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Weekly Chest CasesImaging Conference Cases

Case No : 4

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  • Age/Sex 28 / F
  • Case Title A 28-year-old woman presented with polyuria and amenorrhea.
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Diagnosis With Brief Discussion

Courtesy
S
Imaging Findings
Initial chest radiograph shows diffuse reticular opacities in entire lung.
The volume of the lung is relatively normal.
HRCT shows numerous air cysts and some nodular opacities in the lung which are more dominant in upper lung.
Parenchyma in costophrenic region is relatively spared.
Sellar MRI shows thickening of pituitary infundibulum, which suggests pituitary germinoma or histiocytosis.

By open lung biopsy, pathologic diagnosis was eosinophilic granuloma.
She was treated as diabetes inspidus.
Discussion
Pulmonary histiocytosis X is an uncommon disease occurring primarily in young adults.
It is characterized radiographically by preserved lung volumes, costophrenic angle sparing, and upper lobe cystic changes.

Pathologic Findings
Histiocytosis X is one of a group of diseases characterized by an abnormal infiltration of mononuclear cells.
The common feature of this disease is abnormal infiltration of tissue by Langerhans cells.
Early pulmonary histiocytosis X is pathologically characterized by interstitial cellular infiltrates
centered on the walls of bronchioles and often extending into the surrounding alveolar interstitium.
This bronchiolitis and subsequent bronchiolar destruction account for the combination of
obstructive and restrictive pulmonary dysfunction characteristic of this disease.
With progression, the pulmonary histiocytosis X lesions become more fibrotic
and appear as discrete stellate lesions with central scarring.

Clinical Characteristics
Pulmonary histiocytosis X occurs most commonly in persons aged 20-40 years.
About 60% of patients are men, a finding that may be explained by the strong association of pulmonary histiocytosis X with cigarette smoking.
Up to 25% of patients with biopsy-proved pulmonary histiocytosis X are asymptomatic at presentation.
Spontaneous pneumothorax is the presenting feature in 15% of cases.
Systemic symptoms (i.e., fever, weight loss, and fatigue) occur in 15-30% of patients.

Radiologic Features
The classic signs of pulmonary histiocytosis X on the chest radiograph are ill-defined or stellate nodules (2-10 mm in size),
reticular or reticulonodular opacities, and cysts or honeycombing.
The opacities are usually more marked in the upper lobes, and characteristically spare the costophrenic angles.
HRCT often demonstrates that abnormalities thought to be reticulonodular or emphysematous at chest radiography are actually due to cysts.
HRCT also demonstrates a higher proportion of nodules less than 5 mm in size than does chest radiography.
The combination of nodules and thin-walled cysts at HRCT is virtually diagnostic of pulmonary histiocytosis X.
Reference
1. Kulwiec EL, Lynch DA, Aguayo SM, Schwarz MI, King TE. Imaging of pulmonary histiocytosis X. RadioGraphics 1992; 12: 515-526
2. Brauner MW, Grenier P, Tijani K, Battesti JP, Valeyre D. Pulmonary Langerhans cell histiocytosis: evolution of lesions on CT scans. Radiology 1997; 204: 497-502

Keywords
Lung, Interstitial lung disease, LCH,
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Current Editor : Sung Shine Shim, MD, PhD. Email : sinisim@ewha.ac.kr

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