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Query: UMLS:C0019621 (Langerhans cell histiocytosis)
 3,250 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Malignant mesothelioma typically encases lungs as a thick rind, while relatively sparing lung parenchyma. We describe an unusual presentation of mesothelioma characterized by diffuse intrapulmonary growth, with absent or inconspicuous pleural involvement, clinically simulating interstitial lung disease (ILD). We identified 5 patients (median age 56 y, all men) with diffuse intrapulmonary malignant mesothelioma in our pathology consultation practice from 2009 to 2012. Clinical history, imaging, and pathology materials were reviewed. Symptoms included chronic dyspnea (4 cases), cough (3), and acute dyspnea with bilateral pneumothorax (1). Chest imaging showed irregular opacities (5), reticulation (4), pleural effusions (2), and subpleural nodular densities (1), without radiologic evidence of pleural disease or masses. A clinicoradiologic diagnosis of ILD was made in all cases, and wedge biopsies were performed. Histologic evaluation revealed a neoplastic proliferation of bland epithelioid or spindled cells, showing various growth patterns simulating silicotic nodules, desquamative interstitial pneumonia, organizing pneumonia, and Langerhans cell histiocytosis. Some areas mimicked adenocarcinoma, with lepidic, acinar, micropapillary, and solid patterns. Initial diagnoses by referring pathologists included reactive changes (1), hypersensitivity pneumonitis versus drug reaction (1), desquamative interstitial pneumonia versus neoplasm (1), and mesothelioma (2). Microscopic pleural involvement was identified in 4 cases. Immunohistochemistry confirmed the characteristic immunophenotype of mesothelioma in all cases. Median survival of 3 patients treated with chemotherapy was 28 months. Two patients received no therapy and survived 3 and 4 weeks, respectively. "Diffuse intrapulmonary malignant mesothelioma" is a rare variant with a distinctive presentation that clinically mimics ILD. Recognition is essential to avoid misdiagnosis.
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PMID:Diffuse intrapulmonary malignant mesothelioma masquerading as interstitial lung disease: a distinctive variant of mesothelioma. 2379 22

Although Langerhans cell histiocytosis (LCH) is a familiar entity to most radiologists and to pediatric radiologists in particular, it is but one of a group of disorders caused by the overproduction of histiocytes, a subtype of white blood cells. Other less familiar diseases in this category are Erdheim-Chester disease (ECD), juvenile xanthogranuloma (JXG), Rosai-Dorfman disease (RDD), and hemophagocytic lymphohistiocytosis (HLH). This review describes the classification system, clinical manifestations, and pathophysiology of each disease, with particular attention to differential radiographic findings, including typical locations of involvement and varying appearances at radiography, computed tomography, magnetic resonance imaging, ultrasonography, and nuclear medicine imaging. Although LCH has a wide variety of manifestations and appearances, classic imaging findings include vertebra plana, skull lesions with a beveled edge, the "floating tooth" sign, bizarre lung cysts, and an absent posterior pituitary bright spot with infundibular thickening. The classic imaging findings of ECD are a perirenal rind of soft tissue and patchy long bone osteosclerosis. RDD has more nonspecific imaging findings, including lymphadenopathy (most commonly cervical) and intracranial lesions. Imaging findings in HLH are broad, with the most common abnormalities being hepatosplenomegaly, cerebral volume loss, and periventricular white matter abnormalities. JXG can manifest at imaging, but radiology does not play a major role in diagnosis. Familiarity with these disorders and their associated imaging findings facilitates correct and timely diagnosis. Imaging also features prominently in the assessment of treatment response.
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PMID:More than just Langerhans cell histiocytosis: a radiologic review of histiocytic disorders. 2538 98