="KhachekianShargh2015">{{cite journal|last1=Khachekian|first1=Arsineh|last2=Shargh|first2=Sean|last3=Arabian|first3=Sarkis|title=Pulmonary Lymphangitic Carcinomatosis From Metastatic Gastric Adenocarcinoma: Case Report|journal=The Journal of the American Osteopathic Association|volume=115|issue=5|year=2015|pages=332|issn=0098-6151|doi=10.7556/jaoa.2015.064}}</ref>
*If left untreated, [#%] of patients with lymphangitis carcinomatosa may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
**The first [[theory]] states there is [[Haematogen|haematogenous]] [[metastasis]] producing [[Endarteritis obliterans|obliterative]] [[Endarteritis obliterans|endarteritis]] and then [[Tumor cell|tumor]] [[Cells (biology)|cells]] subsequently egress through [[vascular]] walls into the [[Perivascular cell|perivascular]] [[lymphatics]].
*Common complications of lymphangitis carcinomatosa include [complication 1], [complication 2], and [complication 3].
**The second [[theory]] states there may be [[diffuse]] retrograde permeation and [[embolization]] of [[lymphatics]] after involvement of the [[hilar]] [[Lymph nodes|lymph nodes.]]
*Prognosis is generally [excellent/good/poor], and the [1/5/10­year mortality/survival rate] of patients with lymphangitis carcinomatosa is approximately [#%].
*Early [[clinical]] features include [[dyspnea]], [[fatigue]], and [[weight-loss]].
*Lymphangitis carcinomatosa may precede or obscure or dominate any local [[symptoms]] and present as a [[diagnostic]] problem in [[dyspnoea]].<ref name="JaswalAhuja2019">{{cite journal|last1=Jaswal|first1=Sofia|last2=Ahuja|first2=Vanita|last3=Aggarwal|first3=Deepak|last4=Kaur|first4=Harkirat|title=Incidental finding of pulmonary lymphangitis carcinomatosa in a patient of chest trauma|journal=Indian Journal of Anaesthesia|volume=63|issue=1|year=2019|pages=70|issn=0019-5049|doi=10.4103/ija.IJA_581_18}}</ref>
*Lymphangitis carcinomatosa may also occur during the evolution of a [[growth]], which has already been identified during [[life]], in which case [[diagnosis]] becomes relatively easier.
*If left untreated, [[patients]] with lymphangitis carcinomatosa may progress to [[Development|develop]] acute [[respiratory failure]].
*Common [[complications]] of lymphangitis carcinomatosa include:<ref name="ThomasLenox2008" />
 
:*[[Pulmonary embolism D-dimer|Pulmonary tumor embolism]]
:*[[Pulmonary hypertension]]  
*[[Prognosis]] is generally poor, and the [[mean]] [[survival rate]] of [[patients]] after [[diagnosis]] of [[lymphangitis]] carcinomatosa is approximately 6 months.
*Approximately half of [[patients]] succumbing to their [[illness]] within a [[year]] of [[diagnosis]].
*Occasionally, long-term [[Survival rate|survival]] is encountered.


== Diagnosis ==
== Diagnosis ==  
===Diagnostic Criteria===
*The diagnosis of lymphangitis carcinomatosa is made when at least [number] of the following [number] diagnostic criteria are met:
:*[criterion 1]
:*[criterion 2]
:*[criterion 3]
:*[criterion 4]
=== Symptoms ===
=== Symptoms ===
*Lymphangitis carcinomatosa is usually asymptomatic.
*The majority of [[Patient|patients]] with lymphangitis carcinomatosa are [[asymptomatic]].
*Symptoms of lymphangitis carcinomatosa may include the following:
*Lymphangitis carcinomatosa is usually [[asymptomatic]].<ref name="RajaSeshadri20112">{{cite journal|last1=Raja|first1=Anand|last2=Seshadri|first2=Ramakrishnan Ayloor|last3=Sundersingh|first3=Shirley|title=Lymphangitis Carcinomatosa: Report of a Case and Review of Literature|journal=Indian Journal of Surgical Oncology|volume=1|issue=3|year=2011|pages=274–276|issn=0975-7651|doi=10.1007/s13193-011-0047-9}}</ref>
:*[symptom 1]
*[[Symptoms]] of [[lymphangitis]] carcinomatosa may include the following:<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis</ref>
:*[symptom 2]
 
:*[symptom 3]
:*[[Hemoptysis]]
:*[symptom 4]
:* [[Chronic (medical)|Chronic]] [[coughing]]
:*[symptom 5]
:* [[Chest pain]]
:*[symptom 6]
:*[[Loss of appetite]]
:* [[Fatigue]]
 
=== Physical Examination ===
=== Physical Examination ===
*Patients with lymphangitis carcinomatosa usually appear [general appearance].
*[[Patients]] with [[lymphangitis]] carcinomatosa usually appear [[Pallor|pale]] and [[malnourished]].  
*Physical examination may be remarkable for:
*[[Physical examination|Physical]] [[Physical examination|examination]] may be remarkable for:<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis</ref>
:*[finding 1]
'''Auscultation'''
:*[finding 2]
*[[Presenting symptom|Present]] [[pleural friction rub]]
:*[finding 3]
*[[Presenting symptom|Present]] [[egophony]]
:*[finding 4]
*Crackling or bubbling [[Noise|noises]]
:*[finding 5]
*Present whispered pectoriloquy
:*[finding 6]
*[[Decreased Breath Sounds|Decreased]]/absent [[breath sounds]]
'''Percussion'''
*Dull [[percussion]]
*[[Reduced]] [[chest]] expansion
 
=== Laboratory Findings ===
=== Laboratory Findings ===
*There are no specific laboratory findings associated with lymphangitis carcinomatosa.
*There are no specific [[laboratory]] findings associated with lymphangitis carcinomatosa.<ref name="GilchristAlton2011">{{cite journal|last1=Gilchrist|first1=F. J.|last2=Alton|first2=H.|last3=Brundler|first3=M.-A.|last4=Edwards|first4=L.|last5=Plunkett|first5=A.|last6=Rao|first6=S.|title=Pulmonary lymphangitic carcinomatosis presenting as severe interstitial lung disease in a 15-year-old female|journal=European Respiratory Review|volume=20|issue=121|year=2011|pages=208–210|issn=0905-9180|doi=10.1183/09059180.00000911}}</ref>
*Even if [[Histology|histologically]] confirmed, the [[chest radiograph]] is normal in 30–50% of cases.


===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</ref>
*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</ref>
:*Subpleural [[nodules]], and thickening on the interlobar [[fissures]]
:*[[Pleural effusion]]
:*[[Hilar]] and [[mediastinal]] [[Nodule (medicine)|nodal]] enlargement (40-50%)
:*Relatively little destruction of overall [[lung]] architecture
:*Involvement of the peripheral ([[interlobular]] [[septa]]) and central [[lymphatic]] [[Lymphatic System|system]]
:*Distribution of changes is [[variable]], but most are asymmetric and [[Patching|patchy]]
:*Usually [[bilateral]] (may be unilateral especially in cases of [[lung]] and [[breast cancer]])
*[[Radiographic]] [[Appearance|appearances]] can most easily be divided into those due to the involvement of the peripheral and central lymphatic [[Lymphatic system|system]].<ref name="MunnPadera2014">{{cite journal|last1=Munn|first1=Lance L.|last2=Padera|first2=Timothy P.|title=Imaging the lymphatic system|journal=Microvascular Research|volume=96|year=2014|pages=55–63|issn=00262862|doi=10.1016/j.mvr.2014.06.006}}</ref>
*Involvement may be [[Diffuse|diffusely]] of both, or predominantly of one [[Compartments|compartment]] or the other.
*[[Distribution of terms|Distribution]] of changes is [[variable]], but most are asymmetric and patchy; it is usually [[bilateral]] but may be unilateral, specifically in cases of [[Lung cancer|lung]] and [[breast cancer]].<ref name="AburtoHerráez2018">{{cite journal|last1=Aburto|first1=Myriam|last2=Herráez|first2=Inmaculada|last3=Iturbe|first3=David|last4=Jiménez-Romero|first4=Ana|title=Diagnosis of Idiopathic Pulmonary Fibrosis: Differential Diagnosis|journal=Medical Sciences|volume=6|issue=3|year=2018|pages=73|issn=2076-3271|doi=10.3390/medsci6030073}}</ref>


*Computed tomography is the imaging modality of choice for lymphangitis carcinomatosa.
[[File:Breast-cancer-with-lymphangitis-carcinomatosa.jpg|300px|thumb|none|Left breast mass, mediastinal lymphadenopathy and features of lymphangitis carcinomatosis. In addition scattered bony metastatic lesions are seen in dorsal vertebrae, Case courtesy of Dr Paresh K Desai , Radiopaedia.org, rID: 17917, Case courtesy of Dr Paresh K Desai , <a href="https://radiopaedia.org/">Radiopaedia.org</a>. From the case <a href="https://radiopaedia.org/cases/17917">rID: 17917</a>]]
*On CT, characteristic findings of lymphangitis carcinomatosa, include:
:*Subpleural nodules, and thickening on the interlobar fissures
:*Pleural effusion(s): pleural carcinomatosis
:*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 ==
== Treatment ==
=== Medical Therapy ===
=== Medical Therapy ===
*There is no treatment for lymphangitis carcinomatosa; the mainstay of therapy is supportive care.
*The mainstay [[therapy]] for lymphangitis carcinomatosa is [[systemic]] [[chemotherapy]] ([[chemotherapeutic]] [[Chemotherapy regimens|regimen]] depends on the [[tumor]] [[histology]]).<ref name="radio">Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis</ref>
 
*The mainstay of therapy for lymphangitis carcinomatosa is [medical therapy 1] and [medical therapy 2].
*[Medical therapy 1] acts by [mechanism of action1].
*Response to [medical therapy 1] can be monitored with [test/physical finding/imaging] every [frequency/duration].
=== Surgery ===
=== Surgery ===
*Surgery is the mainstay of therapy for lymphangitis carcinomatosa.
*[[Surgery]] is not recommended for [[patients]] with lymphangitis carcinomatosa.
*[Surgical procedure] in conjunction with [chemotherapy/radiation] is the most common approach to the treatment of lymphangitis carcinomatosa.
 
*[Surgical procedure] can only be performed for patients with [disease stage] lymphangitis carcinomatosa.
=== Prevention ===
=== Prevention ===
*There are no primary preventive measures available for lymphangitis carcinomatosa.
*There are no primary [[Preventive medicine|preventive]] measures available for lymphangitis carcinomatosa. <ref name="BiswasSriram2015">{{cite journal|last1=Biswas|first1=Abhishek|last2=Sriram|first2=Peruvemba S.|title=Getting the Whole Picture: Lymphangitic Carcinomatosis|journal=The American Journal of Medicine|volume=128|issue=8|year=2015|pages=837–840|issn=00029343|doi=10.1016/j.amjmed.2015.04.007}}</ref>
*Once diagnosed and successfully treated, patients with lymphangitis carcinomatosa are followed-up every [duration]. Follow-up testing includes [test 1], [test 2], and [test 3].
*Once [[Diagnose|diagnosed]] and successfully treated, [[patients]] with lymphangitis carcinomatosa are followed-up periodically.
*Follow-up [[testing]] may include [[Pulmonary function test|respiratory function tests]] and [[disease]] progression monitorization.


==References==
==References==
{{Reflist|2}}
{{Reflist|2}}
   
   
[[Category: Oncology]]
[[Category:Oncology]]
[[Category:Up-To-Date]]
[[Category:Oncology]]
[[Category:Medicine]]
[[Category:Hematology]]
[[Category:Immunology]]
[[Category:Vascular medicine]]

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Swathi Venkatesan, M.B.B.S.[2] Sogand Goudarzi, MD [3]

Synonyms and keywords: Lymphangitis carcinomatosis; Pulmonary lymphangitic carcinomatosis

Overview

Lymphangitis carcinomatosa also known as carcinomatous lymphangitis, 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

Classification

  • There is no established system for the classification of lymphangitis carcinomatosa.[3]

Pathophysiology

  • No remarkable findings
Poorly differentiated adenocarcinoma within lymphatics surrounding an artery. Yale Rosen from USA [CC BY-SA 2.0 (https://creativecommons.org/licenses/by-sa/2.0)],https://upload.wikimedia.org/wikipedia/commons/3/34/Metastatic_gastric_adenocarcinoma-lymphangitic_carcinomatosis_%287261944992%29.jpg,https://commons.wikimedia.org/wiki/File:Metastatic_gastric_adenocarcinoma-lymphangitic_carcinomatosis_(7261944992).jpg

Causes

Differentiating Lymphangitis Carcinomatosa from Other Diseases

Epidemiology and Demographics

  • Lymphangitis carcinomatosa is a rare disease.[12]
  • The prevalence of lymphangitis carcinomatosa is approximately 0.03 per 100,000 individuals worldwide.[13]

Age

Gender

Race

  • There is no racial predilection for lymphangitis carcinomatosa.

Risk Factors

Natural History, Complications and Prognosis

Diagnosis

Symptoms

Physical Examination

Auscultation

Percussion

Laboratory Findings

Imaging Findings

Left breast mass, mediastinal lymphadenopathy and features of lymphangitis carcinomatosis. In addition scattered bony metastatic lesions are seen in dorsal vertebrae, Case courtesy of Dr Paresh K Desai , Radiopaedia.org, rID: 17917, Case courtesy of Dr Paresh K Desai , <a href="https://radiopaedia.org/">Radiopaedia.org</a>. From the case <a href="https://radiopaedia.org/cases/17917">rID: 17917</a>

Treatment

Medical Therapy

Surgery

Prevention

References

  1. ↑ Doyle, L (2018). "Gabriel Andral (1797-1876) and the First Reports of Lymphangitis Carcinomatosa". Journal of the Royal Society of Medicine. 82 (8): 491–493. doi:10.1177/014107688908200814. ISSN 0141-0768.
  2. ↑ Doyle, L (2018). "Gabriel Andral (1797-1876) and the First Reports of Lymphangitis Carcinomatosa". Journal of the Royal Society of Medicine. 82 (8): 491–493. doi:10.1177/014107688908200814. ISSN 0141-0768.
  3. ↑ Aslam HM, Zhi C, Nadeem M, Arsalan M, Wallach SL (April 2019). "A Case of Rapidly Deteriorating Lymphangitic Carcinomatosis in a Patient with Stage IV Pancreatic Cancer". Cureus. 11 (4): e4421. doi:10.7759/cureus.4421. PMC 6559437 Check |pmc= value (help). PMID 31245208.
  4. ↑ 4.0 4.1 Biswas, Abhishek; Sriram, Peruvemba S. (2015). "Getting the Whole Picture: Lymphangitic Carcinomatosis". The American Journal of Medicine. 128 (8): 837–840. doi:10.1016/j.amjmed.2015.04.007. ISSN 0002-9343.
  5. ↑ Beattie, J.W. (1956). "Lymphangitis carcinomatosa". British Journal of Tuberculosis and Diseases of the Chest. 50 (2): 120–129. doi:10.1016/S0366-0869(56)80046-4. ISSN 0366-0869.
  6. ↑ "Lymphangitic carcinomatosis | Radiology Reference Article | Radiopaedia.org".
  7. ↑ Moubax, Kim; Wuyts, Wim; Vandecaveye, Vincent; Prenen, Hans (2012). "Pulmonary lymphangitic carcinomatosis as a primary manifestation of gastric carcinoma in a young adult: a case report and review of the literature". BMC Research Notes. 5 (1): 638. doi:10.1186/1756-0500-5-638. ISSN 1756-0500.
  8. ↑ 8.0 8.1 8.2 Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis
  9. ↑ 9.0 9.1 Thomas, A.; Lenox, R. (2008). "Pulmonary lymphangitic carcinomatosis as a primary manifestation of colon cancer in a young adult". Canadian Medical Association Journal. 179 (4): 338–340. doi:10.1503/cmaj.080142. ISSN 0820-3946.
  10. ↑ Aslam, Hafiz M; Zhi, Cassandra; Nadeem, Muhammad; Arsalan, Mohammad; Wallach, Sara L (2019). "A Case of Rapidly Deteriorating Lymphangitic Carcinomatosis in a Patient with Stage IV Pancreatic Cancer". Cureus. doi:10.7759/cureus.4421. ISSN 2168-8184.
  11. ↑ Thomas, A.; Lenox, R. (2008). "Pulmonary lymphangitic carcinomatosis as a primary manifestation of colon cancer in a young adult". Canadian Medical Association Journal. 179 (4): 338–340. doi:10.1503/cmaj.080142. ISSN 0820-3946.
  12. ↑ Kashihara, Eriko; Kanai, Osamu; Okamura, Misato; Mio, Tadashi (2018). "Cutaneous lymphangitis carcinomatosa made cervicofacial oedema intractable in a patient with superior vena cava syndrome". BMJ Case Reports: bcr-2018–224206. doi:10.1136/bcr-2018-224206. ISSN 1757-790X.
  13. ↑ 13.0 13.1 13.2 13.3 13.4 13.5 13.6 13.7 Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis
  14. ↑ Raja, Anand; Seshadri, Ramakrishnan Ayloor; Sundersingh, Shirley (2011). "Lymphangitis Carcinomatosa: Report of a Case and Review of Literature". Indian Journal of Surgical Oncology. 1 (3): 274–276. doi:10.1007/s13193-011-0047-9. ISSN 0975-7651.
  15. ↑ Khachekian, Arsineh; Shargh, Sean; Arabian, Sarkis (2015). "Pulmonary Lymphangitic Carcinomatosis From Metastatic Gastric Adenocarcinoma: Case Report". The Journal of the American Osteopathic Association. 115 (5): 332. doi:10.7556/jaoa.2015.064. ISSN 0098-6151.
  16. ↑ Jaswal, Sofia; Ahuja, Vanita; Aggarwal, Deepak; Kaur, Harkirat (2019). "Incidental finding of pulmonary lymphangitis carcinomatosa in a patient of chest trauma". Indian Journal of Anaesthesia. 63 (1): 70. doi:10.4103/ija.IJA_581_18. ISSN 0019-5049.
  17. ↑ Raja, Anand; Seshadri, Ramakrishnan Ayloor; Sundersingh, Shirley (2011). "Lymphangitis Carcinomatosa: Report of a Case and Review of Literature". Indian Journal of Surgical Oncology. 1 (3): 274–276. doi:10.1007/s13193-011-0047-9. ISSN 0975-7651.
  18. ↑ Gilchrist, F. J.; Alton, H.; Brundler, M.-A.; Edwards, L.; Plunkett, A.; Rao, S. (2011). "Pulmonary lymphangitic carcinomatosis presenting as severe interstitial lung disease in a 15-year-old female". European Respiratory Review. 20 (121): 208–210. doi:10.1183/09059180.00000911. ISSN 0905-9180.
  19. ↑ Munn, Lance L.; Padera, Timothy P. (2014). "Imaging the lymphatic system". Microvascular Research. 96: 55–63. doi:10.1016/j.mvr.2014.06.006. ISSN 0026-2862.
  20. ↑ Aburto, Myriam; Herráez, Inmaculada; Iturbe, David; Jiménez-Romero, Ana (2018). "Diagnosis of Idiopathic Pulmonary Fibrosis: Differential Diagnosis". Medical Sciences. 6 (3): 73. doi:10.3390/medsci6030073. ISSN 2076-3271.
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