Lymphangitis carcinomatosa: Difference between revisions
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*If left untreated, patients with lymphangitis carcinomatosa may progress to develop acute respiratory failure. | *If left untreated, patients with lymphangitis carcinomatosa may progress to develop acute respiratory failure. | ||
*Common complications of lymphangitis carcinomatosa, include: | *Common complications of lymphangitis carcinomatosa, include: | ||
:* | :*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. | *Prognosis is generally poor, and the mean survival rate of patients after diagnosis of lymphangitis carcinomatosa is approximately 6 months. | ||
Revision as of 18:51, 29 April 2016
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 thickened interlobular septae (imaging finding), 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.
Age
- Patients of all age groups may develop lymphangitis carcinomatosa.
- Lymphangitis carcinomatosa is more commonly observed among patients aged 40–49 years old.
- 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:
- 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:
- 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.
Diagnosis
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
- The mainstay of therapies for lymphangitis carcinomatosa, include:
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].