Recurrent pneumothoraces with diffuse small ground‐glass and solid nodules

• Published in: Respirology case reports Vol. 9; no. 12; pp. e0870 - n/a
• Main Authors: Hui, Li Yan Sandra, Cheng, Xin Min, Soo, Ing Xiang, Low, Su Ying
• Format: Journal Article
• Language: English
• Published: Chichester, UK John Wiley & Sons, Ltd 01.12.2021; John Wiley & Sons, Inc; Wiley
• Subjects: Biopsy; Case Report; Case Reports; Cell growth; Chest tubes; cystic lung disease; Cysts; Disease; Females; Granulomas; Hyperplasia; lung nodules; lymphangioleiomyomatosis; multifocal micronodular pneumocyte hyperplasia; Mutation; Patients; Pneumothorax; Smooth muscle; Tuberculosis; Tuberous sclerosis; tuberous sclerosis complex; multifocal micronodular pneumocyte hyperplasia; lung nodules; cystic lung disease; tuberous sclerosis complex; lymphangioleiomyomatosis
• ISSN: 2051-3380; 2051-3380
• DOI: 10.1002/rcr2.870

Multifocal micronodular pneumocyte hyperplasia (MMPH) is the lesser known pulmonary manifestation of tuberous sclerosis. It manifests radiologically as diffuse small ground‐glass and solid nodules. Accurate diagnosis is essential as it can be mistaken for miliary tuberculosis or malignant lesions which necessitates specific treatment. Constellation of radiological features such as multicentric disease at onset and stability over time can help to distinguish MMPH from its differentials. Histologically, MMPH is characterized by hamartomatous proliferation of type II pneumocytes with a lack of high nuclear to cytoplasmic ratio. MMPH confers a benign prognosis unlike its differentials. Therefore, accurate diagnosis is paramount in ensuring appropriate care is delivered. Here, we describe the radiological and histological features of MMPH in a patient with genetically proven tuberous sclerosis complex and co‐existing lymphangioleiomyomatosis. We present here a case multifocal micronodular pneumocyte hyperplasia (MMPH) co‐existing with lymphangioleiomyomatosis in a patient with genetically proven tuberous sclerosis complex. MMPH is the lesser known pulmonary manifestation of tuberous sclerosis and can be easily mistaken for its radiological differentials of malignant/infective causes or pulmonary Langerhans cell histiocytosis if histological evaluation is not obtained. We describe here the radiological and histological findings of MMPH in our patient.