Lymphangitis carcinomatosa

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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].

References