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Lymphangiomatosis

Lymphangiomatosis is a rare condition characterised by a diffuse proliferation of lymphatic vessels. It can affect internal organs, bones, soft tissue and/or skin [1][2]. It is most common in children and young adults [3]. Symptoms and prognosis vary greatly between individuals and according to the organs involved. Though little is understood about causes, lymphangiomatosis is thought to be a rare neoplasm (growth) which results from an abnormal development of the lymphatic system [4].

The name lymphangiomatosis comes from the words 'lymph-' (describing the lymphatic system), '-angio-' (meaning relating to vessels), '-oma-' (tumour or cyst), '-tosis' (condition) [5]. Other names used for the disease or very similar conditions include: generalised lymphangioma, systemic cystic angiomatosis, multiple lymphangiectasias [6], generalised lymphatic malformation, generalized lymphatic anomaly, diffuse lymphatic malformation among others.

A single mass of lymph vessels or cyst is known as a lymphangioma. Lymphangiomatosis descibes cases where a lymphangioma is not present in a single localised mass, but in a widespread or multifocal manner [7] (i.e. involving several parts of the body).

Signs and symptoms

Almost any body part may be affected, except for the central nervous system due to its lack of lymphatic vessels [8].  Signs and symptoms depend on which areas are affected [9].

Patients may present complications with include pericardial effusions (fluid around the heart), pleural effusion (fluid around the lung cavity), ascites (fluid in the abdominal cavity), bone fractures, skin lesions [10], fever and internal bleeding [11]. The fluid involved in effusions is chylous (milky type fluid) in nature. Symptoms include shortness of breath, cough [12], difficulty breathing in (inhaling), severe pain in the abdominal cavity and pelvic cavity, and lymphedema (swelling). Some patients do not have symptoms but show abnormal results on imaging [13].

Diagnosis

There is no current standard for diagnosing this condition. The symptoms, clinical presentation, and typical radiographical findings can assist in the diagnosis [14]. A biopsy is the ultimate method for diagnosis, but is not always possible.

Whole body magnetic resonance imaging has good capability for evaluating the extent of disease [15]. MRI has been found to give better results than CT scans [16], though CT is commonly used too.

Lymphangiomatosis can be confused with other diseases, including lymphatic displasia, lymphangiectasis, and the similar-sounding lymphangioleiomyomatosis (LAM) [17]. LAM is primarily a disease found in women of child-bearing age [18].

Treatment

If it is possible to remove the abnormal lymphatic tissue through surgery, then patients may be cured. However, due to the infiltrative character of the lymphatic vessels in lymphangiomatosis, most patients have inoperable disease [20][21]. Most treatment is therefore palliative (aimed at relieving symptoms) or aimed at slowing the progress of the disease.

Vascular endothelial growth factors are known to regulate the growth of lymphatic vessels. A protein called VEGFR3 is particularly important in the lymphatic system [22]. This means that treatment with vascular growth inhibitors may slow the progress of the disease.

A wide variety of other treatments has been tried. These include [23]:

  • drainage of the spaces around the lungs and around the heart to remove fluid from these areas (pleural and pericardial drainage)
  • pleurodesis (a procedure to restrict the space around the lung where fluid can collect)
  • a restricted diet that avoids fats except for medium chain triglycerides (a certain type of fat)
  • drug treatments including methylprednisilone, bisphosphonates, alpha interferon, chlorambucil/etoposide
  • radiotherapy
  • amputation
  • lung transplant [24]

Prognosis

The lymph vessel tumours tumours that the diseases produces are not metastatic (cancerous) [26], but can show aggressive growth behaviour and they do appear to spread over time.

The prognosis depends on the extent of the disease [25]. When there is a lot of soft tissue involved, and chylothorax (lymph fluid around the lung cavity) then prognosis is usually poor. If just the extremities are affected, the prognosis is better. [26]

History

Generalized lymphangiomatosis was first described in 1828. [27]

Note

The decsription of lymphangiomatosis has been written based on literature review and it has been reviewed by professional of med. Currently the understanding of lymphangiomatosis among professionals is not fully congruent and slightly different opinion may appear. We do not intent to delineate the definition.

 

References

  1. Pathology and genetics of skin tumours, Volume 6 By Philip E. LeBoit, p249
  2. Dong Hyun Yang, Hyun Woo Goo, Korean J Radiol 2006;7:287-291
  3. Enzinger and Weiss's soft tissue tumors By Sharon W. Weiss, John R. Goldblum, Franz M. Enzinger, p967
  4. Marom, EM; Moran, CA; Munden, RF (2004). "Generalized lymphangiomatosis". AJR. American journal of roentgenology 182 (4): 1068. PMID 15039189.
  5. http://www.lgdalliance.org/en/aboutLymphangiomatosis/Default.aspx
  6. Pathology and genetics of skin tumours, Volume 6 By Philip E. LeBoit, p249
  7. Hwang, SS; Choi; Park (2009). "Cavernous mesenteric lymphangiomatosis mimicking metastasis in a patient with rectal cancer: a case report". World journal of gastroenterology : WJG 15 (31): 3947–9. doi:10.3748/wjg.15.3947.PMID 19701979.
  8. Pediatr Blood Cancer 2007;48:108–111 ; Interferon Alpha 2b Treatment in an Eleven-Year-Old Boy With Disseminated Lymphangiomatosis ; Christian Timke, MD,1* Martin F. Krause, MD,1 Hans-Conrad Oppermann, MD,2 Ivo Leuschner, MD,3 and Alexander Claviez, MD 1
  9. Enzinger and Weiss's soft tissue tumors By Sharon W. Weiss, John R. Goldblum, Franz M. Enzinger, p967
  10. Pathology and genetics of skin tumours, Volume 6 By Philip E. LeBoit, p249
  11. Angiomatosis of Bone and Soft Tissue: A Spectrum of Disease from Diffuse Lymphangiomatosis to Vanishing Bone Disease in Young Patients ; R. I. AVIV*, K. MCHUGH*, J. HUNT{*Department of Radiology, Great Ormond Street Hospital, U.K. and {Hemel Hempstead Hospital, U.K.
  12. Color Atlas and Text of Pulmonary Pathology By Philip T. Cagle, Timothy C. Allen, Roberto Barrios, Carlos Bedrossian, Megan K Dishop
  13. Dong Hyun Yang, Hyun Woo Goo, Korean J Radiol 2006;7:287-291
  14. Agarwal, P.; Matzinger, F.; Seely, J. (2008). "Case 132: Lymphangiomatosis". Radiology247 (1): 288–290. doi:10.1148/radiol.2471042092. PMID 18372473.
  15. Dong Hyun Yang, Hyun Woo Goo, Korean J Radiol 2006;7:287-291
  16. Orhan Konez, Pranav K.Vyas, Manish Goyal ; Disseminated lymphangiomatosis presenting with massive chylothorax ; Pediatr Radiol (2000) 30: 35±37
  17. John LF, Gerald JB, Thomas VC, et al. Thoracic Lymphangiomas, Lymphangiectasis, Lymphangiomatosis, and Lymphatic Dysplasia Syndrome. Am J Respir Crit Care Med 2000; 161: 1037-1046.
  18. http://www.ispub.com/ostia/index.php?xmlFilePath=journals/ijpm/vol5n2/malignant.xml
  20. Pediatr Blood Cancer 2007;48:108–111 ; Interferon Alpha 2b Treatment in an Eleven-Year-Old Boy With Disseminated Lymphangiomatosis ; Christian Timke, MD,1* Martin F. Krause, MD,1 Hans-Conrad Oppermann, MD,2 Ivo Leuschner, MD,3 and Alexander Claviez, MD 1
  21. Rostom AY. Treatment of thoracic lymphangiomatosis. Arch Dis Child 2000;83:138-139
  22. Pediatr Blood Cancer 2007;48:108–111 ; Interferon Alpha 2b Treatment in an Eleven-Year-Old Boy With Disseminated Lymphangiomatosis ; Christian Timke, MD,1* Martin F. Krause, MD,1 Hans-Conrad Oppermann, MD,2 Ivo Leuschner, MD,3 and Alexander Claviez, MD 1
  23. Angiomatosis of Bone and Soft Tissue: A Spectrum of Disease from Diffuse Lymphangiomatosis to Vanishing Bone Disease in Young Patients ; R. I. AVIV*, K. MCHUGH*, J. HUNT{*Department of Radiology, Great Ormond Street Hospital, U.K. and {Hemel Hempstead Hospital, U.K.
  24. http://www3.interscience.wiley.com/journal/121356527/abstract?CRETRY=1&SRETRY=0
  25. Enzinger and Weiss's soft tissue tumors By Sharon W. Weiss, John R. Goldblum, Franz M. Enzinger, p967
  26. Musculoskeletal imaging: a teaching file By Felix S. Chew, Catherine C. Roberts, Anand P. Lalaji
  27. Wong, CS; Chu, TY (2008). "Clinical and radiological features of generalised lymphangiomatosis". Hong Kong medical journal = Xianggang yi xue za zhi / Hong Kong Academy of Medicine 14 (5): 402–4. PMID 18840914.

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