Pulmonary Langerhans cell histiocytosis

Case contributed by Dr Jan Frank Gerstenmaier

Presentation

This patient presented with progressive shortness of breath. No history of previous lung disease.

Patient Data

Age: 45
Gender: Female

Ill defined nodular opacities predominantly in the lower zones

There are numerous spiculated nodules, some of which demonstrate cavitation. No evidence of interstitial lung disease. Pleural spaces are clear. Mediastinum unremarkable.

The patient underwent surgical biopsy (wedge resection)

MACROSCOPIC DESCRIPTION:

"Wedge resection": A lung wedge 35x25x15mm with an L-shaped staple line 40mm in length. There is a pleural defect on the opposite surface to the staple line (inked blue). Sectioning reveals an indiscrete pale tan nodule 8mm. No solid tumours are seen.

MICROSCOPIC DESCRIPTION:

Sections of lung show multiple discrete foci of stellate parenchymal fibrosis containing plump oval cells with grooved nuclei and a mixed chronic inflammatory cell infiltrate including numerous eosinophils. No refractile foreign body material is seen. The adjacent alveolar spaces contain modest numbers of pigment respiratory macrophages. There is no evidence of atypia, dysplasia or malignancy. Immunohistochemically cells stain CD1a+, S-100+ and CD68-. Special stains are negative for micro-organisms.

DIAGNOSIS:

Lung, wedge resection: Langerhan's cell histiocytosis.

Case Discussion

Pulmonary LCH has variable appearances but because there was cavitation of some of the nodules, LCH was included in the imaging differential diagnosis. Given the spiculated nature of the nodules, a malignancy was suspected. 

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Case information

rID: 28305
Case created: 20th Mar 2014
Last edited: 14th Mar 2016
System: Chest
Inclusion in quiz mode: Included

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