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Cystic lymphangiomatosis of spleen: A case report with special emphasis on CT findings

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大 훌훌 放射線 훌§ 學會誌第26卷第2 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-

울산의대 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 비장의 다른 종양들, 특히 악성임파종과 의 감별은 아직도 용이하지 않은 것으로 알려져있다. 저자들은 최근에 수술로 확진된 비장 낭성 임파관종종 1 예를 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 differential diagnosis.

Index Words: Spleen, lyrnphangioma, Spleen, computed tomography, 775.3142

775.1211

이 논문은 1989 년 11월 27 일 접 수하여 1990 년 2 월 23 일에 채 택 되 었음

Received November 27, 1989, accepted February 23, 1990

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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-

(3)

- 大韓放射線훌훌學會픔 : 26 卷 第2 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. Sundaram 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

-380-

수치

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

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