Castleman Disease of the Thorax: Clinical, Radiologic, and Pathologic Correlation: From the Radiologic Pathology Archives.
Castleman disease is a complex lymphoproliferative disease pathologically divided into two subtypes, the hyaline vascular variant (HVV) and the plasma cell variant (PCV). The HVV is the most common, is thought to represent a benign neoplasm of lymph node stromal cells, and is treated with surgical resection. It is most commonly found in the mediastinum, where it classically appears as a unicentric, avidly enhancing mass at computed tomography (CT) and magnetic resonance imaging. This appearance can mimic other avidly enhancing mediastinal masses, and location, clinical history, laboratory data, and nuclear medicine single photon emission CT (SPECT) and positron emission tomography (PET) studies can help narrow the differential diagnosis. Multicentric Castleman disease (MCD), which in the majority of cases is composed of the PCV, is an aggressive lymphoproliferative disorder associated with human herpesvirus infection, interleukin 6 dysregulation, and other systemic disorders. While it can be difficult to differentiate MCD from lymphoma, the presence of avidly enhancing lymph nodes can suggest the diagnosis. The purpose of this article is to review the clinical, immunologic, and pathologic findings associated with both unicentric Castleman disease and MCD and discuss how the imaging findings correlate with the pathophysiology of the disease.
From the Department of Diagnostic Radiology and Nuclear Medicine, Division of Chest Imaging (S.J.K., J.R.G.), and Department of Internal Medicine, Division of Pulmonary/Critical Care (J.R.G.), University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201; Department of Hematopathology (A.A.) and Department of Pulmonary and Mediastinal Pathology (T.J.F.), the Joint Pathology Center, Department of Defense, Defense Health Agency, Silver Spring, Md; and Department of Chest Imaging, American Institute for Radiologic Pathology, Silver Spring, Md (J.R.G.).
Seth J
SJ
Kligerman
From the Department of Diagnostic Radiology and Nuclear Medicine, Division of Chest Imaging (S.J.K., J.R.G.), and Department of Internal Medicine, Division of Pulmonary/Critical Care (J.R.G.), University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201; Department of Hematopathology (A.A.) and Department of Pulmonary and Mediastinal Pathology (T.J.F.), the Joint Pathology Center, Department of Defense, Defense Health Agency, Silver Spring, Md; and Department of Chest Imaging, American Institute for Radiologic Pathology, Silver Spring, Md (J.R.G.).
Aaron
A
Auerbach
From the Department of Diagnostic Radiology and Nuclear Medicine, Division of Chest Imaging (S.J.K., J.R.G.), and Department of Internal Medicine, Division of Pulmonary/Critical Care (J.R.G.), University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201; Department of Hematopathology (A.A.) and Department of Pulmonary and Mediastinal Pathology (T.J.F.), the Joint Pathology Center, Department of Defense, Defense Health Agency, Silver Spring, Md; and Department of Chest Imaging, American Institute for Radiologic Pathology, Silver Spring, Md (J.R.G.).
Teri J
TJ
Franks
From the Department of Diagnostic Radiology and Nuclear Medicine, Division of Chest Imaging (S.J.K., J.R.G.), and Department of Internal Medicine, Division of Pulmonary/Critical Care (J.R.G.), University of Maryland School of Medicine, 22 S Greene St, Baltimore, MD 21201; Department of Hematopathology (A.A.) and Department of Pulmonary and Mediastinal Pathology (T.J.F.), the Joint Pathology Center, Department of Defense, Defense Health Agency, Silver Spring, Md; and Department of Chest Imaging, American Institute for Radiologic Pathology, Silver Spring, Md (J.R.G.).
Jeffrey R
JR
Galvin
D005871:Castleman Disease / Q000000981:diagnostic imaging* / Q000473:pathology*; D003937:Diagnosis, Differential; D003952:Diagnostic Imaging; D006801:Humans