Unicentric Castleman's disease of abdomen

Abstract

Castleman's disease (CD) is a rare lymphoproliferative disease of uncertain etiology that affects lymph nodes. CD can be classified as a) unicentric vs. multicentric, based on clinical and radiological findings, b) hyaline vascular (80-90%) vs. plasmacytic (10-20%) vs. mixed cellularity variety based on histopathology. Unicentric disease is more common in the 3rd and 4th decade, whereas the multicentric form is more common in the 5th and 6th decade with no sex predilection. HIV seropositive individuals appear to be at an increased risk for multicentric castleman's disease (MCD) at a younger age due to the increased incidence of HHV- 8 infection. Diagnosis is usually based on histopathology features as imaging features show considerable overlap, thus posing diagnostic difficulties. Overall prognosis is good, particularly in the unicentric variety of disease. We have presented a case of the unicentric CD in a 40 year old male patient having abdominal pain and hematuria as chief complaints.

Keywords: CT; Castleman's Disease; Computed Tomography; MRI; Magnetic Resonance Imaging; idiopathic; unicentric.

Figure 1

40 year old male patient with unicentric Castleman's disease. MRI IMAGES, 1.5 T magnetic strength and Gadolinium 7cc as contrast agent: Lesion is heterogeneously hyperintense in the retroperitoneum, antero-medial to the right renal hilum, displacing renal vessels antero-superiorly and compressing the right renal pelvis (C, D) on T2-weighted images and isointense to muscles (A, B) on T1-weighted images showing intense inhomogeneous post contrast enhancement (E, F).

Figure 2

40 year old male patient with unicentric Castleman's disease. 64 slice thickness MDCT SCAN with 1mm abdominal cuts, 250 mAs and 120 kVp with optiscan 120ml as contrast agent: A, B-Axial and coronal abdominal post contrast cuts showing well defined soft tissue density lesion with intense inhomogeneous enhancement noted in relation to the right renal hilum in the retroperitoneal region, displacing right renal vessels anteriorly with multiple enlarged lymphnodes in pre and para-aortic region. C to F-Axial and coronal thoracic pre and post contrast cuts showing few enlarged lymphnodes in thorax, which do not show the same intense enhancement.

Figure 3

40 year old male patient with Castleman's disease. Histology slides A, B with haematoxylin and eosin stain and 10 × magnifications showing marked vascular proliferation and hyalinization of germinal centres and many large cells with vesiculous nuclei.