J Korean Soc Radiol.  2016 Aug;75(2):133-137. 10.3348/jksr.2016.75.2.133.

Rare Lung Manifestation of Multifocal Micronodular Pneumocyte Hyperplasia in a Teenage Girl with Tuberous Sclerosis Complex

• Ha SM
• Yoon HK
• Lee SK

• KMID: 2344803
• DOI: http://doi.org/10.3348/jksr.2016.75.2.133

Abstract

Multifocal micronodular pneumocyte hyperplasia (MMPH) is a relatively rare pulmonary disorder that can be associated with tuberous sclerosis complex (TSC). It has been rarely reported in children or adolescents. MMPH is a hamartomatous process of the lung with multiple small nodules, composed of type II pneumocytes. Plain radiography and chest CT in MMPH may demonstrate numerous small nodules measuring 1-10 mm in diameters, distributed randomly throughout both lungs. If MMPH is an initial presentation of TSC, and unless we are familiar with this lung manifestation of TSC, radiologic findings can mimic miliary tuberculosis or metastatic disease. We report a teenage girl with TSC and histologically confirmed MMPH which mimicked miliary tuberculosis at the initial presentation.

MeSH Terms

Adolescent
Child
Female*
Humans
Hyperplasia*
Lung*
Pneumocytes*
Radiography
Tomography, X-Ray Computed
Tuberculosis, Miliary
Tuberous Sclerosis*

Figure

• 

  Fig. 1 Multifocal micronodular pneumocyte hyperplasia with tuberous sclerosis complex in a 15-year old girl. A. Initial chest radiograph for student screening shows numerous fine nodular opacities evenly scattered throughout both lungs. B. High resolution chest CT shows nodules and multiple, randomly distributed, 3–12 mm nodules. No cysts are seen.

• 

  Fig. 2 Brain MR imaging of multifocal micronodular pneumocyte hyperplasia with tuberous sclerosis complex in a 15-year old girl. A. Axial FLAIR image shows a subependymal nodule near the right foramen Monro and multiple high signal intensities at the cerebral cortex and subcortical white matter suggesting cortical and subcortical tubers. B. Axial gradient echo image shows calcified subependymal nodules along the lateral ventricular margins. FLAIR = fluid attenuated inversion recovery, MR = magnetic resonance

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  Fig. 3 Microscopic findings of VATS biopsy specimens of multifocal micronodular pneumocyte hyperplaisa with tuberous sclerosis complex in a 15-year old girl. A. Close-up view of the nodule show increased septal thickness and pneumocyte hyperplasia (arrows) (hematoxylin and eosin stain, × 100). B. Immunohistochemically, most of the proliferating epithelial cells of the lesion are positive for pan-cytokeratin (brown color) (pan-cytokeratin stain, × 40). VATS = video-assisted thoracoscopic surgery

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