Korean J Radiol.  2014 Apr;15(2):295-299. 10.3348/kjr.2014.15.2.295.

A Rare Case of Diffuse Pulmonary Lymphangiomatosis in a Middle-Aged Woman

• Lim HJ
• Han J
• Kim HK
• Kim TS

• KMID: 1705586
• DOI: http://doi.org/10.3348/kjr.2014.15.2.295

Abstract

Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder characterized by lymphatic channel proliferation. It is mostly reported in children and young adults. Here, we report a case involving a 52-year-old asymptomatic woman who presented with increased interstitial markings, as seen on a chest radiograph. Diffuse interstitial septal thickening was found on a serial follow-up chest computed tomography scan, and lymphangitic metastasis was the primary radiologic differential diagnosis. However, histologic sections of wedge resected lung revealed diffuse pleural and interlobular septal lymphatic proliferation characteristic of DPL.

Keyword

Lymphangiomatosis; Interstitial; Lung; Computed tomography

MeSH Terms

Diagnosis, Differential
Female
Humans
Lung Neoplasms/pathology/*radiography
Lymphangioma/pathology/*radiography
Middle Aged
Rare Diseases/pathology/*radiography
Tomography, X-Ray Computed

Figure

• 

  Fig. 1 52-year-old woman with diffuse pulmonary lymphangiomatosis. Posteroanterior chest radiograph revealing increased interstitial markings in both lungs (A). Initial CT scan demonstrating diffuse interstitial thickening in both lungs (B, C) and lymph node enlargement in right anterior diaphragmatic area (D, arrow). Follow-up CT taken eight months after initial CT scan showing increased interlobular septal thickening (E, F) with more prominent bronchovascular bundle thickening (arrows) and increased bilateral pleural effusion (G). Intra-operative photograph showing hypervascularity of lung surface (H). I. Hematoxylin and eosin-stained section of lesion showing proliferation of thin-walled, anastomosing lymphatic vessels lined by single layer of endothelial cells lacking cytological atypia (arrows, × 200). J. Immunohistochemical staining with D2-40 revealing proliferative lymphatic channels (arrows, × 200).

Reference

1. Swensen SJ, Hartman TE, Mayo JR, Colby TV, Tazelaar HD, Müller NL. Diffuse pulmonary lymphangiomatosis: CT findings. J Comput Assist Tomogr. 1995; 19:348–352.

2. Yoo SH, Song JS, Lee JJ, Lee M, Hwang HS, Jang SJ. Diffuse pulmonary lymphangiomatosis: Pulmonary lymphatic disorder in an adult. Basic Appl Pathol. 2012; 5:63–66.

3. El Hajj L, Mazières J, Rouquette I, Mittaine M, Bolduc JP, Didier A, et al. Diagnostic value of bronchoscopy, CT and transbronchial biopsies in diffuse pulmonary lymphangiomatosis: case report and review of the literature. Clin Radiol. 2005; 60:921–925.

4. Boland JM, Tazelaar HD, Colby TV, Leslie KO, Hartman TE, Yi ES. Diffuse pulmonary lymphatic disease presenting as interstitial lung disease in adulthood: report of 3 cases. Am J Surg Pathol. 2012; 36:1548–1554.

5. Du MH, Ye RJ, Sun KK, Li JF, Shen DH, Wang J, et al. Diffuse pulmonary lymphangiomatosis: a case report with literature review. Chin Med J (Engl). 2011; 124:797–800.

6. Resten A, Maitre S, Humbert M, Rabiller A, Sitbon O, Capron F, et al. Pulmonary hypertension: CT of the chest in pulmonary venoocclusive disease. AJR Am J Roentgenol. 2004; 183:65–70.

7. Wittenberg KH, Swensen SJ, Myers JL. Pulmonary involvement with Erdheim-Chester disease: radiographic and CT findings. AJR Am J Roentgenol. 2000; 174:1327–1331.

8. Copley SJ, Coren M, Nicholson AG, Rubens MB, Bush A, Hansell DM. Diagnostic accuracy of thin-section CT and chest radiography of pediatric interstitial lung disease. AJR Am J Roentgenol. 2000; 174:549–554.

9. Nobre LF, Müller NL, de Souza Júnior AS, Marchiori E, Souza IV. Congenital pulmonary lymphangiectasia: CT and pathologic findings. J Thorac Imaging. 2004; 19:56–59.

10. Tazelaar HD, Kerr D, Yousem SA, Saldana MJ, Langston C, Colby TV. Diffuse pulmonary lymphangiomatosis. Hum Pathol. 1993; 24:1313–1322.