submit.radiology.or.kr J Korean Soc Radiol 2011;65(3):271-274 271

INTRODUCTION

Castleman’s disease is a rare benign lymphoproliferative dis-order of unknown etiology. It is characterized by giant lymph node hyperplasia and a nonmalignant course. Castleman’s disease usually involves the mediastinum, with about 70% of cases occurring in the thorax. However, the disease can also occur in an extrathoracic site where lymphoid tissue is pres-ent such as the neck, axilla, shoulder area, mesentery, pelvis, pancreas, and retroperitoneum (1).

The presence of Castleman’s disease in the abdomen or pel-vis is rare, and extremely rare at the omentum. To the best of our knowledge, only one case was reported in 1990 (2). We report one case of omental Castleman’s disease in a 69-year- old female patient, imaged by three cross-sectional modali-ties: US, CT, and MRI with gadolinium.

CASE REPORT

A 69-year-old woman with no previous history of medical illness visited the emergency room with right upper quadrant

pain and mild sweating. She had no fever or weight loss. Initial laboratory testing showed an elevated white blood cell number of 12,000/uL and C-reactive protein level of 17.80 mg/dL. Oth-er laboratory tests, such as electrolytes and urine analysis, were normal. A subsequent constrast-enhanced abdominal CT re-vealed several small stones within the mild dilated intrahepat-ic biliary trees. The findings were consistent with acute chol-angitis. In addition, a 3.3 × 2.7 cm well enhancing mass was incidentally shown in the greater omentum (Fig. 1A). The mass showed a well-defined margin without adjacent abdom-inal solid organ invasion and heterogeneous hypoechogenici-ty to muscle with internal vascularity on ultrasonography (Fig. 1B). To gather more information, the patient underwent MRI with gadolinium enhancement. The results indicated a homogenous mass that was hypointense with T1 weighting (Fig. 1C) and hyperintense with T2 weighting (Fig. 1D) com-pared to the signal intensity of muscles. Homogeneous en-hancement was present on a dynamic study with gadolinium. With these findings, this mass was suspected to be a lympho-ma, desmoid tumor, or Castleman’s disease.

The omental mass was surgically resected (laparoscopic

Case ReportpISSN 1738-2637J Korean Soc Radiol 2011;65(3):271-274

Received June 12, 2011; Accepted July 12, 2011Corresponding author: Eun-Ju Kang, MDDepartment of Radiology, Dong-A University College of Medicine, 1 Dongdaesin-dong 3-ga, Seo-gu, Busan 602-715, Korea. Tel. 82-51-240-5367 Fax. 82-51-253-4931E-mail: medcarrot@naver.com

Copyrights © 2011 The Korean Society of Radiology

Castleman’s disease is a rare benign lymphoproliferative disorder of unknown etiol-ogy with about 70% of cases occurring in the thorax. However, the disorder can also occur in an extrathoracic site where lymphoid tissue is present. We report here a rare case of omental Castleman’s disease in a 69-year-old female patient, imaged by three cross-sectional modalities: US, CT, and MRI with gadolinium.

Index termsCastleman’s DiseaseOmentumMRICTUltrasonography

A Case of Castleman’s Disease Arising from the Greater Omentum대망에서 발생한 Castleman 병의 증례 보고 Eun-Ju Kang, MDDepartment of Radiology, Dong-A University College of Medicine, Busan, Korea

A Case of Castleman’s Disease Arising from the Greater Omentum

submit.radiology.or.krJ Korean Soc Radiol 2011;65(3):271-274272

Fig. 1. A. CT scan of the patient shows a well-circumscribed, well enhancing 3.3 × 2.7 cm mass in the greater omentum (arrow). B. On ultrasonography, the mass shows heterogeneous hypoechogenicity compared to muscle with internal vascularity. C, D. On the MRI, the mass is homogenous hypointense with T1 weighting (C) and hyperintense with T2 weighting (D), compared to the signal intensity of the muscles. E, F. On histopathologic examination, diffusely scattered large follicles with markedly expanded mantle zone (E) (H&E stain, × 20) are present. The enlarged lymphoid follicle shows a small germinal, markedly vascular proliferation, and a broad mantle zone composed of a concentric layer of lymphocytes resulting in an onion skin appearance (F) (H&E stain, × 200).

E

C

A

F

D

B

Eun-Ju Kang

submit.radiology.or.kr J Korean Soc Radiol 2011;65(3):271-274 273

lymphadenitis demonstrated by mild to moderate enhance-ment (9). MRI shows a low intensity mass on T1-weighted images and higher signal intensity on T2-weighted images. The differential diagnosis to distinguish it from lymphoma, leiomyoma, and leiomyosarcoma must be performed (10). However, the radiologic findings of Castleman’s disease are non-specific, and in most cases, the diagnosis of Castleman’s disease is confirmed after the resection and histopathological examination of the specimen.

REFERENCES

1.KellerAR,HochholzerL,CastlemanB.Hyaline-vascular

andplasma-celltypesofgiantlymphnodehyperplasiaof

themediastinumandother locations.Cancer1972;29:

670-683

2.VoltaS,CarellaI,GaetaM,MantineoG,BlandinoA.Cas-

tlemandiseaseofthegreateromentum.AJRAmJRoent-

genol1990;154:654

3.CastlemanB,TowneVW.CaserecordsoftheMassachu-

settsGeneralHospital;weeklyclinicopathologicalexercis-

es;foundedbyRichardC.Cabot.NEnglJMed1954;251:

396-400

4.WalterJF,RottenbergRW,CannonWB,SheridanLA,Pizzi-

mentiJ,OrrJT.Giantmediastinallymphnodehyperplasia

(Castleman’sdisease):angiographicandclinicalfeatures.

AJRAmJRoentgenol1978;130:447-450

5.LowenthalDA,FilippaDA,RichardsonME,BertoniM,

StrausDJ.Generalized lymphadenopathywithmorpho-

logicfeaturesofCastleman’sdisease inanHIV-positive

man.Cancer1987;60:2454-2458

6.FrizzeraG.Castleman’sdiseaseand relateddisorders.

SeminDiagnPathol1988;5:346-364

7.WeisenburgerDD,NathwaniBN,WinbergCD,Rappaport

H.Multicentricangiofollicularlymphnodehyperplasia:a

clinicopathologicstudyof16cases.HumPathol1985;16:

162-172

8.McCartyMJ,VukeljaSJ,BanksPM,WeissRB.Angiofollicu-

larlymphnodehyperplasia(Castleman’sdisease).Cancer

TreatRev1995;21:291-310

9.ZhouLP,ZhangB,PengWJ,YangWT,GuanYB,ZhouKR.

ImagingfindingsofCastlemandiseaseoftheabdomen

mass excision) to get a definite diagnosis. On histopathologic examination, diffusely scattered large follicles with markedly vascular proliferation and small hyalinized germinal center were compatible with the hyaline vascular type of Castleman’s disease (Fig. 1E, F). The mass was completely excised and no other lymph node involvement was seen.

DISCUSSION

Castleman’s disease was first described by Dr. Benjamin Castleman in 1954 in a group of patients with large mediasti-nal lymphadenopathy (3). For the successive cases reported afterward, the names were relatively confusing, including gi-ant lymph node hyperplasia, hamartoma of the lymphatics, giant benign lymphoma, angiofollicular lymphadenosis, tu-mor-like hyperplasia of lymphoid tissue, and so on (4). The etiology of Castleman’s disease is unknown, but there are sev-eral hypotheses involving chronic low-grade inflammation, a hamartomatous process, and abnormal immunological func-tions that have been proposed as likely pathogenetic mecha-nisms (1, 5, 6).

Histologically, Castleman’s disease is divided into two clini-cal types: localized-type and diffuse-type according to the ex-tent of disease spread (7, 8). The hyaline vascular type is the most common variant (encountered in 80-90% of the cases), which is considered to undergo a benign clinical course. Most patients with hyaline vascular type are asymptomatic and a surgical resection is the primary choice of treatment and has the most favorable prognosis. In contrast, the plasma cell type accounts for 9% of cases and is more aggressive. Patients with the plasma cell type tend to experience systemic symptoms, characterized by fever, sweating, weight loss, anemia, an ele-vated sedimentation rate, and hypergammaglobulinemia. Un-like the hyaline vascular type, masses in the plasma cell type are characterized by multiple discrete enlarge lymph nodes, which are mainly treated with radiotherapy, chemotherapy, and immunosuppression, with a poor prognosis (8).

The localized hyaline vascular Castleman’s disease was characterized by homogeneous high enhancement in the ear-ly phase of dynamic enhancements, and a persistent enhance-ment mode similar to that of large arteries in CT and MR im-ages. The imaging feature of the diffuse type was multiple

A Case of Castleman’s Disease Arising from the Greater Omentum

submit.radiology.or.krJ Korean Soc Radiol 2011;65(3):271-274274

usualretroperitonealmass.JUrol1988;139:118-120andpelvis.AbdomImaging2008;33:482-488

10.BartkowskiDP,FerrigniRG.Castleman’sdisease:anun-

대망에서 발생한 Castleman 병의 증례 보고

강은주

Castleman 병은 림프조직증식을 보이는 원인이 잘 알려지지 않은 양성질환으로서 대부분이 흉부에서 발생하지만 림프조

직이 있는 흉부 외 조직에서도 발생할 수 있다. 저자는 아주 드물게 보고된 대망을 침범한 Castleman 병을 경험하였기에

US, CT, MRI 소견을 보고하고자 한다.

동아대학교병원 영상의학과