Weekly Chest CasesArchive of Old Cases

Case No : 387 Date 2005-03-26

  • Courtesy of Left hilar abnormality on routine chest radiograph / Severance Hospital, Yonsei University, Seoul, Korea
  • Age/Sex 28 / F
  • Chief ComplaintLeft hilar abnormality on routine chest radiograph
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Diagnosis With Brief Discussion

Diagnosis
Castleman disease, hyaline vascular type
Radiologic Findings
Chest radiograph shows a left hilar mass. Axial, coronal and sagittal reformatted images of MDCT reveal that the left hilar mass shows a well-defined, lobulated, homogenously enhanced mass with insinuation to adjacent bronchovascular structures. The mass abuts to adjacent pericardium and extrapericardial left superior pulmonary vein without invasion to the structures. There is no calcification, cystic change or necrosis within the mass.
Left upper lobectomy was performed. The specimen showed a well-defined yellowish solid mass in the left upper lobe of lung, measuring 3.5x3cm in dimension. On it뭩 opening, there is no endobronchial lesion. It abuts to the bronchial wall and involves lung parenchyma. The remaining parenchyma is unremarkable
Brief Review
Castleman disease, also known as angiofollicular hyperplasia or giant lymph node hyperplasia, is a rare disorder of lymphoid tissue. unclear etiology and pathogenesis. This disease may occur anywhere along the lymphatic chain but it is most commonly found as a solitary mass in the mediastinum. Two distinct histologic patterns of Castleman disease have been described, including the hyaline-vascular type, accounting for 90% of cases, and the remainder of cases as the plasma cell type, which is often associated with constitutional symptoms. Three patterns have been reported on CT or MRI, including a solitary noninvasive mass (50%), a dominant infiltrative mass with associated lymphadenopathy (40%), and a matted lymphadenopathy without a dominant mass (10%)
In Castleman disease, CT with contrast material usually shows a dense uniform enhancement. Dynamic CT demonstrates early rapid enhancement and washout in the delayed phase, which are considered as typical imaging characteristics that help to differentiate this disease from other mediastinal tumors such as lymphoma etc. Furthermore, peripheral hypervascularity is a characteristic finding on power Doppler ultrasonography. A punctate or arborizing pattern of calcification may be seen. Some recent studies have reported a considerable number of cases showing heterogeneous attenuation. Meador and McLarney reported that tumors greater than 5 cm in diameter generally demonstrate heterogeneous enhancement. In several studies, a focal low attenuation area within the mass showing delayed enhancement on dynamic CT or MRI, was pathologically proven to be central stellate fibrosis interspersed within the mass. An MRI study has been reported to be useful for the evaluation of peripheral or tumoral hypervascularity and the relationship with adjacent vascular structures, because vascular structures appear signal void with high contrast to the mass.
Treatment of Castleman disease is as follows. Surgical resection is recommended for patients with the unicentric variant of CD because surgical removal of a unicentric mass of hyaline-vascular or hyaline-vascular/plasma cell type is curative. But if it is not possible, partial resection, radiotherapy, or observation alone may be helpful instead of excessively aggressivie therapy. Patients with multicentric disease, either hyaline-vascular or plasma cell type, do not benefit from surgical management and should be candidates for multimodality therapy, the nature of which has yet to be defined..
References
1. Kim YJ, Choi BW, Seo JS, Choe KO. Mediastinal Castleman disease: heterogeneous enhancement

with filling-in pattern on dynamic CT and MRI. Eur J Radiol Extra 52 2004;103-105

2. Bowne WB, Lewis JJ, Filippa DA, Niesvizky R, Brooks AD, Burt ME, Brennan MF. The

Management of Unicentric and Multicentric Castleman뭩 Disease; A Report of 16 Cases and a

Review of the Literature. Cancer 1999;85:706?7

3. Meador TL, McLarney JK. CT features of Castleman disease of the abdomen and pelvis. AJR

2001;175:115?

4. 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?

5. Ota T, Mitsuyoshi A, Zaima M, et al. Visualization of central stellate fibrosis in hyaline vascular type

Castleman disease. Brit J Radiol 1997;70:1060?
Keywords
Lung, Benign tumor, Lymphoid hyperplasia,

No. of Applicants : 32

▶ Correct Answer : 9/32,  28.1%
  • - Annecy Hospital, France Gilles Genin
  • - Busan Veterans Hospital, Korea Suhku Huh
  • - Dong-A University Hospital, Korea Ki-Nam Lee
  • - Good Gang-An Hospital, Korea Sang Hee Lee
  • - Hangang SacredHeart Hospital, Korea Eil Seong Lee
  • - Hanyang University Hospital, Seoul, Korea Yo Won Choi
  • - Seoul National University Hospital, Korea Dae Sik Kim
  • - Social Security Hospital, Ankara, Turkey Meric Tuzun
  • - Yon sei Univ. Hospital, Korea EunHye Yoo
▶ Semi-Correct Answer : 7/32,  21.9%
  • - Annemasse, Polyclinique de Savoie, France Gay-Depassier Philippe
  • - CHU Besancon, France Sebastien Aubry
  • - CIM Saint Dizier, France JC Leclerc
  • - Ev. Krkhs. Hubertus, Berlin, Germany Michael Weber
  • - Hospital de la Marina Baixa, Villajoyosa, Spain Carlos F. Munoz-Nunez
  • - Jankharia Imaging Bhavin Jankharia
  • - Kyunghee university hospital, Seoul, Korea Su Youn Sim
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