Castleman Disease of the Thorax: Clinical, Radiologic, and Pathologic Correlation:From the Radiologic Pathology Archives

Abstract

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.

International, evidence-based consensus diagnostic criteria for HHV-8–negative/idiopathic multicentric Castleman disease

“…by guest www.bloodjournal.org From iMCD 34 and HHV-8-associated MCD 35 may show diffuse lymph node hypermetabolic abnormalities with lower uptake than high-grade lymphomas. 36 …”

Castleman's Disease

“…CH bir kanseröz hastalık olmamakla birlikte Kaposi sarkomu, non-Hodgkin lenfoma, "polinöropati (P), organomegali (O), endokrinopati (E), monoklonal proteinler (M) ve deri değişiklikleri (S) (POEMS)" sendromu gibi malign durumlara eşlik edebilmektedir. 4 Toraks veya batında tek lenf nodu ile en sık görülen lokalize CH veya birden fazla lenf nodu tutulumu ile multisentrik CH olarak da görülebil-mektedir. Multisentrik formu genellikle HIV ve HHV-8 ile birliktedir.…”

“…Genç hastalarda daha çok HIV enfeksiyonu ile birlikte görülmektedir. 4 Lokalize formu asemptomatik olup rutin çeki-len radyografilerde saptanmaktadır. Kitle saptandı-ğında, büyüklüğü 1-12 cm arasında değişebil-mekte ve kitlenin basısına bağlı semptomlar görü-lebilmektedir.…”

Castleman’s Disease with All Aspects: Single Center Experience with Long Term Follow-up

“…CH erişkinlerde sık olmakla birlikte, çocukluktan itibaren her yaşta görülebilmektedir (12). Hastaların %70'inden fazlasında toraks içinde mediasten veya hilusta yerleşmekle birlikte tüm vücutta da bulunabilmektedir (13).…”