Images in...

Multifocal pneumocyte hyperplasia in tuberous sclerosis

A M Nagar¹, H Seong Teh¹, R Nee Khoo¹, A C Morani¹, K Vrishni¹, J Raghuram²

¹ Department of Radiology, Changi General Hospital, 2 Simei Street 3, Singapore 560623
² Department of Medicine, Changi General Hospital, 2 Simei Street 3, Singapore 560623

Correspondence to:
cyber_xray{at}yahoo.com

A 29-year-old woman, a known case of tuberous sclerosis, presented to our hospital with a 1-day history of multiple episodes of convulsions. This was associated with vague chest and abdominal pain. CT scans of the brain showed multiple calcific foci in the gray-white matter junctions and in the periventricular region. CT of the abdomen revealed liver hamartomas and renal angiomyolipoma.

CT scans of the thorax showed multiple, tiny, randomly distributed, nodular densities of 3–10 mm in size and numerous miliary nodules 1–3 mm in both lung fields compatible with multifocal micronodular pneumocyte hyperplasia. They were more predominant in the lung periphery and the upper lobes. Few tiny simple cysts were also noted. Plain chest radiographs revealed bilateral diffuse fine nodular opacities (figs 1 and 2).

View this figure (89K): in this window | in a new window | PowerPoint for Teaching   Figure 1 (A) Frontal chest radiographic and (B) magnified view over left upper zone showing bilateral diffuse fine nodule opacities.

 

View this figure (121K): in this window | in a new window | PowerPoint for Teaching   Figure 2 CT scan of thorax through upper lobes showing multiple 1–10 mm nodules (arrows) with random distribution, predominately in the periphery of both lung fileds bilaterally. Note also the presence of a lung cyst (open arrows).

 

Multifocal micronodular pneumocyte hyperplasia is a recently described pulmonary manifestation of tuberous sclerosis. First described by Popper et al in 1991, there are only slightly more than 30 cases reported in the literature since. These lesions are benign hamartomatous proliferations of type II pneumocytes along alveolar septa that exhibit fibrous thickening, increased elastic fibres, and aggregated alveolar macrophages.^{1 2} It is usually with limited clinical significance and no known malignant potential.²

This article has been adapted from Nagar A M, Teh H Seong, Khoo R Nee, Morani A C, Vrishni K, Raghuram J. Multifocal pneumocyte hyperplasia in tuberous sclerosis Thorax 2008;63:186

1. Franz, DN, Brody, A, Meyer, C, et al. Mutational and radiographic analysis of pulmonary disease consistent with lymphangioleiomyomatosis and micronodular pneumocyte hyperplasia in women with tuberous sclerosis. Am J Respir Crit Care Med 2001;164:661–668.[Abstract/Free Full Text]
2. Ristagno, RL, Biddinger, PW, Pina, EM, et al. Multifocal Micronodular Pneumocyte Hyperplasia in Tuberous Sclerosis. AJR 2005;184:S37–S39.[Free Full Text]

CiteULike    Complore    Connotea    Del.icio.us    Digg    Reddit    Technorati    What's this?