Lymphangitis carcinomatosa
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Maria Fernanda Villarreal, M.D. [2]
Synonyms and keywords: Lymphangitis carcinomatosis
Overview
Lymphangitis carcinomatosa is inflammation of the lymph vessels secondary to a malignancy. Breast, lung, stomach, pancreas, and prostate cancers are the most common tumors that result in lymphangitis.
Historical Perspective
- Lymphangitis carcinomatosa was first discovered by Gabriel Andral, a French pathologist, in 1829.
Classification
- Lymphangitis carcinomatosa may be classified according to [classification method] into [number] subtypes/groups:
- [group1]
- [group2]
- [group3]
- Other variants of lymphangitis carcinomatosa include [disease subtype 1], [disease subtype 2], and [disease subtype 3].
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:
- On microscopic histopathological analysis, characteristic findings of lymphangitis carcinomatosa, include:
Causes
- Common causes of lymphangitis carcinomatosa, include:
- Breast cancer (most common)
- Lung cancer
- Colon cancer
- Stomach cancer
- Prostate cancer
- Cervical cancer
- Thyroid cancer
Differentiating Lymphangitis Carcinomatosa from Other Diseases
- Lymphangitis carcinomatosa must be differentiated from other diseases that cause dyspnea, fatigue, and weight loss, such as:
- Sarcoidosis
- Viral pneumonia
- Pulmonary edema
- Radiation pneumonitis
- Lymphocytic interstitial pneumonitis
Epidemiology and Demographics
- The prevalence of lymphangitis carcinomatosa is approximately [number or range] per 100,000 individuals worldwide.
- In [year], the incidence of lymphangitis carcinomatosa was estimated to be [number or range] cases per 100,000 individuals in [location].
Age
- Patients of all age groups may 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].
Gender
- Lymphangitis carcinomatosa affects men and women equally.
- [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
- There is no racial predilection for lymphangitis carcinomatosa.
Risk Factors
- Common risk factors in the development of lymphangitis carcinomatosa, include:
- Personal history of cancer
- Preexistent malignant cancer
Natural History, Complications and Prognosis
- The majority of patients with lymphangitis carcinomatosa remain asymptomatic for [duration/years].
- Early clinical features include dyspnea, fatigue, and weight-loss.
- If left untreated, [#%] of patients with lymphangitis carcinomatosa may progress to develop [manifestation 1], [manifestation 2], and [manifestation 3].
- Common complications of lymphangitis carcinomatosa include [complication 1], [complication 2], and [complication 3].
- Prognosis is generally [excellent/good/poor], and the [1/5/10year mortality/survival rate] of patients with lymphangitis carcinomatosa is approximately [#%].
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
- Lymphangitis carcinomatosa is usually asymptomatic.
- Symptoms of lymphangitis carcinomatosa may include the following:
- [symptom 1]
- [symptom 2]
- [symptom 3]
- [symptom 4]
- [symptom 5]
- [symptom 6]
Physical Examination
- Patients with lymphangitis carcinomatosa usually appear pale and malnourished.
- Physical examination may be remarkable for:
- [finding 1]
- [finding 2]
- [finding 3]
- [finding 4]
- [finding 5]
- [finding 6]
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.
- On CT, characteristic findings of lymphangitis carcinomatosa, include:
- 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
- There is no treatment for lymphangitis carcinomatosa; the mainstay of therapy is supportive care.
- 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 is the mainstay of therapy for 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
- There are no primary preventive measures available for lymphangitis carcinomatosa.
- 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].