Lymphangitis carcinomatosa

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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 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

Pathophysiology

  • No remarkable findings

Causes

Differentiating Lymphangitis Carcinomatosa from Other Diseases

  • Pulmonary lymphangitic carcinomatosis occurs in 6%–8% of patients with pulmonary metastases [4]

Epidemiology and Demographics

Age

Gender

Race

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. 2.0 2.1 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.
  3. 3.0 3.1 3.2 Lymphangitis carcinomatosa. Libre Pathology. https://librepathology.org/wiki/Pulmonary_lymphangitic_carcinomatosis
  4. 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.
  5. 5.0 5.1 5.2 5.3 5.4 5.5 5.6 5.7 Lymphangitis carcinomatosa. Radiopedia. http://radiopaedia.org/articles/lymphangitic-carcinomatosis
  6. 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.
  7. 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.

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