Weekly Chest CasesArchive of Old Cases

Case No : 319 Date 2003-12-06

  • Courtesy of Kyung Won Lee, M.D. / Seoul National University Bundang Hospital, Gyunggi-do, Korea
  • Age/Sex 31 / F
  • Chief ComplaintIncidental CXR abnormality on routine health exam
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Diagnosis With Brief Discussion

Diagnosis
Castleman's disease, hyaline vascular type
Radiologic Findings
Chest PA shows a mass at right hilar area.
CT scans show a mass with smooth margin at right hilum. The mass dose not infiltrate surrounding structure. The mass is well enhanced more than muscle. Right upper lobectomy was performed. The gross specimen shows a yellowish glistening cut surface. Microscopic specimen shows multiple germinal centers interspersed in a population of mononuclear cells.
Brief Review
Castleman disease is a relatively rare disorder of lymphoid tissue, and it is also known as angiofollicular hyperplasia or giant lymph node hyperplasia. The disease may occur anywhere along the lymphatic chain but most commonly is found as a solitary mass in the mediastinum. Two distinct histologic patterns have been described, the hyaline-vascular type, which accounts for 90% of cases, and the plasma cell type accounting for the remainder.
Disseminated Castleman disease is currently regarded as a potentially malignant lymphoproliferative disorder that has been associated POEMS (polyneuropathy, organomegaly, endocrinopathy, monoclonal proteinemia, and skin changes) syndrome, osteosclerotic myeloma, Kaposi sarcoma, and acquired immunodeficiency syndrome. The patient of presented case had the axonal polyneuropathy, borderline hepatosplenomegaly, diabetes mellitus, hyperpigmentation, and hypertricosis.


CT images obtained after the intravascular administration of contrast material show marked enhancement within the mass. Imaging studies show one of three morphologic patterns: (a) a solitary mass (50%), (b) a dominant infiltrative mass with associated lymphadenopathy (40%), or (c) diffuse lymphadenopathy confined to a single mediastinal compartment (10%).
References
1. Moon WK, Im J-G, Kim JS, et al. Mediastinal Castleman disease: CT findings. J Comput Assist Tomogr 1994; 18:43-46
2. McAdams HP, Rosado-de-Christenson M, Fishback NF, Templeton PA. Castleman disease of the thorax: radiologic features with clinical and histopathologic correlation. Radiology 1998; 209:221-228
Keywords
Mediastinum, Lymphproliferative disorder,

No. of Applicants : 24

▶ Correct Answer : 15/24,  62.5%
  • - Annecy Hospital, France Gilles Genin
  • - Armed Force Seoul Hospital, Korea Change Min Park
  • - CH Lyon sud, France Xavier Riviere
  • - CHU Nancy-Brabois, France Denis Regent
  • - Chungju Hospital, Konkuk University, Korea Chang Hee Lee
  • - CIM Saint Dizier, France JC Leclerc
  • - Dong-A University Hospital, Korea Ki-Nam Lee
  • - Ewha Women's University Hospital, Korea Yookyung Kim
  • - Ewha Women's University Hospital, Korea Sung Shine Shim
  • - Incheon Sarang Hospital, Korea Jung Hee Kim
  • - Kyunghee University Hospital, Korea Kyung Ran Ko
  • - Mallinckrodt Institute of Radiology, USA Jin Mo Goo
  • - Seoul National University, Korea Sang-Youn Kim
  • - Seoul National University Hospital, Korea Eun-Ah Park
  • - Social Security Hospital, Ankara, Turkey Meric Tuzun
▶ Semi-Correct Answer : 4/24,  16.7%
  • - Bharat Scans,Chennai R G Gopinath
  • - Centre d'imagerie Jacques Callot, Nancy, France Lionel Cannard
  • - Ev. Krkhs. Hubertus, Berlin, Germany Michael Weber
  • - Hangang SacredHeart Hospital, Korea Eil Seong Le
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