大 훌훌 放 射 線 훌§ 學 會 誌 第26卷 第 2 號 pp . 378 - 380, 1990 Journal of Korean Radiological Society , 26 (2) 378-380, 1990

Cystic Lyrnphangiomatosis of Spleen A Case Report with Special Emphasis on CT Findings-

Hye Young Choi , M.D. , Yong Ho Auh , M.D. , Kyung Sik Cho , M.D. ,

Mun Gyu Lee , M.D. , Tae Hwan Lim , M.D. , Dae Chul Suh, M.D.

Department of Diagnostic Radiology, College of Medicine , UJsan University

〈국문초록〉

비장의 낭성 입파관종증의 전산화단층 소견

-1 예 보고-

울산의 대 진단방사선과학 교실

최 혜 영·오용호 · 조경 식·이 문규·임 태 환·서 대 철

비장의 낭성 임파관종증은 아주 드문 양성종양으로 지금까지 문헌상 90예의 보고가 있을 뿐

이다. 이 종양은 일반 방사선학적 검사로는 진단이 어려웠고 최근에 전산화단층촬영과 초음파검

사퉁의 이용으로 보다 정확한 진단이 가능하게 되었지만 비 장의 다른 종양들， 특히 악성임파종과

의 감별은 아직도 용이하지 않은 것으로 알려져있다. 저자들은 최근에 수술로 확진된 비 장 낭성

임파관종종 1예 를 전산화단충촬영소견을 중심으로 문헌고찰과 함께 보고함으로써 이 질환의 감별

진단에 도움을 주고자 한다.

- Abstract-

We report a case of cystic lyrnphangiomatosis of the spleen. Splenic involvement of

lyrnphangioma is quite rare : approximately 90 cases have been reported in the literature. Recent wide use of ultrasound (US) and computed tomography (CT) of the abdomen

renders high detection rate of this benign and usually asymptomatic lesions , alarming

radiologist for recognition of this entity not to be mistaken more frequent and malicious

splenic focal lesion such as lyrnphoma ,

Careful observation of CT and US finding may lead readers to the correct diagnosis or

at least, suggest it as a high priority of differentia l diagnos is.

Index Words: Spleen , lyrnphangioma,

Spleen , computed tomography,

775.3142

775.1211

이 논문은 1989년 11월 27일 접 수하여 1990년 2월 23일에 채 택 되 었음 Received November 27 , 1989, accepted February 23 , 1990

댐

Hye young Choi , et al. : Cystic Lymphangiomatosis of Spleen -

Case Report

A4α-year--old woman was admitted to the

Asan Medical Center with presentation of

generalized weakness. anorexia and known G­

B stones. On physical examination. she had a

mass in left upper quadrant of addomen.

Laboratory examinations demonstrated nor­

mocytic normochronic anemia and pancyt­

openia on peripheral blood. A CT examination

showed an enlarged spleen with multiple sma-

11 irregular low density lesions (Fig. 1). The

splenectomy was performed. A gross specimen

revealed darkish enlarged spleen measuring

13X9X5 cm with protruding loculated multi­

ple cystic masses on surface of the spleen up

to 1.5 cm in diameter(Fig. 2).

Fig. l. CT feature. An enlarged spleen with multiple small irregular low-density cyst-like lesions in the entire spleen.

Discussion

Lymphangiomas are benign malformation

composed of endotheliallined cysts containing

lymph. Lymphangiomas were first described

by Rodenber in 18241l. Lymphangiomas are

classified as cone of three histologic types:

simple. cavernous. or cystic. depending on the

size of the lymphatic channels2l . Our case rep-

Fig. 2. Gross speclmen. Small multlple loculated and some protruding cystic masses in the entlre spleen measuring up to 1.5 cm in diameter.

resent cystic lymphangiomatosis of the spleen.

Cystic lymphangiomas are uncommon tum­

urs. Of cystic lymphangiomas. 75 % occur in

the neck(cystic hygroma). 20 % in the axilla.

and 5 % in the mediastinum. retroperit­

oneum. mesentery. spleen. or colon3l. The ori­

gin of cystic lymphangiomas in unknown. The

most widly accepted theory is the early de­

velonmental sequestration of lymphatic ves­

sels that failed to communicate with normal

draining lymphatics and to become markedly

dilated under pressure of accumulating lym­

ph. Sequestration of central lymphatics forms

cystic hygromas. whereas peripheral obstr­

uction of lymph f10w may cause cavernous or

simple lymphangiomas4l . Cystic lymphangi­

omas may be single or multiple cystic masses

containing serous or chylous f1uid. The chyl­

ous f1uid contains varying amount of protein

and lipid materials . The difference in the f1uid

content depend on the degree of stasis and

the number of channels that communicate

with lymphatic systems5 .6l .

CT appearance of cystic lymphangioma

shows water-density. non-enhancing. and

thin-walled lesions with attenuation ranging

from 10 to 36 HU6.7.8l. This range is consi­

dered due to varying composition of cystic

f1uid. Pyatt et al기 reported a case of lymphan-

댐

- 大韓放射線훌훌學會픔 : 第 26 卷 第 2 號 1990 -

giomatosis of the spleen which showed mult­

iple thin-walled water-density cystic lesions

with attenuation 15-35 HU on CT. Frank et

al8) reported a case of splenic lymphangiomat­

osis which showed an enlarged spleen with

m비tiple discret. non-enhancing cystic masses

with attenuation of 15-20 HU on CT. Our case

외so showed an enlarged spleen with multiple

small irregular lesions with attenuation of 10-

40 HU on CT. Many of the lesions did not

show water-density and it may be due to diffe­

rent cystic content and multiple septation

which gives partial volume effect.

The differential diagnosis of multifocal spl­

enic disease includes lymphoma , infarction ,

septic emboli , metastases , and splenic cysts

Determination of the cystic nature of the les­

ions is helpful to the differential diagnosis.

REFERENCES

1. Avigad S , Jaffe R, Frand M, Yakov 1, Yaacov R:

Lymphangiomatosis with splenic involvement

JAMA 236:2315-2317 , 1976

2 . Wegner G: Unber Lymphangioma. Ach KlinChir

20:641-707 , 1877

3. Singh S , Baboo ML , Pathak IC: Cystic lymphan­

gioma in children (Report of 32 cases including

lesions at rare sites). Surgery 69:947-951 , 1971

4. Leonidas JC , BriII PW, Bhan 1: Cystic retroperi­

toneal Iymphangioma. Radiology 127:203-208,

1978

5. Radinn R, Weiner S , Koenigsberg M et aI: Re-

troperitone외 cystic Iymphangioma. AJR

140:733-734, 1983

6. PilIa TJ. Wolverson MK. Sundara m M et al: CT

evaluation of cystic Iymphangioma of the

mediastinum. Radiology 144:841-842. 1982

7. Pyatt RS. Williams ED. Clark M. Gaskins R: CT

diagnosis of splenic cystic lymphangiomatosis . J

Comput Assist Tomogr 5(3):446-448 , 1981

8 . Frank Pistoia. Stuart K. Markowitz: Splenic lym­

phangiomatosis: CT Diagnosis . AJR 150: 121-

122 ‘ 1988

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