Weekly Chest CasesArchive of Old Cases

Case No : 621 Date 2009-09-20

  • Courtesy of Sang Hyun Paik, Jai Soung Park, Bea So young / Soonchunhyang University Bucheon Hospital
  • Age/Sex 27 / M
  • Chief ComplaintIncidental findings on chest X-ray
  • Figure 1
  • Figure 2
  • Figure 3
  • Figure 4
  • Figure 5
  • Figure 6

Diagnosis With Brief Discussion

Diagnosis
Castleman's disease, hyaline-vascular variant
Radiologic Findings
Chest radiograph shows a right inferior hilar mass. Axial and coronal reformatted images of MDCT reveal that the right middle mediastinal mass shows a well-defined, lobulated, homogenously enhanced mass. The mass is not involvement to adjacent great vessel and abuts to adjacent pericardium. There is no calcification, or necrosis within the mass. CT attenuation of the mass is 173 HU.

Result of immunohistochemical stain :
CD20: Positive, CD21: Not available, BCL-2: Positive,
CD31: Positive in endothelial cells



H-E x 200. Large follicles showing marked vascular proliferation and hyalinization in their germinal center.

Brief Review
# Form of benign lymphoproliferative disorder of unknown etiology and pathogenesis
- Angiofollicular mediastinal lymph node hyperplasia
- Angiomatous lymph node hamartoma
- Giant mediastinal lymph node hyperplasia
# Affect
- Mediastinal lymph nodes, lung, pleura, chest wall
- Extrathoracic locations : cervical, mesenteric, and retroperitoneal nodes and spleen
# Etiology and pathogenesis : unknown
- Unusual form of lymphoid reaction to viral nature
# Two classification system
- Histological : hyaline vascular (90%), plasma cell (9%), mixed forms (rare)
- Distribution : localized and multicentric
# Localized : hyaline vascular (90%) and asymptomatic
- Solitary, well-circumscribed mediastinal mass
- Infiltrative mass with associated lymphadenopathy
- Occur in young adults, good prognosis after surgical treatment
# Multicentric (disseminated) : Plasma cell (80%) and often symptomatic
- Mediastinal, hilar, cervical, and abdominal lymphadenopathy
- Older
- Fever, sweating, fatigue, anemia, lymphadenopathy, and hepatosplenomegaly : systemic disease

# Localized hyaline vascular form
- Most commonly situated in hila or middle posterior mediastinum
- Focal, well-defined, smooth or lobulated mass
# Multicentric plasma cell variety
- Involves multiple mediastinal compartments
- Diffuse mediastinal widening
- Pulmonary involvement often occurs
Due to lymphoid interstitial pneumonia

# Localized hyaline vascular Castleman's disease
- Mass or enlarged lymph nodes of homogeneous soft tissue attenuation
- Coarse and central calcifications : may be seen
- Highly vascular nature : marked homogeneous enhancement
# Multicentric Castleman's disease
- Multiple, slightly enlarged mediastinal and hilar lymph nodes
- Homogeneous attenuation
- Little enhancement
- Pulmonary manifestations : lymphoid interstitial pneumonia
HRCT : ill defined centrilobular nodules, thin-walled cysts, and thickening of bronchovascular bundles and septa
Less common findings : subpleural nodules, GGO, airspace consolidation, and bronchiectasis





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
3. Kim TJ, Han JK, Kim YH et al. Castleman disease of the abdomen: imaging spectrum and clinicopathologic correlations. J Comput Assist Tomogr 2001;25:207?14.
4. 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
Keywords
Mediastinum, Lymphproliferative disorder, Lymphoid hyperplasia,

No. of Applicants : 91

▶ Correct Answer : 36/91,  39.6%
  • - McGill University Health Centre , Canada Amr Ajlan
  • - PingTung Christian Hospital ,China Medical University ,Taiwan,R.O.C. , Taiwan Jun JunYeh
  • - NOUR EL-ISLAM RADIOLOGY CENTER , Egypt Elsayed Mousa
  • - Soonchunhyang university hospital, Bucheon , Korea (South) ALEUM LEE
  • - hospital Sao Paulo , Brazil israel missrie
  • - MRI Center , nanavati hospital , India vijay jadhav
  • - Shinsegye Rad Clinic, Daegu , Korea (South) Gi Beom Kim
  • - Shinchon Severance Hospital , Korea (South) Ho-Joon Lee
  • - Bundang CHA hospital , Korea (South) Jung Jin Young
  • - Aba Prefecture People Hospital竊ŒSichuan Maerkang竊Œchina , China pu peng
  • - Seoul Nationial University Bundang Hospital , Korea (South) Kyoung Jin Oh
  • - Seoul St Mary , Korea (South) Jin Kyoung Oh
  • - CHRU Lille , France Benjamin Damarey
  • - All India Institute of Medical Sciences , India Ashish Gupta
  • - Hangang Sacred Heart Hospital , Korea (South) Eil Seong Lee
  • - CH Sud Rnion , Reunion jean-baptiste Noel
  • - KING KHALID UNIVERSITY HOSPITAL RADIOLOGY DEPARTMENT , Saudi Arabia elbagir nasser
  • - Diskapi Yildirim Beyazit Hospital, Ankara , Turkey Meric Tuzun
  • - SAISEIKAI KURIHASHI HOSPITAL , Japan YASUO OKUBO
  • - severance hospital , Korea (South) lee kyung jong
  • - CHRU lille , France manuel toledano
  • - Trakya University School of Medicine, Turkey , Turkey Ahmet POLAT
  • - DCA, , India Rajesh Gothi
  • - Dongguk University International Hospital , Korea (South) Ah-young Kim
  • - IRSA , France jean-luc BIGOT
  • - Cairo University , Egypt Mamdouh Eissa
  • - clinique de SAVOIE , France, Metropolitan gay-depassier philippe
  • - Beaulieu clinic Geneva , Switzerland gilles GENIN
  • - CHU GRENOBLE FRANCE , France anne-line MORONI
  • - EKH-Berlin , Germany Michael Weber
  • - NASA SCANS , India RAKESH BHATIA
  • - Mallinckrodt Institute of Radiology , United States Naganathan Mani
  • - Deptt of Radiodiagnosis & Imaging, PGIMER chandigarh , India Ram Galwa
  • - UZ GASTHUISBERG , Belgium Johan COOLEN
  • - CHU caen , France nicolas gautier
  • - Chungnam national university hospital , Korea (South) Jeoung Eun Lee
▶ Correct Answer as Differential Diagnosis : 19/91,  20.9%
  • - SMG-SNUH boramae medical center , Korea (South) Kwang Nam Jin
  • - NTUH , Taiwan Kuei-pin Chung
  • - Father Muller Medical College , Mangalore , India nabil mahmood
  • - SMC , Korea (South) Jihoon Cha
  • - IRCCS Istituto Oncologico Bari , Italy Carlo Florio
  • - Uzunkopru Devlet Hastanesi , Turkey Armagan Sarac
  • - , Korea (South) Gil Hong
  • - Radiologie Centrale CHU Saint Etienne , France Romain MALEZIEUX
  • - Trakya University School of Medicine , Turkey Umut Ugur
  • - Kashan University of Medical Sciences, kashan , Iran Ebrahim Razi
  • - IRSA La Rochelle France , France Denis Chabassiere
  • - Hital SacrCoeur, Montrl , Canada Andrea Ojanguren
  • - Assam medical college , India Karunakaran M
  • - Saga University , Japan Ryoko Egashira
  • - Homs National Hospital , Syria Rami Abou Zalaf
  • - Hospital Sotiria, Athens, Greece , Greece Vasilios Tzilas
  • - Vital Imaging Centre, Mumbai,India , India Ganesh Agrawal
  • - jaslok hospital & research centre mumbai , India JAINENDRA JAIN
  • - All India Institute of medical sciences , India Justin Moses
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