Lymphangiomatosis

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Classification according to ICD-10
D18.1- Lymphangioma, any location
ICD-10 online (WHO version 2019)

Lymphangiomatosis is a rare disease condition that is characterized by a diffuse proliferation of lymph vessels . This can affect internal organs, bones, soft tissues, and the skin. It is most widespread among children and young adults. Symptoms and chances of recovery vary widely between individuals and depending on the organs affected. Although little is known about the causes, it is believed that it is a rare malformation that results in abnormal development of the lymphatic system .

The name lymphangiomatosis has its origin in the words lymph- (description of the lymphatic system), -angio- (vessel), -oma- (tumor or cyst), -tosis (condition). Other names for these or similar diseases are: general lymphangioma, systemic cystic angiomatosis, multiple lymphangiectasias, general or diffuse lymphatic malformation, general lymphatic anomaly.

A single collection of lymph vessels or cysts is called a lymphangioma . Lymphangiomatosis describes cases where a lymphangioma is not present as a singular mass, but where the disorder is widespread or in different places (e.g. when different parts of the body are affected).

General lymphangiomatosis was first described in 1828.

Signs and symptoms

Almost any part of the body can be affected by lymphangiomatosis. Only the central nervous system is not affected due to the lack of lymph vessels. Signs and symptoms depend on the area of ​​the body that is affected.

The following problems can occur: pericardial effusions (fluid in the heart area), pleural effusions (fluid in the lung cavity), ascites (fluid in the abdominal cavity), broken bones, skin lesions, fever, and internal bleeding. The effusion fluid is of the chylous (milky) type. Symptoms include shortness of breath, coughing, problems breathing in, massive pain in the abdomen and pelvis, and lymphedema (swelling). Some patients have no symptoms, but show abnormalities in various imaging procedures.

diagnosis

There is currently no standard for diagnosing this disease. The symptoms, clinical appearance, and typical radiographic findings help in the diagnosis. A biopsy is the best method, but not always possible. Whole-body MRI is a good way to determine the spread of the disease. MRI gives better results compared to CT scans.

Lymphangiomatosis can be confused with other diseases, such as: B. lymphatic displasia, lymphangiectasis and the similar-sounding lymphangioleiomyomatosis (LAM). LAM occurs mainly in women of childbearing age.

Other diseases associated with lymphangiomatosis

In Gorham-Stout disease , lymph vessels grow into bones, which is usually associated with their dissolution. Many patients are affected by both clinical pictures, which makes a precise distinction difficult. However, there are also cases in which patients are diagnosed exclusively with lymphangiomatosis or exclusively with Gorham-Stout disease. See also the rare Hennekam syndrome .

Treatment options

If surgery can remove the abnormal lymphoid tissue, then cure is possible. However, because of the infiltrating behavior of the lymphatic vessels in lymphangiomatosis, the problem cannot be solved by surgery in most patients. Treatment therefore often focuses on relieving symptoms and trying to contain the progression of the disease. Vascular endothelial growth factors are known to regulate lymphatic vessel growth. The protein VEGFR3 is partly important in the development of the lymphatic system. This means that treatment with vascular growth inhibitors can contain the progression of the disease.

A wide range of additional treatments were used. These include:

  • Drainage of the space around the lungs and around the heart (pleural and pericardial drainage)
  • Pleurodesis (procedure to restrict the space around the lungs to prevent fluid retention)
  • restricted diet that avoids fat intake (except medium-chain triglycerides)
  • drug treatment with: methylprednisilone, bisphosphonates, alpha interferon, chlorambucil / etoposide
  • Radiotherapy
  • amputation
  • Lung transplant

forecast

The tumors that this disease produces are not metastatic like cancer, but they often spread over time.

The prognosis depends on the affected tissue and the spread. If a lot of soft tissue is affected, and if there is a chylothorax (lymph fluid in the lung cavity), the prognosis is generally relatively poor. As long as only extremities are affected, the prognosis is usually better.

research

So far, relatively little is known about this rare disease and there are no major research projects in patients with this disease. Case reports represent the primary format of previous publications, specifically in lymphangiomatosis and Gorham's disease. The number of affected patients is unknown as case reports are not published for each.

Web links

Individual evidence

  1. ^ A b c Philip E LeBoit, David Weedon, Alain Sarasin: Pathology and genetics of skin tumors. (= WHO Classification of Tumors. Volume 6). 2005, ISBN 92-832-2189-3 , p. 249.
  2. a b c Dong Hyun Yang, Hyun Woo Goo In: Korean J Radiol. 2006; 7, pp. 287-291.
  3. ^ A b c Sharon W. Weiss, John R. Goldblum, Franz M. Enzinger: Enzinger and Weiss's soft tissue tumors. Year ?, p. 967.
  4. EM Marom, CA Moran, RF Munden: Generalized lymphangiomatosis. In: American Journal of Roentgenology . (2004); 182 (4), p. 1068. PMID 15039189
  5. LGD Alliance ( Memento of the original from March 23, 2010 in the Internet Archive ) Info: The archive link was inserted automatically and not yet checked. Please check the original and archive link according to the instructions and then remove this notice. @1@ 2Template: Webachiv / IABot / www.lgdalliance.org
  6. SS Hwang, Choi, Park: Cavernous mesenteric lymphangiomatosis mimicking metastasis in a patient with rectal cancer: a case report. In: World journal of gastroenterology: WJG. (2009); 15 (31), pp. 3947-3949. doi: 10.3748 / year 15.3947 PMID 19701979 .
  7. ^ CS Wong, TY Chu: Clinical and radiological features of generalized lymphangiomatosis. In: Hong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicine. (2008); 14 (5), pp. 402-404. PMID 18840914
  8. a b c Christian Timke, Martin F. Krause, Hans-Conrad Oppermann, Ivo Leuschner, Alexander Claviez: Interferon Alpha 2b Treatment in an Eleven-Year-Old Boy With Disseminated Lymphangiomatosis In: Pediatr Blood Cancer . (2007); 48, pp. 108-111.
  9. a b R. I. Aviv, K. McHugh, J. Hunt: Angiomatosis of Bone and Soft Tissue: A Spectrum of Disease from Diffuse Lymphangiomatosis to Vanishing Bone Disease in Young Patients. In: Clin Radiol. 2001 Mar; 56 (3), pp. 184-190.
  10. ^ Philip T. Cagle, Timothy C. Allen, Roberto Barrios, Carlos Bedrossian, Megan K Dishop et al: Color Atlas and Text of Pulmonary Pathology. Lippincott Williams & Wilkins, Philadelphia / London 2005, ISBN 0-7817-3453-3 .
  11. P. Agarwal, F. Matzinger, J. Seely: Case 132: Lymphangiomatosis. In: Radiology. (2008); 247 (1), pp. 288-290. doi: 10.1148 / radiol.2471042092 . PMID 18372473 .
  12. Orhan Konez, Pranav K. Vyas, Manish Goyal: Disseminated lymphangiomatosis presenting with massive chylothorax. In: Pediatr Radiol. (2000); 30, pp. 35-37.
  13. LF John, JB Gerald, VC Thomas et al .: Thoracic Lymphangiomas, Lymphangiectasis, Lymphangiomatosis, and Lymphatic Dysplasia Syndrome. In: Am J Respir Crit Care Med. 2000; 161, pp. 1037-1046.
  14. ^ AY Rostom: Treatment of thoracic lymphangiomatosis. In: Arch Dis Child 2000; 83, pp. 138-139.
  15. ^ Felix S. Chew, Catherine C. Roberts, Anand P. Lalaji: Musculoskeletal imaging: a teaching file.