대한흉부영상의학회 Weekly Chest Cases

Case No : 745 Date 2012-02-08

Courtesy of Hwan Seok Yong MD, Kyung Won Doo MD, Eun-Young Kang MD. / Korea University Guro Hospital
Age/Sex 50 / M
Chief ComplaintDry cough and mild exertional dyspnea

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Figure 1

Diagnosis With Brief Discussion

Diagnosis: Multicentric Castleman Disease with Lung Involvement
Radiologic Findings: Chest CT shows diffuse increased interstitial markings, bronchovascular bundle thickening, interlobular septal thickening, focal ground-glass opacities, and small nodules in both lungs, bilateral symmetrically distributed. And also there are multiple enlarged lymph nodes in the mediastinum, both hilum, axillary areas, and upper abdomen.
The histopathological diagnosis of pulmonary involvement of Castleman's disease (plasma cell type) with interstitial infiltration of plasma cells was made by transbronchial lung biopsy of the right middle lobe.
Brief Review: Castleman disease (CD) is an uncommon benign lymphoproliferative disorder characterized by hyperplasia of lymphoid follicles. On the basis of histologic criteria, it has been divided into two types: hyaline vascular and plasma cell. From a clinical standpoint, CD is also classified as localized or multicentric.
The hyaline vascular type accounts for approximately 90% of cases and usually manifests as a solitary perihilar or mediastinal mass in asymptomatic patients. The plasma cell types are often associated with multicentric Castleman disease (MCD), with generalized lymphadenopathy and hepatosplenomegaly. Clinically, MCD occurs in older population than localized CD, with most patients being in their fifth and sixth decade. It often results in various systemic manifestations, such as fever, anemia, and infections and malignancies such as lymphoma and Kaposi sarcoma. The frequency of MCD associated with a lung lesion is relatively low (10-20%) in the United States. In contrast, Nishimoto et al. reported that 18 of 28 Japanese cases (=64.3%) had a lung lesion.
The CT findings of intrathoracic involvement in MCD include bilateral hilar and mediastinal lymphadenopathy, centrilobular nodular opacities, thin-walled cysts, interlobular septal thickening, thickening of bronchovascular bundles, and ground-glass attenuation. Some reports have noted that the lung lesion in MCD is compatible with lymphocytic interstitial pneumonia (LIP). The enlarged lymph nodes seen in the multicentric form of Castleman disease have histologic findings predominantly of the plasma cell type, with only a small amount of capillary proliferation. This presumably accounts for the relatively low level of enhancement after the intravenous administration of contrast material. MCD is difficult to treat and usually progressive, even with the use of steroids and chemotherapeutic agent. Multicentric CD had a much worse prognosis than localized CD and currently regarded as a potentially malignant lymphoproliferative disorder.
References: 1. Thoracic Imaging: Pulmonary and Cardiovascular Radiology, North American Edition. W. Richard Webb, Charles B. Higgins. Lippincott Williams&Wilkins
2. Takeshi J, Nestor LM, Kazuya I et al. Intrathoracic multicentric Castleman disease: CT Findings in 12 patients. Radiology 1998; 209:477-481
3. A Case of Multicentric Castleman’s Disease Having Lung Lesion Successfully Treated with Humanized Anti-interleukin-6 Receptor Antibody, Tocilizumab. J Korean Med Sci 2010; 25: 1364-1367
Keywords: lung, lymphoproliferative disease,

No. of Applicants : 122

▶ Correct Answer : 12/122, 9.8%

- Onomichi municipal hospital , Japan Hirofumi Mifune
- Yokohama-asahi-chuo-general hospital , Japan Kyoko Nagai
- Kyungpook National University Hospital , Korea (South) Jaekwang Lim
- Oita University, Faculty of Medicine , Japan Fumito Okada
- Yonsei uni Severance hospital , Korea (South) SeokMin Ko
- Saga University , Japan Ryoko Egashira
- Severance hospital , Korea (South) Saerom Hong
- Toyama University Hospital, Laboratory of Pathology , Japan TOMONORI TANAKA
- Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
- China Medical University ,Taiwan,R.O.C. , Taiwan Jun Jun Yeh
- Onomichi municipal hospital , Japan Ryotaro Kishi
- IRSA La Rochelle France , France Denis Chabassiere

▶ Correct Answer as Differential Diagnosis : 16/122, 13.1%

- Kurashiki Seijin-byo Center , Japan Akihiro Tada
- Kohka Public Hospital , Japan Akitoshi Inoue
- Fukuyama daiichi Hospital , Japan Mototsugu Saeki
- jaslok hospital & research centre mumbai , India JAINENDRA JAIN
- XiangYa hospital , China Xia Yu
- Dongnam Institute of Radiological and Medical Sciences , Korea (South) Dae-Wook Yeh
- KRRS , Korea (South) Eui Byun
- Kizawa Memorial Hospital , Japan Yo Kaneko
- Asan Medical Center, Ulsan University , Korea (South) Mi Young Kim
- onomiti , Japan HIdeyuki Kobayashi
- IRSA , France, Metropolitan BIGOT
- KUMC ansan , Korea (South) kihwan kim
- IRCCS Istituto Oncologico - Bari , Italy Carlo Florio
- All India Institute of medical sciences , India Justin Moses
- Medical College Chest Hospital,Thrissur,Kerala , India Raveendran TK
- NDMVP Nashik , India Imran Jindani

▶ Semi-Correct Answer : 6/122, 4.9%

- Seoul St Mary Hospital , Korea (South) Chae Lim
- Heart Center Pontica , Bulgaria VLADISLAV RUSINOV
- Yonsei University Severance Hospital , Korea (South) Kim Hee Yeong
- SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OOKUBO
- Private sector , Greece Vasilios Tzilas
- Hanyang university medical center , Korea (South) YOONAH SONG

대한흉부영상의학회 Korean Society of Thoracic Radiology

Archive of Old Cases

Case No : 745 Date 2012-02-08

Discussion

No. of Applicants : 122