Clinical spectrum of intrathoracic Castleman disease: a retrospective analysis of 48 cases in a single Chinese hospital

Abstract

Background: Thorax is the common place to develop Castleman disease (CD), but there is no systemic clinical analysis for intrathoracic CD.

Methods: We conducted a retrospective analysis of 48 intrathoracic CD patients with definite pathological diagnosis who were hospitalized between 1992 and 2012 in a Chinese tertiary referral hospital.

Results: The study included 16 cases with unicentric CD (UCD) and 32 cases with multicentric CD (MCD). UCD were younger than MCD (30.5y vs 41.6ys, P < 0.05). MCD were more symptomatic (50% vs 96.9%, P < 0.001) and sicker than UCD, including more fever, hepatomegaly and/or splenomegaly and hypoalbuminemia. All of UCD showed solitary mass in various sites and two of them were complicated by small pleural effusion. In the MCD group, their chest CT showed obvious lymphadenopathy in the hilum and/or mediastinum (100%), diffuse parenchymal lung shadows (43.75%), pleural effusion (40.6%), mass in the mediastinum (6.25%) or hilum (3.12%) and bronchiolitis obliterans (BO) (3.12%). Besides LIP-like images, multiple nodules of different size and sites, patchy, ground-glass opacities and consolidation were showed in their chest CT. Surgery were arranged for all UCD for diagnosis and treatment and all were alive. In MCD group, superficial lymph nodes biopsies (21 cases), surgery biopsy (9 cases) and CT-guided percutaneous lung biopsy (2 cases) were performed. Hyaline vascular (HV) variant were more common in the UCD group (75% vs 37.5%, P < 0.05). In MCD group, 28 cases were prescribed with chemotherapy, one refused to receive therapy and the rest three were arranged for regular follow-up. Among MCD, 18 cases was improved, 7 cases was stable, 4 cases lost follow-up and 3 cases died.

Conclusions: Intrathoracic MCD was more common than UCD in our hospital. MCD was older, more symptomic and sicker than UCD. HV variant were more common in UCD. All of UCD showed mass in various intrathoracic locations and surgery resection was performed for all and all were alive. Mass, pleural effusion, BO and diffuse pulmonary shadows, including LIP-like images, multiple nodules of different size and sites, patchy, GGO and consolidations were showed in our MCD. Most of MCD cases were arranged with chemotherapy and their prognosis were worse than UCD's.

Figure 1

A 40-year-old woman was diagnosed with unicentric Castleman disease of hyaline vascular variant after thoracic surgery. She was admitted to our hospital because of slight chest distress for 9 months. Contrasted chest CT showed a well-defined and homogeneous enhanced mass in the right hilum, with coarse calcification (A-C). It was almost normal under the examination of bronscopy and the transbronchial needle aspiration (TBNA) biopsy of the right hilar mass showed chronic inflammation. Whole resection of the mass was performed and prominent diffuse proliferation of lymphoid cells with central hyalinized vascular structures was showed pathologically (D, Hematoxylin and eosin, ×200). she was diagnosed with Castleman disease of hyaline vascular variant. Chemotherapy was not suggested. she had been alive without recurrence for 7 years.

Figure 2

A 34-year-old man was diagnosed with multicentric Castleman disease of plasma cell variant after 11 years. He was complained of multiple superficial lymphadenopathy and intermittent low-grade fever for 11 years and exertional dyspnea for 4 months. His local multiple biopsies showed lymph node reactive hyperplasia. Short-term prednisone and repeated kinds of antibiotics were prescribed for him but his symptoms recurred. In our hospital, erythrocyte sedimentation rate (ESR) was 140 mm/h and IgG was 133 g/l, which was polycolonal. His high resolution chest CT showed multiple cysts and nodules, consolidations, diffuse bronchovascular thickening (LIP-like images) and multiple hilar and mediastinal lymphadenopathy (A-C). The fifth biopsy was tried in his left epitrochlear lymph nodes and the pathological photomicrographs showed marked interfollicular infiltration of plasma cells [D (Hematoxylin and eosin, ×200) and E (CD138 staining, ×200)]. Then he was diagnosed with Castleman disease of plasma cell variant. He and his families refused chemotherapy, but he showed up in the local clinic regularly.