Neoadjuvant radiotherapy of primary irresectable unicentric Castleman's disease: a case report and review of the literature

Abstract

Background: Castleman disease (CD) is a rare benign disorder characterised by hyperplasia of lymphoid tissue that may develop at a single site or throughout the body. The etiology of this disorder is unclear, although the histopathological presentation can be differentiated into a hyaline vascular variant, a plasma cell variant and a mixed variant. Clinically, it has been recorded that 3 manifestations of CD are characterized: a localized unicentric type, a generalized multicentric type and a mixed form. Surgery remains the main treatment for resectable unicentric CD, since removal of the large node is possible without further complications. No consensus has been reached concerning the most adequate treatment for irresectable unicentric CD.

Methods: Case report of a 67 year old woman.

Results: This report, describes the case of a 67-year-old woman with unicentric Castleman disease located in the right lower abdomen. The patient had symptoms of fatigue, dyspnoea and pain in the right lower abdomen. Computed tomography (CT)- examination revealed a tumour, which had grown to form a close relationship with the common iliac vessels and the sacral bone. A Laparotomy procedure revealed that the tumour was an irresectable mass. Neo-adjuvant radiotherapy (40 Gy) was administered in order to downsize the tumour. Six weeks later a new CT-scan revealed a major reduction of the tumour, which enabled a successful radical resection of the tumour to be performed. Histopathological analysis of the tumour showed the hyaline vascular type of CD.

Conclusions: Neo-adjuvant radiotherapy should be considered in case of an irresectable unicentric CD.

Figure 1

A: Computed Tomography (CT) of the abdomen showing the tumour and its relationship with the sacral vertebrum, the adipose tissue and the common iliac artery and vein, before neo-adjuvant radiotherapy. B: Computed Tomography (CT) of the abdomen showing the marked downsizing of the tumour, after neo-adjuvant radiotherapy.

Figure 2

Histopathology showing lymphoid hyperplasia, atrophic germinal centers and radialy penetrating vessels (lollypop phenomenon) as seen in the HV variant of CD.