British Journal of Radiology (2004) 77, 433-435
© 2004 British Institute of Radiology
doi: 10.1259/bjr/71328192
Paracardiac pleural Castleman disease: radiographic and MR findings
S-H Ng, MD
1
S-F Ko, MD
2
J-W Lin, MD
3
Y-L Wan, MD
1
M-J Hsieh, MD
4 and
T-Y Lee, MD
2
1 Department of Radiology, Chang Gung Memorial Hospitals at Linkou and Keelung, Chang Gung University, Taoyuen Hsien and Keelung Hsien and Departments of 2 Radiology, 3 Pathology and 4 Cardiovascular and Thoracic Surgery, Chang Gung Memorial Hospital at Kaohsiung, Chang Gung University, 123 Ta-Pei Road, Niao-Sung Hsiang Kaohsiung Hsien 833, Taiwan
Correspondence: Dr Tze-Yu Lee
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Abstract
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Castleman disease is a rare benign lymphoid tumour of uncertain aetiology that usually appears as a solitary mediastinal mass. We report a rare case of Castleman disease in the right paracardiac pleural space, occurring in a young woman with non-specific chest discomfort. MRI showed a well-defined, oval mass that was slightly hyperintense on T1 weighted images, inhomogeneously hyperintense on T2 and enhanced T1 weighted images. The patient underwent radical tumour resection and has remained well for 8 years.
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Introduction
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Castleman disease (CD) is an uncommon benign lymphoproliferative disorder. It can be found whenever lymph nodes are present, but approximately 70% of the cases are located in the thorax, 14% in the neck, and 12% in the abdomen and 4% in the axilla [1]. Thoracic CD usually affects the mediastinum or pulmonary hilus, but it also can occur in other spaces [2–7]. Paracardiac pleural CD is extremely rare and only one case has been reported thus far [5]. To our knowledge, this is the second reported case of CD occurring in the paracardiac pleural space and the first reported case diagnosed with MRI.
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Case report
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A 22-year-old non-smoking Chinese woman presented with right lower chest discomfort for 10 months. Physical examination and laboratory findings were unremarkable. Chest radiograph revealed a 4 x 5 cm ovoid mass in the right paracardiac region (Figure 1
). MRI revealed that the mass was sharply defined, pleural-based, slightly hyperintense to the skeletal muscle on T1 weighted images and heterogeneously hyperintense on T2 weighted images. It exhibited moderate enhancement after contrast administration of gadolinium diethylenetriamine penta-acetic acid (Figure 2). A few linear areas of hypointensity within the mass were also demonstrated. During thoracotomy, an encapsulated, hypervascular mass with broad attachment to the right paracardiac parietal pleura was seen. There were several vessels supplying the mass arising from the intercostal arteries. Severe adhesion to the adjacent pericardium, right hemidiaphragm, right middle and lower lobes was also present. Radical tumour resection, partial pericardiectomy and wedge resection of the contiguous right middle and lower lobes were performed. Grossly, the mass was pinkish yellow in colour and had a rubbery consistency with fibrous bands interspersed within the mass. Microscopically, the lesion was composed of germinal follicles with hyalinized vessels surrounded by onion-like arranged layers of small lymphocytes and prominent vascular stroma in the interfollicular tissue. The histolopathological diagnosis was CD of the hyaline-vascular subtype. The patient has been followed-up for 8 years, during which time she has experienced no chest discomfort.
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Figure 1. Posteroanterior chest radiograph shows an oval, well-defined mass (open arrow) in the right cardiophrenic region.
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Figure 2. (a) Axial T1 weighted image shows that the mass (open arrow) is well defined and slightly hyperintense relative to the chest wall muscles. (b) Axial T2 weighted image shows that the mass (open arrow) becomes heterogeneously hyperintense. (c) Axial enhanced T1 weighted image shows moderate enhancement of the mass (open arrow). Note small linear areas of hypointensity in all pulse-sequence images.
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Discussion
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CD is a rare, generally benign disease characterized by atypical proliferation of lymphocytes. There are two major histological subtypes of CD. The hyaline-vascular subtype is more common (90% of cases) and is usually asymptomatic. It is characterized microscopically by small hyaline-vascular follicles and interfollicular capillary proliferation. The less frequent plasma-cell subtype is often associated with systemic manifestations, and is characterized by large hyperplastic follicles with intervening sheets of mature plasma cells. Uncommonly, a transitional or mixed form may be seen [8]. Based on biological behaviour, CD is currently categorized as unicentric form and multicentric form. Most of the former cases can be cured by surgical resection, while most of the latter do not benefit from surgical management [9]. The present case was unicentric CD of the hyaline-vascular type, occurring in a 22-year-old woman who had non-specific chest discomfort and was cured by surgical resection.
CD can be found wherever lymph nodes are present, but most cases are located in the thorax. It also occurs in the pelvis, neck, retroperitoneum, mesentery and axilla [1, 2]. Uncommonly, it can occur in extralymphatic tissues such as the larynx, lung, parotid gland, pancreas, or muscles [10]. The majority of cases of thoracic CD are located along the tracheobronchial tree in the mediastinum or pulmonary hilus, but it also can occur in the intrapulmonary fissure and intercostal space [2–4, 6, 7, 11]. The paracardiac pleural space is an extremely unusual location for CD. Gibbons et al reported the first case of paracardiac pleural CD in 1981 [5], in which the lesion resembled a pericardial cyst.
Radiographically, CD typically appears as a rounded mediastinal or hilar soft tissue density mass with well-defined margins. On CT it generally manifests as a homogeneous or heterogeneous mass of soft tissue attenuation and exhibits prompt contrast enhancement. Arborizing, central, or discrete calcification has been reported, but it is unusual [2, 10–12]. On MRI, the disease is typically isointense or slightly hyperintense relative to muscle on T1 weighted images. It becomes hyperintense on T2 weighted images, and enhances after administration of contrast medium [11, 13–17]. Low signal intensity septa are sometimes depicted on T1 and T2 weighted images, which may correspond to calcification, fibrous tissue, or vessels [13, 15]. Our cases showed similar MR findings, with a few intratumoural hypointense foci in all pulse-sequence images, correlating well with fibrous septa in histopathological examination, providing clues for pre-operative prognosis of this uncommon tumour. Although the differential diagnosis includes other pleural tumours, such as mesothelioma, lymphoma, fibrous tumours, or metastasis, a high degree of suspicion for CD is important for surgical planning because CD is hypervascular and may adhere to the surrounding structures.
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Footnotes
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This paper has been presented at the European Congress of Radiology, EURORAD, ECR 2003, Vienna, 7–11, March 2003. 
Received for publication May 8, 2003.
Revision received June 30, 2003.
Accepted for publication August 20, 2003.
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References
|
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- Fizzera G. Castleman disease: more questions than answers. Hum Pathol 1985;16:202–5.[Medline]
- Keller AR, Hochholzer L, Castleman B. Hyaline-vascular and plasma-cell types of giant lymph node hyperplasia of the mediastinum and other locations. Cancer 1972;29:670–83.[CrossRef][Medline]
- Stavridis GT, Lau OJ. Castleman's disease arising from the intercostal space. Eur J Cardiothorac Surg 1993;7:218–9.[Abstract]
- Reynolds SP, Gibbs AR, Weeks R, Adams H, Davies BH. Massive pleural effusion: an unusual presentation of Castleman's disease. Eur Respir J 1992;5:1150–3.[Abstract]
- Gibbons JA, Rosencrantz H, Posey DJ, Watts M. Angiofollicular lymphoid hyperplasia (Castleman's tumor) resembling a pericardial cyst: differentiation by computerized tomography. Ann Thorac Surg 1982;32:193–6.
- Rena O, Caterina C, Giulinao M. Castleman's disease: unusual intrathoracic localization. Eur J Cardiothorac Surg 2001;19:519–21.[Abstract/Free Full Text]
- Kim JH, Jun TG, Sung SW, Shim YS, Han SK, Kim YW, et al. Giant lymph node hyperplasia (Castleman's disease) in the chest. Ann Thorac Surg 1995;589:1162–5.
- Fizzera G. Castleman's disease and related disorders. Semin Diagn Pathol 1988;5:346–64.[Medline]
- Bowne WB, Lewis JJ, Filippa DA, Niewvizky R, Brooks AD, Burt ME, et al. The management of unicentric and multicentric Castleman's disease: a report of 16 cases and a review of the literature. Cancer 1999;85:706–17.[CrossRef][Medline]
- Ferreiros J, Leon NG, Mata MA, Casanova R, Pedrosa CS, Cuevas A. Computed tomography in abdominal Castleman's disease. J Comput Assist Tomogr 1989;13:433–6.[Medline]
- McAdams HP, Rosado-de-Christenson M, Fishback NF, Templeton PA. Castleman disease of the thorax: radiologic features with clinical and histopathologic correlation. Radiology 1998;209:221–8.[Abstract/Free Full Text]
- Moon WK, IM JG, Han MC. Castleman's disease of the mediastinum: MR imaging features. Clin Radiol 1994;49:466–8.[CrossRef][Medline]
- Ecklund K, Hartnell GG. Mediastinal Castleman disease: MR and MRA features. J Thorac Imaging 1994;9:156–9.[Medline]
- Hsieh ML, Quint Le, Faust JM, Turner JE. Enhancing mediastinal mass at MR: Castleman disease. Magn Reson Imaging 1993;11:599–601.[CrossRef][Medline]
- Luburich P, Nicolau C, Ayuso MC, Torra R, Clavero JA. Pelvic Castleman disease: CT and MR appearance. J Comput Assist Tomogr 1992;16:657–9.[Medline]
- Freeman SJ, Irvine GH, Glew D. Case report: cervical Castleman's disease shown by CT and MRI. Clin Radiol 1994;49:721–3.[CrossRef][Medline]
- Khan J, von Sinner W, Akhtar M, Bazarbashi M. Castleman's disease of the chest. Chest 1994;105:1608–10.[Abstract/Free Full Text]
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Abstract
Introduction
