Despite the considerable progress in the classification of the idiopathic interstitial pneumonias (IIPs), the lack of an international standard has resulted in variable and confusing diagnostic criteria and terminology. The advent of high-resolution computerized tomography, the narrowed pathologic definition of usual interstitial pneumonia (UIP) and recognition of the prognostic importance of separating UIP from other IIP patterns have profoundly changed the approach to the IIPs. This is an international Consensus Statement defining the clinical manifestations, pathology, and radiologic features of patients with IIP. The major objectives of this statement are to standardize the classification of the idiopathic interstitial pneumonias (IIPs) and to establish a uniform set of definitions and criteria for the diagnosis of IIPs. The targeted specialties are pulmonologists, radiologists, and pathologists. A multidisciplinary core panel was responsible for review of background articles and writing of the document. In addition, this group reviewed the clinical, radiologic, and pathologic aspects of a wide spectrum of cases of diffuse parenchymal interstitial lung diseases to establish a uniform and consistent approach to these diseases and to clarify the terminology, definitions, and descriptions used in routine clinical practice. The final statement was drafted after a series of meetings of the entire committee. The level of evidence for the recommendations made in this statement is largely that of expert opinion developed by consensus. This classification of IIPs includes seven clinico-radiologic-pathologic entities: idiopathic pulmonary fibrosis (IPF), nonspecific interstitial pneumonia, cryptogenic organizing pneumonia, acute interstitial pneumonia, respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and lymphoid interstitial pneumonia. The need for dynamic interaction between pathologists, radiologists, and pulmonologists to accurately diagnose these disorders is emphasized. The level of evidence for the recommendations made in this Statement is largely that of expert opinion developed by consensus. This Statement is an integrated clinical, radiologic, and pathologic approach to the classification of the IIPs. Use of this international multidisciplinary classification will provide a standardized nomenclature and diagnostic criteria for IIP. This Statement provides a framework for the future study of these entities. Key Messages * Unclassifiable interstitial pneumonia : Some cases are unclassifiable for a variety of reasons (see text). † This group represents a heterogeneous group with poorly characterized clinical and radiologic features that needs further study. ‡ COP is the preferred term, but it is synonymous with idiopathic bronchiolitis obliterans organizing pneumonia.

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Dermatofibrosarcoma protuberans has been considered to be of fibrohistiocytic or fibroblastic origin. The purpose of this paper is to identify the original cell strain from which this neoplasm derives, using tissue culture and electron microscopic methods. Thirteen cases of DFSP characterised by clinical, topographical, histological and behavioral criteria were explanted. The emigrating cells were bipolar with two opposed processed and showed a radial arrangement in respect to the explants. After the second week the distal processes tended to curve back towards the cell body forming flame-like structures. This cell morphology and cellular orientation persisted during the whole life of the culture. Electron microscopy was performed in three cases; the newly grown cells maintained an electron microscopic picture similar to that found in the original tumors. This pattern of behaviour is characteristic of fibroblastic tumors and has been found in explants of normal fibroblasts, of fibromatosis and of fibrosarcomas used as controls. On this basis, we believe that DFSP is a fibrosarcoma of the skin of low grade malignancy.

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