Lymphangitis carcinomatosa

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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

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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