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The Korean Journal of Pathology2008; 42: 125-7

Solitary pulmonary lymphangiomas are extremely rare. We report here on an unique case ofsolitary pulmonary lymphangioma in an adult. A well-circumscribed, 6 cm-sized, pleural basedlesion with fluid attenuation was found in a 50-year-old Korean male. He had no previous his-tory of disease or trauma. The wedge-resected lung revealed an ill-demarcated lesion withmultiple microscopic cysts and the cystic walls had loose intervening stroma.

Key Words : Cavernous lymphangioma; Lung neoplasm

Hye-Jong Song∙∙Joungho HanKwhanmien Kim1∙∙Kyung Soo Lee2

Jinwon Seo3

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Solitary Pulmonary Lymphangioma in an Adult

- A Brief Case Report -

125 125

Corresponding AuthorJoungho Han, M.D.Department of Pathology, Samsung Medical Center,Sungkyunkwan University School of Medicine, 50Irwon-dong, Gangnam-gu, Seoul 135-710, KoreaTel: 02-3410-2800Fax: 02-3410-0025E-mail: hanjho@skku.edu

Departments of Pathology, 1ThoracicSurgery, and 2Radiology, SamsungMedical Center, Sungkyunkwan University School of Medicine, Seoul;3Department of Pathology, HallymUniversity Sacred Heart Hospital, Anyang,Korea

Received : October 20, 2007Accepted : November 7, 2007

Lymphangioma can occur in any region of the body in whichthere is lymphatic drainage. Abnormal proliferation of lymphaticvessels in the lung is seen in lymphangiomatosis and solitarylymphangioma. Among these maladies, solitary pulmonary lym-phagioma is extremely rare. Herein, we report on an unique caseof solitary pulmonary lymphangioma in a 50-year-old man.

CASE REPORT

A 50-year-old Korean man present with a left pleural basedovoid lesion (6 cm in diameter) on a routine health examination.He was a smoker of 30 pack-years, but he had no previous his-tory of pulmonary disease or trauma. He did not complain ofany specific respiratory symptoms. On computerized tomogra-phy (CT), there was a well-circumscribed, ovoid lesion withfluid attenuation at the posterobasal segment of the left lowerlobe (Fig. 1A). This lesion was suspected to be attached nearthe pleura rather than to the pulmonary parenchyma. The mag-

netic resonance imaging (MRI) showed intermediate to lowsignal intensity on the T2-weighted image and no significantenhancement was seen on the T1-weighted image. The possi-bility of loculated pleural effusion or a localized pleural masswas proposed based on the radiologic findings. Video-assistedsurgical resection was performed. Although the lesion showedfocal adhesion to the pleura, the lesion showed continuity fromthe pulmonary parechyma on intraoperative inspection. No feed-ing vessel was identified. The wedge-resected lung specimenshowed an ill-defined lesion with multiple microscopic cysts(Fig. 1B, C). Each cyst was lined by a mono-layer of endothe-lial cells. The cyst walls had loose stroma, and this formed a‘‘common wall’’ with the nearby cyst. Lymphoid aggregates inthe lumen revealed that the channel came from the lymphatics.Immunohistochemical stain for D2-40 (podoplanin, 1:130,DAKO, Glostrup, Denmark) shows positivity in the endothe-lial cells of the tumor (Fig. 1D).

He was discharged 4 days after the surgery. There has been noevidence of recurrence during 9 months of follow-up.

126 Hye-Jong Song∙Joungho Han∙Kwhanmien Kim, et al.

DISCUSSION

Localized pulmonary lymphangioma is rare, and it has beenfound in patients with a wide age range (6 months to 67 years).Especially, different clinical presentations in adults and childrenhave been described.1 In infants and neonates, pulmonary lym-phangiomas often present with pneumothorax and respiratorydistress. However, adult patients present with an asymptomat-ic lung lesion that requires differentiation from primary lungcancer. Two cases of cystic lymphangioma in pediatric patientshave been reported in Korea, and both of them also presentedwith respiratory distress.2,3 Our case is the first report of solitarylymphangioma in a Korean adult patient, and this case alsoshowed a clinical presentation that was similar to the previous-ly reported cases.

Histologically, most of the solitary lymphangiomas of lungthat have been reported in the literature showed features com-

patible with cystic lymphangioma.1-5 However, our case showedhistologic similarity to cavernous lymphangioma. The mass wasmainly composed of microcysts rather than grossly visible largecysts, and the cysts contained thick, but loose stroma.

Making the preoperative diagnosis of solitary pulmonary lym-phangioma in adult is difficult. The most common radiologicfinding is a solitary, cystic, peripheral lung lesion. However,atypical findings like spiculation and calcification have beenalso reported and one pediatric case that presented with a largemass has also been reported.1,6 Therefore, making the radiologicdiagnosis of solitary pulmonary lymphangioma is not alwayspossible. Awareness of its occurrence in adults is necessary.

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Fig. 1. (A) Computer tomography shows a well-circumscribed mass lesion (arrows) with fluid attenuation at the posterior aspect of leftlower lung zone. (B) and (C) Cavernous lymphangioma of lung shows many thick anastomosing lymphatic vessels, which grow along thepleura and interlobular septa, compressing normal lung parenchyma. (D) Immunostaining for D2-40 highlight endothelial cells of the tumor.

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