ABSTRACT
I. Introduction Diffuse malignant pleural mesothelioma (DMPM) is a rare, insidious disease that poses unique challenges in every aspect of its management, from diagnosis and staging to treatment and follow-up. The actual incidence of this rare disease is difficult to quantify in the United States given the lack of a national registry. The SEER (Surveillance Epidemiology and End Results) database reports a steady incidence for women and a decreasing incidence for men. Whether the SEER database captures the actual rate or misses geographic regions where the incidence is much higher, however, is debatable. On average an incidence of 2000 to 3000 cases each year can be anticipated (1,2). The average physician does not see such cases routinely, and the index of suspicion is not high when a patient presents with a pleural effusion, alone. Consequently, there is often a delay of months from initial visit to eventual diagnosis. Radiographic studies do not reliably predict pathologic staging. In addition, universally accepted and validated pathologic staging systems are lacking. To complicate matters further, consensus treatment guidelines do not exist. Surgery, chemotherapy, radiotherapy, and immunotherapy alone or in various combinations have been applied over the years but rarely in the context of a randomized phase III trial. Surgery is usually offered in the setting of a multimodality treatment approach. Over the last several years, enthusiasm has grown for neoadjuvant chemotherapy given prior to extrapleural pneumonectomy (EPP) or pleurectomy/decortication (P/D). On the other hand, surgical intervention for nonepithelial tumors has waned because of limited survival in patients with nonepithelial histology, even in the setting of the most aggressive protocols.
