Lymphangioma natural history

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Badria Munir M.B.B.S.[2] Haytham Allaham, M.D. [3]

Overview

Most of the patients with lymphangioma will develop a cervico-facial mass that often grows in proportion to the patient’s body growth rate. The mass usually recurs after surgical interventions. Common complications of lymphangioma include esophageal obstruction, upper respiratory tract obstruction, infections, and compression of adjacent tissues such as nerves and blood vessels. Prognosis of lymphangioma is generally excellent.

Natural History, Complications and Prognosis

Natural History

Lymphangiomas have slower rate of progression. These malformations continue to grow as with the growth of patient.[1]

  • These are present on birth and may not be evident until the age of 5 years.
  • Initially they grow as soft, cystic, translucent swelling in head and neck region, but it is commonly found in extremities. Some cases are reported in the internal organs such as pancreas and stomach as well.[2]
  • The presence of lymphangiomas and soft tissues and bone is not unusual and this form of disease has been termed as lymphangiomatosis.
  • If left untreated lymphangiomas can either progress into large swellings or they can resolve spontaneously.[3]
  • If the continue to progress, they cause overgrowth and swelling of tissue involved, such as tongue, jaws, cheeks, neck, arms, fingers and toes.[4]
  • Clinical picture may vary from person to person because certain events such as puberty, infection, trauma and bleeding into lymphatic malformation can trigger rapid growth.
  • Overgrowth can subsequently cause compressive effects on surrounding tissues, such as dyspnea due to compression on trachea, or dysphagia due to compression on esophagus and diplopia due if eye socket is involved.[5][6]
  • Rarely do cutaneous lymphangiomas interfere with the well-being of patients. Patients are expected to live a full healthy life, and they usually seek medical intervention because of cosmetic reason.
  • The signs and symptoms of lymphangioma vary depending on the type, size and location of the mass.[7]

Complications:

Common complications of lymphangiomas are primarily associated to the location of lesion.[8]

Growth of lesion causes compressive effects on surrounding tissues. Which include following:[9]

Prognosis

Prognosis of lymphangioma is generally excellent after surgical intervention.

References

  1. Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, Burrows P, Frieden IJ, Garzon MC, Lopez-Gutierrez JC, Lord DJ, Mitchel S, Powell J, Prendiville J, Vikkula M (July 2015). "Vascular Anomalies Classification: Recommendations From the International Society for the Study of Vascular Anomalies". Pediatrics. 136 (1): e203–14. doi:10.1542/peds.2014-3673. PMID 26055853.
  2. Jayappa SN, Rao P, Tandon AS, Bharathy K, Sikora SS (January 2018). "Large cystic lympangioma of the pancreas: a case reportum". Ann R Coll Surg Engl. 100 (1): e12–e14. doi:10.1308/rcsann.2017.0178. PMID 29046074.
  3. Lymphangioma. PathologyOutlines (2016) http://www.pathologyoutlines.com/topic/softtissuelymphangiomacystic.html Accessed on March 5, 2016
  4. Kocher HM, Vijaykumar T, Koti RS, Bapat RD (1995). "Lymphangioma of the chest wall". J Postgrad Med. 41 (3): 89–90. PMID 10707725.
  5. Dhrif AS, El Euch D, Daghfous M, Cherif F, Mokni M, Dhahri AB (September 2008). "[Macrocystic lymphatic lymphangioma (cystic lymphangioma) of the upper extremity: a case report]". Arch Pediatr (in French). 15 (9): 1416–9. doi:10.1016/j.arcped.2008.06.003. PMID 18667296.
  6. Selz PA, Arjmand EM (March 1998). "Laryngeal lymphangioma: a case report of an uncommon entity". Otolaryngol Head Neck Surg. 118 (3 Pt 1): 382–4. doi:10.1016/S0194-59989870320-1. PMID 9527122.
  7. Faul JL, Berry GJ, Colby TV, Ruoss SJ, Walter MB, Rosen GD, Raffin TA (March 2000). "Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome". Am. J. Respir. Crit. Care Med. 161 (3 Pt 1): 1037–46. doi:10.1164/ajrccm.161.3.9904056. PMID 10712360.
  8. Runyon BA, Forker EL, Sopko JA (May 1979). "Pleural-fluid kinetics in a patient with primary lymphedema, pleural effusions, and yellow nails". Am. Rev. Respir. Dis. 119 (5): 821–5. doi:10.1164/arrd.1979.119.5.821. PMID 453703.
  9. 10.0 10.1 Williams WT, Cole RR (March 1993). "Lymphangioma presenting as congenital stridor". Int. J. Pediatr. Otorhinolaryngol. 26 (2): 185–91. PMID 8444562.
  10. Duhra PM, Quigley EM, Marsh MN (November 1985). "Chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome". Gut. 26 (11): 1266–9. PMC 1432907. PMID 4065700.
  11. Duhra PM, Quigley EM, Marsh MN (November 1985). "Chylous ascites, intestinal lymphangiectasia and the 'yellow-nail' syndrome". Gut. 26 (11): 1266–9. PMC 1432907. PMID 4065700.
  12. Runyon BA, Forker EL, Sopko JA (May 1979). "Pleural-fluid kinetics in a patient with primary lymphedema, pleural effusions, and yellow nails". Am. Rev. Respir. Dis. 119 (5): 821–5. doi:10.1164/arrd.1979.119.5.821. PMID 453703.
  13. Papsin BC, Evans JN (October 1996). "Isolated laryngeal lymphangioma: a rare cause of airway obstruction in infants". J Laryngol Otol. 110 (10): 969–72. PMID 8977864.
  14. Ogita S, Deguchi E, Tokiwa K, Iwata J, Kubota Y, Iwai N (January 1998). "Ongoing osteolysis in patients with lymphangioma". J. Pediatr. Surg. 33 (1): 45–8. PMID 9473098.
  15. MANN TP (May 1955). "Hemihypertrophy left side of body; congenital lymphatic oedema of left arm; radiological enlargement of heart shadow". Proc. R. Soc. Med. 48 (5): 330–1. PMC 1918895. PMID 14395215.


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