Eosinophilic Granuloma of the Lung and Rib
Francis J Podbielski, MD,
Todd A Worley, MD,
Jason M Korn, MD,
Mark M Connolly, MD
Department of Surgery, Resurrection–St. Joseph Hospital, Chicago, Illinois, USA
Francis J Podbielski, MD, Tel: +1 413 748 9628, Fax: +1 413 748 9662, Email: francis.podbielski{at}sphs.com, Division of Thoracic Surgery, 271 Carew Street, Springfield, MA 01104, USA.
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ABSTRACT
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Langerhans-cell histiocytosis is characterized by abnormal proliferation of Langerhans cells, and frequently associated with cigarette smoking. A 24-year-old man who underwent excisional biopsy of a pulmonary nodule and segmental rib resection was found to have the eosinophilic granuloma variation of Langerhans-cell histiocytosis.
Key Words: Bone Diseases • Eosinophilic Granuloma • Histiocytosis • Langerhans-Cell • Lung Diseases
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INTRODUCTION
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Pulmonary Langerhans-cell histiocytosis (PLCH) is identified in only 5% of lung biopsy specimens from patients with interstitial lung disease.1 It is characterized histologically as excessive proliferation of Langerhans cells, and affects the lung in isolation or in addition to other organ systems.2
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CASE REPORT
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A 24-year-old man with a history of tobacco use presented to his primary care physician with complaints of left shoulder pain after lifting heavy boxes at work. His medical/surgical history was unremarkable. He was a robust man in no distress, with no point tenderness over the left upper extremity or shoulder, and no neurological or vascular deficits. A chest radiograph showed a 0.5-cm left upper lobe lung nodule and a lytic lesion in the mid portion of the left 3rd rib. Computed tomography of the chest corroborated these findings (Figure 1
). A left thoracotomy and wedge resection of the left upper lobe to include the nodule was undertaken. Segmental resection of the 2nd rib was performed, with a grossly negative margin. Final pathology of the specimens showed eosinophilic granulomas of the lung and rib. Microscopic examination of lung tissue with an S-100 protein immunostain demonstrated an abundance of histiocytes (Figure 2). The patient was without complaint at the 2-year follow-up, and there were no new lesions on his chest radiograph.
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Figure 1. Computed tomograph of the chest with intravenous contrast, demonstrating destruction of the lateral portion of the 3rd rib.
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Figure 2. Photomicrograph of lung tissue, demonstrating an abundance of histiocytes. S-100 protein immunostain, original magnification x400.
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DISCUSSION
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Almost 25% of patients with PLCH are asymptomatic. Pulmonary function depends on the extent of disease, with the most consistent abnormality being a reduction in the carbon monoxide diffusion capacity in 60%–90% of patients.2 The most common early chest radiography findings are a micronodular or reticulonodular and interstitial infiltrate with predominantly middle and upper lobe involvement. The infiltrates become cystic or cavitary lesions as the disease progresses. Similar features are observed on computed tomograms.2 There are no known genetic predisposing factors for PLCH. Most cases are sporadic, although there is a slight preponderance in women.3,4
Morphologic confirmation of the diagnosis can be obtained by bronchoalveolar lavage, transbronchial lung biopsy, or surgical lung biopsy. Transbronchial lung biopsy has a diagnostic yield of 10%–40% because of the patchy nature of the disease.3 The cells are definitively identified by 2 unique features: Birbek granules (5-layered rod-shaped intracellular structures) and the CD1a antigen.2 PLCH should be suspected in an adult with upper lobe infiltrates, especially if pulmonary function testing reveals mild impairment of CO diffusion capacity. It should be stressed that while PLCH is considered a reactive process and not a neoplastic disease, patients presenting with nonspecific symptoms and radiographic findings should be evaluated thoroughly to rule out underlying cancer.
The variable natural history of PLCH and lack of prognostic indicators complicate its management. Smoking cessation is essential. Corticosteroids have been the mainstay of medical therapy, despite limited data supporting their efficacy.5 Chemotherapeutic agents, such as vinblastine, methotrexate, cyclophosphamide, and etoposide, have been used in patients with progressive disease unresponsive to corticosteroids, or with multiorgan involvement.6 Most have an excellent prognosis. Asymptomatic or minimally symptomatic patients tend to have the best outcome. Factors associated with a poor prognosis include advanced age at diagnosis, multiorgan involvement, prolonged constitutional symptoms, extensive parenchymal cavitation, markedly decreased diffusing capacity, and prolonged corticosteroid therapy. Lymphoma or bronchial carcinoma may occur more often in patients with PLCH. Our patient was asymptomatic after 2 years, but periodic radiographic surveillance is mandated to detect recurrent or progressive disease.
Presented at the Southeastern Surgical Congress Annual Meeting, Savannah, GA, USA, February 7–11, 2003.
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REFERENCES
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- Gaensier EA, Carrington CB. Open biopsy of chronic diffuse infiltrative lung disease: Clinical roentgenographic, and physiological correlations in 502 patients. Ann Thorac Surg 1980; 30:411–26.[Abstract/Free Full Text]
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- Casolaro MA, Bernaudin JF, Saltini C, Ferrans VJ, Crystal RG. Accumulation of Langerhans’ cells on the epithelial surface of the lower respiratory tract in normal subjects in association with cigarette smoking. Am Rev Respir Dis 1988;137:406–11.[Medline]
- Schönfeld N, Frank W, Wenig S, Uhrmeister P, Allica E, Preussler H, et al. Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X. Respiration 1993;60:38–44.[Medline]
- Giona F, Caruso R, Testi AM, Moleti ML, Malagnino F, Martelli M, et al. Langerhans’ cell histiocytosis adults: a clinical and therapeutic analysis of 11 patients from a single institution. Cancer 1997;80:1786–91.[Medline]
Asian Cardiovasc Thorac Ann 2009;
17:194-195
© 2009 by SAGE Publications
DOI: 10.1177/0218492309103314
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ABSTRACT
INTRODUCTION
