ABSTRACT
PURPOSE: The authors retrospectively reviewed six cases of histologically proven Erdheim-Chester disease (ECD) to evaluate organ involvement and clinical and radiological findings. MATERIALS AND METHODS: Through a search of the pathology databases of four Italian hospitals, we identified six men (mean age, 56 years) with a histological diagnosis of ECD. Histology was performed on retroperitoneal or pulmonary biopsy, depending on disease involvement on imaging. Patients underwent plain radiography of the lower limbs and chest, total-body computed tomography (CT) and bone scintigraphy. Magnetic resonance (MR) imaging was performed in two patients to evaluate the lower limbs and in one patient to study the brain, the chest and the abdomen. RESULTS: Clinical manifestations included dyspnoea (n=2), hydronephrosis (n=2) and bone pain (n=1). Bilateral symmetrical osteosclerosis of the metaphyseal and diaphyseal portions of the lower-limb long bones was present in five patients. Imaging studies revealed extraskeletal manifestations in all patients, including involvement of the retroperitoneal space (n=4), the lung (n=4) and the heart (n=2). CONCLUSIONS: ECD is a multiorgan disease that displays constant involvement of the bones and retroperitoneum; in particular, of the perirenal fat. Although the diagnosis of ECD is histological, imaging can raise suspicion and help to establish a presumptive diagnosis.
Subject(s)
Erdheim-Chester Disease/diagnosis , Adult , Aged , Biopsy , Dyspnea/etiology , Erdheim-Chester Disease/diagnostic imaging , Humans , Hydronephrosis/etiology , Italy , Magnetic Resonance Imaging , Male , Middle Aged , Pain/etiology , Retrospective Studies , Tomography, X-Ray ComputedABSTRACT
Some uncommon diseases may involve the central airways focally or diffusely. These include Wegener's granulomatosis, relapsing polychondritis, tracheobronchopathia osteochondroplastica, amyloidosis, papillomatosis, rhinoscleroma, sarcoidosis and tuberculosis. Related computed tomography (CT) findings essentially include calcifications, luminal stenosis, wall thickening and nodules. The purpose of this paper is to help refine the differential diagnosis among these diseases through the use of multidetector CT (MDCT) imaging.
Subject(s)
Bronchial Diseases/diagnostic imaging , Bronchial Diseases/etiology , Tomography, X-Ray Computed/methods , Tracheal Stenosis/diagnostic imaging , Tracheal Stenosis/etiology , Bronchial Diseases/pathology , Humans , Risk Factors , Tracheal Stenosis/pathologyABSTRACT
Gastroesophageal reflux disease is associated with various otolaryngological disorders. The aim of this retrospective study was to determine the role of gastroesophageal reflux disease in the development of laryngeal squamous cell carcinoma (SCC) in non-smoking and non-drinking patients. The study population consisted of 36 consecutive non-smoking and non-drinking patients with histologically confirmed SCC of the larynx. As a control, a group of 125 lifetime non-smoking and non-drinking cancer-free subjects were selected. Patients with laryngeal cancer had a higher prevalence of gastroesophageal reflux disease than the control subjects (P < 0.0001). Our results confirm the fact that gastroesophageal reflux disease in itself is associated with an increased risk of laryngeal cancer.
