Diffuse pulmonary lymphangiomatosis

Definition

There is a diffuse proliferation of lymphatics and smooth muscle. By definition, in diffuse pulmonary lymphangiomatosis both lungs are involved and there is no extrathoracic abnormality8., although many cases are systemic

Synonyms

Pulmonary lymphangiectasis1,2,3, generalized lymphangiectasis4, (intra)thoracic lymphangiomatosis5,6, and diffuse pulmonary angiomatosis7. The confusing nomenclature of pulmonary lymphatic disorders has been reviewed8.

Epidemiology

Lymphangiomatosis may present at any age but is predominantly a condition of children and young adults, with no gender predilection8.

Clinical features

Patients present with "asthma", dyspnea or haemoptysis8,12. Pleural effusions are common8,12.

Radiology

There are increased interstitial markings. CT shows smooth thickening of the interlobular septa, major fissures, central airways and pleura, perihilar and mediastinal fat11. There are often patchy pulmonary ground glass changes11.

Macroscopic appearances

The lymphatic proliferation renders the bronchovascular bundles, pleura and septa prominent.

Histopathology

Endothelium-lined lymphatic spaces permeate pleura, interlobular septa and bronchovascular sheaths12. There may be bland spindle cells between the vascular channels. There is often eosinophilic acellular material. The lung parenchyma may contain iron-laden macrophages12 but the lung architecture is preserved8.

Immunohistochemistry

Ultrastructure

Differential diagnosis

• Lymphangiectasis: the lymphatics are dilated but not increased in number and do not show an anastomosing pattern. There is no spindle cell proliferation8.

• Lymphangioleiomyomatosis: there are cysts and the spindle cells are positive for HMB45, disrupting the lung architecture.

• Kaposi sarcoma: lacks anastomosing lymphatic channels.

• Diffuse pulmonary haemangiomatosis: the spaces are blood-filled.

• Interstitial emphysema: the spaces are air-filled and lack smooth muscle.

Management

Percutaneous sclerotherapy with doxycycline has been employed with good results9.

Prognosis

The condition is commonly fatal and tends to be more aggressive in younger children12.

References

0 Tumours of the Lung, Pleura, Thymus and Heart. WHO Classification of Tumours. IARC Press 2004.

1 Felman, A. H., R. M. Rhatigan, and K. K. Pierson. 1972. Pulmonary lymphangiectasia: observation in 17 patients and proposed classification. Am. J. Roentgenol. Radium. Ther. Nucl. Med. 116: 548-558

2 Lloyd, E. S., and H. C. Press Jr.. 1979. Congenital pulmonary lymphangiectasis. South. Med. J. 72: 1205-1206

3 Kirchner, J., V. Jacobi, M. Schneider, and R. Wagner. 1997. Primary congenital pulmonary lymphangiectasiaa case report. Wien. Klin. Wochenschr. 12:109:922-924.

4 White, J. E., D. Veale, D. Fishwick, L. Mitchell, and P. A. Corris. 1996. Generalised lymphangiectasia: pulmonary presentation in an adult. Thorax 51: 767-768

5 Swank, D. W., N. G. Hepper, K. E. Folkert, and T. V. Colby. 1989. Intrathoracic lymphangiomatosis mimicking lymphangioleiomyomatosis in a young woman. Mayo Clin. Proc. 64: 1264-1268

6 Margraf, L. R.. 1996. Thoracic lymphangiomatosis. Pediatr. Pathol. Lab. Med. 16: 155-160

7 Canny GJ, Cutz E, MacLusky IB, et al. Diffuse pulmonary angiomatosis. Thorax 1991; 46:851-3

8 Faul JL, Berry GJ, Colby TV, et al. Thoracic lymphangiomas, lymphangiectasis, lymphangiomatosis, and lymphatic dysplasia syndrome. Am J Respir Crit Care Med 2000; 161:1037-46

9 Molitch, H. I., E. C. Unger, C. L. Witte, and E. vanSonnenberg. 1995. Percutaneous sclerotherapy of lymphangiomas. Radiology 194: 343-347

10 Ramani P,Shah A Lymphangiomatosis. Histologic and immunohistochemical analysis of four cases. Am J Surg Pathol 1993; 17:329-35

11 Swensen SJ, Hartman TE, Mayo JR, et al. Diffuse pulmonary lymphangiomatosis: CT findings. J Comput Assist Tomogr 1995; 19:348-52

12 Tazelaar HD, Kerr D, Yousem SA, et al. Diffuse pulmonary lymphangiomatosis. Hum Pathol 1993; 24:1313-22

This page last revised 10.5.2005.

©SMUHT/PW Bishop