Treatment of Thoracic Lymphangiomatosis

138 Arch Dis Child 2000;83:138–139

CURRENT TOPIC Arch Dis Child: first published as 10.1136/adc.83.2.138 on 1 August 2000. Downloaded from

Treatment of thoracic lymphangiomatosis

A Y Rostom

Lymphangiomas are rare benign neoplasms involvement in the young may result in believed to be the result of abnormal develop- shortening or deformity of the aVected bone. ment of the lymphatic system. They grow very The plain chest radiograph will reveal either slowly usually localised to one organ, but occa- a localised lung lesion, with the solitary type, or sionally involve several organs in one part of the diVuse pulmonary involvement with or without body. In the chest, involvement of the mediasti- thoracic lymphadenopathy. Pleural or pericar- num, lung, heart, pleura, pericardium, ribs, dial eVusion is also common at presentation. and vertebrae may be seen in the same patient. Rib or vertebral involvement will be seen as Histologically they are made up of spaces lined rarefaction of bone, earlier in the course of the with endothelium, varying from capillary size disease, or pathological fracture later on. to several centimetres in diameter. They Parenchymal lung involvement with lym- contain clear straw coloured fluid occasionally phangiomas has a characteristic computed mixed with blood. The spaces are supported by tomography (CT) scan appearance. This can a stroma of variable thickness containing be seen as lobular or a cystic mass of heteroge- lymphoid tissue. The aetiology is unknown but neous soft tissue density in the localised type or believed to be a result of congenital malforma- diVuse involvement, seen as patchy areas of ground glass appearance. tion of the lymph vessels. Radiation induced Using conventional and high resolution CT cutanous lymphangioma has been described in scan (HRCTS), Swensen et al, reporting on a patient with breast cancer following mastec- 1 eight patients with diVuse disease concluded tomy and radiotherapy. that the appearance is distinctive and includes Clinically, lymphangiomas are classified into diVuse, smooth thickening of interlobular septa three types: and bronchovascular bundles with extensive (1) Simplex, which is made up of capillary infiltration of the mediastinal fat and associated 5 sized thin, walled lymphatic channels. perihilar infiltration. Pleural thickening and/or http://adc.bmj.com/ This type usually aVects the skin (lym- eVusion were seen in seven of the eight phangioma circumscriptum) patients. Despite the typical appearance, they (2) Cystic lymphangioma (or cystic hy- stressed the importance of biopsy for a defini- groma): this may range in size from a few tive diagnosis. In one study comparing chest millimetres to several centimetres, seen x ray with HRCTS of the chest in children with in a young age, commonly in the neck or diVuse lung disease, Lynch et al confirmed the the axilla value of HRCTS scan over other conventional (3) Cavernosum: this type is made up of radiological methods.6 on October 1, 2021 by guest. Protected copyright. dilated lymphatic channels, often with Any part of the mediastinum can be fibrous adventitial coats. This is the type aVected—anterior, middle, posterior, and su- which usually aVects organs in the perior, perhaps with slight prevalence in the thorax, abdomen, and bones. anterior part. This will be seen as a well defined Bill and Sumner2 pointed out that the three multilocular water density mass with or with- types may coexist in the same patient. Reports out compression or displacement of nearby of single organ involvement have been pub- structures.7 ShaVer et al pointed out that the lished relating to nearly all organs of the body magnetic resonance imaging (MRI) findings except for the CNS, which is devoid of are not dissimilar from those of a CT scan and lymphatic channels. may include a cystic component isointense to In one large series, lymphangioma of the muscles on T1 weighted images and hyperin- Department of head and neck accounted for nearly 50% of tense to fat on T2 weighted images.8 Compar- Radiotherapy, The ing CT, ultrasound, and MRI findings, Pui et al Royal Marsden cases in children, while 10% of cases had visceral disease including the thorax.3 Thoracic concluded that MRI was more accurate in Hospital, Downs Road, 9 Sutton, Surrey lymphangioma has been described in all age delineating extent of disease. This is important SM2 5PT, UK groups including infants, neonates, and in the for preoperative surgical planning, and for A Y Rostom stillborn.4 Presenting symptoms are related to these cases, coronal and sagittal views will be of great help to the surgeon Correspondence to: pulmonary and/or mediastinal mass, and signs Dr Rostom and symptoms of pleural and/or pericardial email: assem.rostom@ eVusion. Rib or thoracic vertebral bone in- Treatment rmh.nthames.nhs.ac.uk volvement may present as chronic pain or acute Understanding the natural history of the Accepted 27 April 2000 pain in cases of pathological fracture. Bone disease aids the choice of treatment option.

www.archdischild.com Treatment of thoracic lymphangiomatosis 139

While spontaneous regression has been re- tion of these channels, not unlike that seen in

ported in two cases, and stabilisation of disease arterioles and microvasculature following Arch Dis Child: first published as 10.1136/adc.83.2.138 on 1 August 2000. Downloaded from following surgery in five of 11 patients radiotherapy.19 described in one series, this is very rare.7 The In summary, thoracic lymphangiomatosis is majority of cases will have slowly progressing a benign congenital condition that may involve disease; eventually this may lead to serious any or all organs within the chest. This can be morbidity and even mortality in some cases.10 11 seen in the neonate, infants, children, and For localised mediastinal or solitary lung adults. Although the radiological appearance lesions, thoracotomy and resection or thoraco- may be typical, a tissue diagnosis should be scopic resection12 is recommended. However, obtained in all suspected cases. Observation of with the diVuse type, especially when there is small asymptomatic lesions may be justified. multiorgan involvement, for example, mediasti- However, with more extensive localised lesions num, lungs, heart, pleura, pericardium, ribs, excision is recommended. Recurrent pleural and thoracic vertebrae, the role of surgery is eVusion should be treated by drainage and limited apart from a biopsy for tissue diagnosis pleurodesis. For more extensive and compli- or draining of pericardial eVusions and aspira- cated lesions involving more than one organ in tion with pleurodesis for patients with recur- the chest, interferon alfa or a fractionated dose rent pleural eVusion. In one patient with recur- of 18–20 Gy is an eVective and safe treatment. rent chylothorax, ligation of the thoracic duct resulted in permanent cure.13 14 15 1 Rosso R, Gianelli U, Carnevali L. Acquired progressive Systemic chemotherapy and interferon lymphangioma of the skin following radiotherapy for breast alfa16 have been tried for patients with extensive carcinoma. J Cutan Pathol 1995;22:164–7. 2 Bill AHJR, Sumner DS. Unified concept of cystic hygroma. inoperable lesions with limited success. Radio- Surg Gynecol Obstet 1965;120:79–86. therapy has been used for patients with exten- 3 Alqahtani A, Nguyen LT, Flageol H, Shaw K, Laberge JM. 17 18 J Pediatr Surg 1999;34:1164–8. sive cutaneous involvement and for patients 4 Dutheil P, Leraillezz J, Guillemette J. Wallch D. Generalized with abdominal involvement with excellent lymphangiomatosis with chylothorax and skin lymphangi- 19 oma in a neonate. Pediatr Dermatol 1998;15:296–8. results. Similar results were reported for 5 Swensen SJ, Hartman TE, Mayo JR, Colby TV, Tazelaar patients with extensive thoracic lymphangi- HD, Muller NL. DiVuse pulmonary lymphangiomatosis. 20 21 Journal of Assisted Tomography 1995;19:348–52. omatosis treated by irradiation. Johnson et 6 Lynch DA, Hay T, Newell JD Jr, Fan LL. Pediatric diVuse al treated one 13 year old patient with lung disease; diagnosis and classification using high resolu- recurrent chylothorax with prompt and perma- tion CT. Am J Radiol 1999;173:713–18. 20 7 Glasson MJ, Taylor SF. Cervical, cervicomediastinal in- nent resolution. In a similar case, a 36 year trathoracic lymphangioma. Prog Pediatr Surg 1991;27:62– old patient with chylothorax and chylopericar- 83. 8 ShaVer K, Rosado-de-christensen ML, Patz EFJ, Young S, dium and a mediastinal mass refractory to sur- Forver CF. Thoracic lymphangioma in adults. CT and MR gery and repeated drainage of pericardial and imaging features. Am J Roentgenol 1994;162:283–9. 9 Pui M, Li Z, Chen W, Chen JH. Lymphangioma: imaging pleural eVusions, was also treated by irradia- diagnosis. Australas Radiol 1997;41:324–8. tion with complete regression of disease lasting 10 Bhatti MAK, Ferrante JW, Gielchinsky I, et al. Pulmonary 21 and skeletal lymphangiomatosis with chylothorax and chy- for 12 months at the time of reporting. In the lopericardium. Ann Thorac Surg 1985;40:398–401. recent report by Kandil et al, three patients 11 Hancock BJ, St-Vil D, Luks FI, DI Lorenzo M, Blanchard

H. Complications of lymphangioma in children. J Pediatr http://adc.bmj.com/ with extensive thoracic lymphangiomatosis Surg 1992;27:220–2. were treated primarily by irradiation; this 12 Takemura T, Watanabe M, Tanaka S, et al. Thoracoscopic resection of solitary lymphangioma of the lung. Surg Today resulted in complete and permanent control of 1995;25:651–3. the disease with a follow up of 20 months, 32 13 Hamada K, Ishii Y, Nakaya M, et al. Solitary lymphangioma 22 of the lung. Histopathology 1995;27:482–3. months, and eight years in the three patients. 14 Browse NL, Allen DR, Wilson NM. Management of chylo- There is no agreement as to the best thorax. Br J Surg 1997;84:1711–16. 15 Turner C, Gros S. Treatment of recurrent suprahyoid cervi- radiation doses to treat this disease. Aristizabal cofacial lymphngioma with intravenous cyclophospha- mide. Am J Pediatr Hematol Oncol 1994;16:325–8.

and Runyon used 40 Gy for disease in the pel- on October 1, 2021 by guest. Protected copyright. 19 16 Reinhardt MA, Nelson SC, Sencer SF, Bostrom BC, vis and 26 Gy to the abdomen ; such dose if Kurachek SC, Nesbit ME. Treatment of childhood given to the lungs will result in radiation pneu- lymphangiomas with interferon-alpha. J Pediatr Hematol Oncol 1997;19:232–6. monitis and lung ﬁbrosis later on. It is generally 17 O’Cathail SO, Johnson ML, Rostom AY. Successful control agreed that radiation pneumonitis will seldom of lymphangioma circumscriptum by superﬁcial X-rays. Br J Dermatol 1985;113:611–15. occur with a fractionated total dose of 20 Gy or 18 Denton AS, Baker-Hines R, Spittle MF. Radiotherapy is a less.23 The dose used by Johnson and useful treatment for lymphangioma circumscriptum: a 20 21 report of two cases. Clin Oncol 1996;8:400–1. colleagues and Dajee and Whitehouse was 19 Aristizabal SA, Runyon TD. Radiotherapy of unusual 20 Gy in 10 fractions. In the report by Kandil benign disease. Int J Radiat Oncol Biol Phys 1981;7:1437– 40. et al a dose of 18 Gy in 12 fractions over 18 days 20 Johnson DW, Kalazynski PT, Gordon WH. Mediastinal (uncorrected for lung transmission) was given lymphangioma and chylothorax. The role of radiotherapy. Ann Thorac Surg 1986;41:325–38. with no acute or chronic lung toxicity, and was 21 Dajee H, Woodhouse R. Lymphangioma of the mediasti- eVective in controlling this disease.22 This dose num with chylothorax: the role of radiation treatment. J Thorac Cardiovasc Surg 1994;108:594–5. has also been used in one of the three patients 22 Kandil A, Rostom AY, Mourad WA, Khafaga Y, Gershuny to treat cervical vertebra and skull base with AR, El-housseiny G. Successful control of thoracic lymphangiomatosis by irradiation. Clin Oncol 1997;9:407– similar good result. The radiation mechanism 11. is not fully understood, but may be a result of 23 Stover D, Kaner RJ. Pulmonary toxicity. In: Devita VT, Hellman S, Rosenberg SA, eds. Cancer: principles and prac- radiation induced lymphatic endothelial tice of oncology. Philadelphia: Lippincott-Raven, 1997: oedema and proliferation leading to obstruc- 2729–39.